Clase Disección Aórtica

Circulation
ACC/AHA CLINICAL PRACTICE GUIDELINE
2022 ACC/AHA Guideline for the Diagnosis and

Management of Aortic Disease: A Report of the
American Heart Association/American College of
Cardiology Joint Committee on Clinical Practice
Guidelines
Developed in collaboration with and endorsed by the American Association for Thoracic Surgery, American College of
Radiology, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of
Thoracic Surgeons, and Society for Vascular Surgery
Endorsed by the Society of Interventional Radiology and Society for Vascular Medicine
Writing Committee Members*

Eric M. Isselbacher, MD, MSc, FACC, Chair; Ourania Preventza, MD, MBA, Vice Chair;
James Hamilton Black III, MD, DFSVS, Vice Chair; John G. Augoustides, MD, FAHA†; Adam W. Beck, MD, DFSVS;
Michael A. Bolen, MD‡; Alan C. Braverman, MD, FACC; Bruce E. Bray, MD, FACC§; Maya M. Brown-Zimmerman, MPH||;
Edward P. Chen, MD, FAHA; Tyrone J. Collins, MD, MSCAI, FACC, FAHA, FSVM¶; Abe DeAnda Jr, MD, FAHA;
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Christina L. Fanola, MD, MSc; Leonard N. Girardi, MD, FAHA#; Caitlin W. Hicks, MD, MS, FSVS**; Dawn S. Hui, MD;
William Schuyler Jones, MD, FACC††; Vidyasagar Kalahasti, MD, FACC; Karen M. Kim, MD, MS‡‡; Dianna M. Milewicz, MD, PhD;
Gustavo S. Oderich, MD; Laura Ogbechie, MSN; Susan B. Promes, MD, MBA; Elsie Gyang Ross, MD, MSc;
Marc L. Schermerhorn, MD, DFSVS§§; Sabrina Singleton Times, DHSc, MPH||||; Elaine E. Tseng, MD, FAHA;
Grace J. Wang, MD, MSCE; Y. Joseph Woo, MD, FACC, FAHA††
AIM: The “2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease” provides recommendations
to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical
treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie,
asymptomatic, stable symptomatic, and acute aortic syndromes).
METHODS: A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews,
and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane
Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published
through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.
STRUCTURE: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery
disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new
*Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry may apply; see Appendix 1
for detailed information. †SCA representative. ‡ACR representative. §AHA/ACC Joint Committee on Clinical Data Standards liaison. ||Lay stakeholder representative.
¶SCAI representative. #AATS representative. **ACC/AHA Joint Committee on Performance Measures liaison. ††AHA/ACC Joint Committee on Clinical Practice
Guidelines liaison. ‡‡STS representative. §§SVS representative. ||||AHA/ACC staff representative.
ACC/AHA Joint Committee on Clinical Practice Guidelines Members, see page e445.
The American Heart Association requests that this document be cited as follows: Isselbacher EM, Preventza O, Black JH 3rd, Augoustides JG, Beck AW, Bolen
MA, Braverman AC, Bray BE, Brown-Zimmerman MM, Chen EP, Collins TJ, DeAnda A Jr, Fanola CL, Girardi LN, Hicks CW, Hui DS, Jones WS, Kalahasti V, Kim KM,
Milewicz DM, Oderich GS, Ogbechie L, Promes SB, Ross EG, Schermerhorn ML, Times SS, Tseng EE, Wang GJ, Woo YJ. 2022 ACC/AHA guideline for the diagnosis
and management of aortic disease: a report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines.
Circulation. 2022;146:e334–e482. doi: 10.1161/CIR.0000000000001106
© 2022 by the American College of Cardiology Foundation and the American Heart Association, Inc.
Circulation is available at www.ahajournals.org/journal/circ
e334 December 13, 2022 Circulation. 2022;146:e334–e482. DOI: 10.1161/CIR.0000000000001106
<zjs;Clinical Statements and Guidelines> • <zjss;10163> • <zdoi;10.1161/CIR.0000000000001106>

Isselbacher et al 2022 ACC/AHA Aortic Disease Guideline
recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added
CLINICAL STATEMENTS
emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before
AND GUIDELINES
and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and
multidisciplinary aortic team expertise in the care of patients with aortic disease.
Key Words: AHA Scientific Statements ◼ abdominal aortic aneurysm ◼ aortic dissection ◼ aortitis ◼ aortopathy ◼ bicuspid aortic valve
◼ blunt traumatic aortic injury ◼ cardiac surgery ◼ guidelines ◼ endovascular aortic repair ◼ heritable thoracic aortic disease
◼ intramural hematoma ◼ malperfusion syndrome ◼ Marfan syndrome ◼ Loeys-Dietz syndrome ◼ penetrating atherosclerotic ulcer
◼ thoracic aortic aneurysm ◼ thoracoabdominal aortic aneurysm ◼ thoracic endovascular aortic repair ◼ vascular surgery
CONTENTS 6.1.2. Genetic Aortopathies. . . . . . . . . . e361

6.1.3. BAV Aortopathy. . . . . . . . . . . . . . . . e373
Abstract. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e334
6.2. AAA: Cause, Risk Factors, and
Top 10 Take-Home Messages. . . . . . . . . . . . . . . . . . . . e336
Screening. . . . . . . . . . . . . . . . . . . . . . . . . . . . . e376
Preamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e337
6.3. Growth and Natural History of
1. Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e338
Aortic Aneurysms . . . . . . . . . . . . . . . . . . . . . e377
1.1. Methodology and Evidence Review. . . . e338
1.2. Organization of the Writing 6.4. Medical Management of Sporadic
Committee. . . . . . . . . . . . . . . . . . . . . . . . . . . e338 and Degenerative Aortic Aneurysm
1.3. Document Review and Approval. . . . . . . e339 Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e378
1.4. Scope of the Guideline . . . . . . . . . . . . . . . e339 6.4.1. Medical Therapy and Risk
1.5. Class of Recommendations and Factor Modification in
Level of Evidence . . . . . . . . . . . . . . . . . . . . e339 Sporadic TAA. . . . . . . . . . . . . . . . . . e378
1.6. Abbreviations. . . . . . . . . . . . . . . . . . . . . . . . e339 6.4.2. Medical Therapy and Risk
2. Normal Anatomy, Abnormal Anatomy, and Factor Modification in AAA. . . . e380
Definitions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e340 6.4.3. Surveillance for Medical
2.1. Normal Aortic Anatomy. . . . . . . . . . . . . . . e340 Management . . . . . . . . . . . . . . . . . e382
2.2. Aortic Landing Zones. . . . . . . . . . . . . . . . . e340 6.5. Surgical and Endovascular Management
2.3. Definitions of Dilation and Aneurysm of Aortic Aneurysms. . . . . . . . . . . . . . . . . . e384
of the Aortic Root and Ascending 6.5.1. Surgery for Sporadic Aneurysms
Thoracic Aorta. . . . . . . . . . . . . . . . . . . . . . . . e341 of the Aortic Root and
2.3.1. Normalizing Aortic Root and Ascending Aorta. . . . . . . . . . . . . . e384
Ascending Aortic Diameters for 6.5.2. Aortic Arch Aneurysms. . . . . . . . . e387
Body Size . . . . . . . . . . . . . . . . . . . . . e343 6.5.3. Descending TAA. . . . . . . . . . . . . . e388
2.4. Definitions and Classification of 6.5.4. Thoracoabdominal Aortic
Acute Aortic Syndrome (AAS). . . . . . . . . . e345 Aneurysms. . . . . . . . . . . . . . . . . . . e392
2.5. Classification of Thoracoabdominal 6.5.5. Abdominal Aortic
Aortic Aneurysm (TAAA). . . . . . . . . . . . . . e349 Aneurysms. . . . . . . . . . . . . . . . . . . e396
2.6. Classification of Endoleaks. . . . . . . . . . . . e349 6.5.6. Surveillance After Aneurysm
3. Imaging and Measurements. . . . . . . . . . . . . . . . . . e349 Repair. . . . . . . . . . . . . . . . . . . . . . . . e401
3.1. Aortic Imaging Techniques to Determine 7. Acute Aortic Syndromes. . . . . . . . . . . . . . . . . . . . . e403
Presence and Progression of Aortic 7.1. Presentation. . . . . . . . . . . . . . . . . . . . . . . . . e403
Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e349 7.2. AAS: Diagnostic Evaluation (Imaging,
3.2. Conventions of Measurements. . . . . . . . . e352 Laboratory Testing). . . . . . . . . . . . . . . . . . . e404
3.2.1. Computed Tomography. . . . . . . . . e353 7.3. Medical Management of AAS. . . . . . . . . e405
3.2.2. Magnetic Resonance Imaging. . . . e353 7.3.1. Acute Medical Management
3.2.3. Echocardiography. . . . . . . . . . . . . . e354 of AAS. . . . . . . . . . . . . . . . . . . . . . . e405
3.2.4. Intravascular Ultrasound. . . . . . . . e354 7.3.2. Subsequent Medical Management
3.2.5. Abdominal Ultrasound. . . . . . . . . . e355 of AAS. . . . . . . . . . . . . . . . . . . . . . . e406
4. Multidisciplinary Aortic Teams . . . . . . . . . . . . . . . . e355 7.4. Surgical and Endovascular Management
5. Shared Decision-Making. . . . . . . . . . . . . . . . . . . . . e357 of Acute Aortic Dissection. . . . . . . . . . . . e406
6. Aneurysms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e358 7.4.1. Acute Type A Aortic
6.1. Thoracic Aortic Aneurysm (TAA) Dissection. . . . . . . . . . . . . . . . . . . . . e407
Causes. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e358 7.4.2. Management of Acute Type B
6.1.1. Sporadic and Degenerative Aortic Dissection. . . . . . . . . . . . . . . e411
TAA. . . . . . . . . . . . . . . . . . . . . . . . . . e361 7.5. Management of IMH . . . . . . . . . . . . . . . . . . e412
Circulation. 2022;146:e334–e482. DOI: 10.1161/CIR.0000000000001106 December 13, 2022 e335

7.6. Management of PAU. . . . . . . . . . . . . . . . . . e414 9.5. Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e441

CLINICAL STATEMENTS
7.6.1. PAU With IMH, Rupture, or 10. Physical Activity and Quality of Life. . . . . . . . . . . e441
AND GUIDELINES
Both . . . . . . . . . . . . . . . . . . . . . . . . . . e414 11. Cost and Value Considerations. . . . . . . . . . . . . . . e442

7.6.2. Isolated PAU. . . . . . . . . . . . . . . . . . . e415 12. Evidence Gaps and Future Directions. . . . . . . . . e443
7.6.3. PAU Open Surgical Repair Versus 12.1. Biomarker Studies. . . . . . . . . . . . . . . . . . . . . e443
Endovascular Repair . . . . . . . . . . . e416 12.2. Genetic and Nongenetic Factors. . . . . . . e443
7.7. Traumatic Aortic Injury. . . . . . . . . . . . . . . . . e416 12.3. Biomechanics of the Aorta. . . . . . . . . . . . . e443
7.7.1. Initial Management of Blunt 12.4. Sex, Race, and Ethnicity. . . . . . . . . . . . . . . e443
Traumatic Thoracic Aortic Injury 12.5. Quality of Life in Patients With Aortic
(BTTAI). . . . . . . . . . . . . . . . . . . . . . . . e416 Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e444
7.7.2. Initial Management of Blunt 12.6. New Endovascular Technology. . . . . . . . . e444
Traumatic Abdominal Aortic 12.7. Optimal Exercise and Rehabilitation
Injury (BAAI). . . . . . . . . . . . . . . . . . . e419 Protocols. . . . . . . . . . . . . . . . . . . . . . . . . . . . . e444
7.7.3. Long-Term Management and 12.8. Equitable Care and Training
Surveillance After Blunt Traumatic Opportunities . . . . . . . . . . . . . . . . . . . . . . . . . e444
Aortic Injury (BTAI). . . . . . . . . . . . . e421 References�� e445
7.8. Long-Term Management and Surveillance Appendix 1
Imaging After AAS . . . . . . . . . . . . . . . . . . . . e421 Author Relationships With Industry and Other
7.8.1. Long-Term Surveillance Imaging Entities (Relevant). . . . . . . . . . . . . . . . . . . . . . . . . . . e478
After Aortic Dissection and Appendix 2
IMH. . . . . . . . . . . . . . . . . . . . . . . . . . . e421 Reviewer Relationships With Industry and Other
7.8.2. Long-Term Management After Entities (Comprehensive). . . . . . . . . . . . . . . . . . . e481
Acute Aortic Dissection and
IMH. . . . . . . . . . . . . . . . . . . . . . . . . . . e422
7.8.3. Long-Term Management and TOP 10 TAKE-HOME MESSAGES FOR
Surveillance for PAUs. . . . . . . . . . e422
THE DIAGNOSIS AND MANAGEMENT OF
8. Pregnancy in Patients With Aortopathy. . . . . . . . e423
8.1. Counseling and Management of AORTIC DISEASE
Aortic Disease in Pregnancy and
1. Because outcomes for patients with aortic disease

Postpartum. . . . . . . . . . . . . . . . . . . . . . . . . . . e423 are enhanced at programs with higher volumes,
8.2. Delivery in Pregnant Patients With experienced practitioners, and extensive manage-
Aortopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . e424 ment capabilities, Multidisciplinary Aortic Team
8.3. Surgery Before Pregnancy in Women care is considered in determining the appropriate
With Aortic Disease. . . . . . . . . . . . . . . . . . . e425 timing of intervention.
8.4. Pregnancy in Patients With Aortopathy: 2. Shared decision-making involving the patient and
Aortic Dissection and Aortic Surgery in a multidisciplinary team is highly encouraged to
Pregnancy. . . . . . . . . . . . . . . . . . . . . . . . . . . . e427 determine the optimal medical, endovascular, and
9. Other Aortic Conditions. . . . . . . . . . . . . . . . . . . . . . e428 open surgical therapies. In patients with aortic dis-
9.1. Inflammatory Aortitis: Diagnosis and ease who are contemplating pregnancy or who
Treatment of Takayasu Arteritis and are pregnant, shared decision-making is especially
Giant Cell Arteritis (GCA). . . . . . . . . . . . . . e428 important when considering the cardiovascular
9.2. Infectious Aortitis. . . . . . . . . . . . . . . . . . . . . . e432 risks of pregnancy, the diameter thresholds for pro-
9.2.1. Diagnosis and Management phylactic aortic surgery, and the mode of delivery.
of Infection of the Native 3. Computed tomography, magnetic resonance imag-
Aorta . . . . . . . . . . . . . . . . . . . . . . . . . e432 ing, and echocardiographic imaging of patients
9.2.2. Diagnosis and Management of with aortic disease should follow recommended
Prosthetic Aortic Graft approaches for image acquisition, measurement and
Infection. . . . . . . . . . . . . . . . . . . . . . . e434 reporting of relevant aortic dimensions, and the fre-
9.3. Atherosclerotic Disease. . . . . . . . . . . . . . . . e435 quency of surveillance before and after intervention.
9.3.1. Aortic Thrombus. . . . . . . . . . . . . . e436 4. At centers with Multidisciplinary Aortic Teams and
9.3.2. Aortic Occlusion. . . . . . . . . . . . . . e436 experienced surgeons, the threshold for surgical
9.3.3. Porcelain Aorta. . . . . . . . . . . . . . . e436 intervention for sporadic aortic root and ascend-
9.4. Coarctation of the Aorta (CoA) and ing aortic aneurysms has been lowered from 5.5
Congenital Abnormalities of the Arch. . . e437 cm to 5.0 cm in selected patients, and even lower
9.4.1. Coarctation of the Aorta. . . . . . . . e437 in specific scenarios among patients with heritable
9.4.2. Other Arch Abnormalities. . . . . . e438 thoracic aortic aneurysms.

5. In patients who are significantly smaller or taller be used to inform regulatory or payer decisions, the intent
CLINICAL STATEMENTS
than average, surgical thresholds may incorporate is to improve quality of care and align with patients’ inter-
AND GUIDELINES
indexing of the aortic root or ascending aortic diam- ests. Guidelines are intended to define practices meeting
eter to either patient body surface area or height, the needs of patients in most, but not all, circumstances
or aortic cross-sectional area to patient height. and should not replace clinical judgment.
6. Rapid aortic root growth or ascending aortic
aneurysm growth, an indication for intervention, is
defined as ≥0.5 cm in 1 year or ≥0.3 cm per year in Clinical Implementation
2 consecutive years for those with sporadic aneu- Management, in accordance with guideline recommen-
rysms and ≥0.3 cm in 1 year for those with heritable dations, is effective only when followed by both practitio-
thoracic aortic disease or bicuspid aortic valve. ners and patients. Adherence to recommendations can
7. In patients undergoing aortic root replacement be enhanced by shared decision-making between clini-
surgery, valve-sparing aortic root replacement is cians and patients, with patient engagement in selecting
reasonable if the valve is suitable for repair and interventions on the basis of individual values, prefer-
when performed by experienced surgeons in a ences, and associated conditions and comorbidities.
Multidisciplinary Aortic Team.
8. Patients with acute type A aortic dissection, if clini-
cally stable, should be considered for transfer to a Methodology and Modernization
high-volume aortic center to improve survival. The The AHA/ACC Joint Committee on Clinical Practice Guide-
operative repair of type A aortic dissection should lines (Joint Committee) continuously reviews, updates, and
entail at least an open distal anastomosis rather modifies guideline methodology on the basis of published
than just a simple supracoronary interposition graft. standards from organizations, including the Institute of Med-
9. There is an increasing role for thoracic endovascular icine,1,2 and on the basis of internal reevaluation. Similarly,
aortic repair in the management of uncomplicated presentation and delivery of guidelines are reevaluated and
type B aortic dissection. Clinical trials of repair of modified in response to evolving technologies and other
thoracoabdominal aortic aneurysms with endografts factors to optimally facilitate dissemination of information to
are reporting results that suggest endovascular health care professionals at the point of care.
repair is an option for patients with suitable anatomy. Numerous modifications to the guidelines have been
10. In patients with aneurysms of the aortic root or implemented to make them shorter and enhance “user
ascending aorta, or those with aortic dissection, friendliness.” Guidelines are written and presented in a
screening of first-degree relatives with aortic imag- modular, “knowledge chunk” format, in which each chunk
ing is recommended. includes a table of recommendations, a brief synopsis,
recommendation-specific supportive text and, when
appropriate, flow diagrams or additional tables. Hyper-
PREAMBLE linked references are provided for each modular knowl-
Since 1980, the American College of Cardiology (ACC) edge chunk to facilitate quick access and review.
and American Heart Association (AHA) have translated In recognition of the importance of cost–value con-
scientific evidence into clinical practice guidelines with siderations, in certain guidelines, when appropriate and
recommendations to improve cardiovascular health. feasible, an analysis of value for a drug, device, or inter-
These guidelines, which are based on systematic meth- vention may be performed in accordance with the ACC/
ods to evaluate and classify evidence, provide a founda- AHA methodology.3
tion for the delivery of quality cardiovascular care. The To ensure that guideline recommendations remain cur-
ACC and AHA sponsor the development and publication rent, new data will be reviewed on an ongoing basis by
of clinical practice guidelines without commercial sup- the writing committee and staff. Going forward, targeted
port, and members volunteer their time to the writing and sections/knowledge chunks will be revised dynamically
review efforts. Guidelines are official policy of the ACC after publication and timely peer review of potentially
and AHA. For some guidelines, the ACC and AHA col- practice-changing science. The previous designations of
laborate with other organizations. “full revision” and “focused update” will be phased out.
For additional information and policies on guideline devel-
opment, readers may consult the ACC/AHA guideline
Intended Use methodology manual4 and other methodology articles.5-7
Clinical practice guidelines provide recommendations
applicable to patients with or at risk of developing car-
diovascular disease. The focus is on medical practice in Selection of Writing Committee Members
the United States, but these guidelines are relevant to The Joint Committee strives to ensure that the guideline
patients throughout the world. Although guidelines may writing committee contains requisite content expertise

and is representative of the broader cardiovascular com- Recommendations are limited to drugs, devices, and
CLINICAL STATEMENTS
munity by selection of experts across a spectrum of treatments approved for clinical use in the United States.
AND GUIDELINES
backgrounds, representing different geographic regions, Joshua A. Beckman, MD, MS, FACC, FAHA
sexes, races, ethnicities, intellectual perspectives/biases, Chair, AHA/ACC Joint Committee on
and clinical practice settings. Organizations and profes- Clinical Practice Guidelines
sional societies with related interests and expertise are
invited to participate as partners or collaborators.
1. INTRODUCTION
Relationships With Industry and Other Entities 1.1. Methodology and Evidence Review
The ACC and AHA have rigorous policies and methods The recommendations listed in this guideline are,
to ensure that documents are developed without bias whenever possible, evidence based. An initial exten-
or improper influence. The complete policy on relation- sive evidence review, which included literature derived
ships with industry and other entities (RWI) can be found from research involving human subjects, published in
online. Appendix 1 of the guideline lists writing com- English, and indexed in MEDLINE (through PubMed),
mittee members’ relevant RWI. For the purposes of full EMBASE, the Cochrane Library, the Agency for Health-
transparency, their comprehensive disclosure information care Research and Quality, and other selected data-
is available in a Supplemental Appendix. Comprehensive bases relevant to this guideline, was conducted from
disclosure information for the Joint Committee is also February 2021 to April 2021. Search terms included
available online. both key words and index terms (eg, MeSH, Emtree);
search terms included but were not limited to the fol-
Evidence Review and Evidence Review lowing: aortic occlusion; aortic aneurysm; aortic aneu-
rysm, thoracic; aortic aneurysm, abdominal; surveillance
Committees after endovascular aneurysm repair; diagnostic imaging;
In developing recommendations, the writing committee monitoring; surveillance; imaging; aorta; aortic; computed
uses evidence-based methodologies that are based on tomography; ultrasound; magnetic resonance imaging;
all available data.4,5 Literature searches focus on ran- arterial occlusive diseases; aortic diseases; aortic ath-
domized controlled trials (RCTs) but also include reg- erosclerosis; atherosclerosis; clinical trial; observational
istries, nonrandomized comparative and descriptive
study; randomized controlled trial; review; atherosclerotic

studies, case series, cohort studies, systematic reviews, aortic disease; plaque, atherosclerotic; aorta; aortitis;
and expert opinion. Only key references are cited. infectious; autoimmune; aortic rupture; penetrating aor-
An independent evidence review committee is com- tic ulcers; comparative studies; nonexperimental studies;
missioned when there are ≥1 question(s) deemed of type A aortic dissection; type A; type B; aneurysm, dis-
utmost clinical importance and merit formal systematic secting; aorta and echocardiography. The final evidence
review to determine which patients are most likely to ben- tables are included in the Online Data Supplement and
efit from a drug, device, or treatment strategy, and to what summarize the evidence used by the writing committee
degree. Criteria for commissioning an evidence review to formulate recommendations. References selected
committee and formal systematic review include absence and published in the present document are representa-
of a current authoritative systematic review, feasibility of tive and not all-inclusive.
defining the benefit and risk in a time frame consistent
with the writing of a guideline, relevance to a substantial
number of patients, and likelihood that the findings can 1.2. Organization of the Writing Committee
be translated into actionable recommendations. Evidence The writing committee consisted of clinicians, cardiolo-
review committee members may include methodologists, gists, internists, interventionalists, surgeons, radiologists,
epidemiologists, clinicians, and biostatisticians. Recom- anesthesiologists, a nurse practitioner, and a lay/patient
mendations developed by the writing committee on the representative. The writing committee included repre-
basis of the systematic review are marked “SR”. sentatives from the ACC, AHA, American Association
for Thoracic Surgery, American College of Radiology,
Society of Cardiovascular Anesthesiologists, Society for
Guideline-Directed Medical Therapy Cardiovascular Angiography and Interventions, Society
The term guideline-directed medical therapy encom- of Thoracic Surgeons (STS), and Society for Vascular
passes clinical evaluation, diagnostic testing, and both Surgery (SVS). Appendix 1 of the present document lists
pharmacological and procedural treatments. For these writing committee members’ relevant RWI. For the pur-
and all recommended drug treatment regimens, the poses of full transparency, the writing committee mem-
reader should confirm dosage with product insert mate- bers’ comprehensive disclosure information is available
rial and evaluate for contraindications and interactions. in a Supplemental Appendix.

1.3. Document Review and Approval Abbreviation Meaning/Phrase
CLINICAL STATEMENTS
The Joint Committee appointed a peer review committee BP blood pressure
AND GUIDELINES
to review the document. The peer review committee was BSA body surface area
comprised of individuals nominated by ACC, AHA, and BTAI blunt traumatic aortic injury
the collaborating organizations. Reviewers’ RWI informa- BTTAI blunt traumatic thoracic aortic injury
tion was distributed to the writing committee and is pub-
CMR cardiac magnetic resonance
lished in this document (Appendix 2).
CoA coarctation of the aorta
This document was approved for publication by the
governing bodies of the ACC and the AHA and was CT computed tomography
endorsed by the American Association for Thoracic Sur- CTA computed tomographic angiography
gery, American College of Radiology, Society of Cardio- DBP diastolic blood pressure
vascular Anesthesiologists, Society for Cardiovascular DMARD disease-modifying anti-rheumatic drug
Angiography and Interventions, Society of Interventional ECG electrocardiogram
Radiology, Society of Thoracic Surgeons, Society for
EVAR endovascular abdominal aortic aneurysm repair
Vascular Medicine, and Society for Vascular Surgery.
FID focal intimal disruption
FDA US Food and Drug Administration
1.4. Scope of the Guideline FDG-PET fluorodeoxyglucose–positron emission tomography
In developing the “2022 ACC/AHA Guideline for the FEVAR fenestrated endovascular aortic repair
Diagnosis and Management of Aortic Disease” (2022
GCA giant cell arteritis
aortic disease guideline), the writing committee reviewed
HRQOL health-related quality of life
previously published guidelines. Table 1 contains a list of
these publications deemed pertinent to this writing effort HTAD heritable thoracic aortic disease
and is intended for use as a resource, thus obviating the ICU intensive care unit
need to repeat existing guideline recommendations. IMH intramural hematoma
IRAD International Registry of Acute Aortic Dissection
1.5. Class of Recommendations and Level of LDL low-density lipoprotein
Evidence
LVV large vessel vasculitis

MR magnetic resonance
The Class of Recommendation (COR) indicates the
strength of recommendation, encompassing the esti- MRA magnetic resonance angiography
mated magnitude and certainty of benefit in proportion MRI magnetic resonance imaging
to risk. The Level of Evidence (LOE) rates the quality of nsHTAD nonsyndromic heritable thoracic aortic disease
scientific evidence supporting the intervention on the PAD peripheral artery disease
basis of the type, quantity, and consistency of data from
PAU penetrating atherosclerotic ulcer
clinical trials and other sources (Table 2).1
PET positron emission tomography
rAAA ruptured abdominal aortic aneurysm
1.6. Abbreviations RCT randomized controlled trial
Abbreviation Meaning/Phrase REBOA resuscitative endovascular balloon occlusion of the aorta
3D 3-dimensional rEVAR endovascular repair for rAAA
AAA abdominal aortic aneurysm SMA superior mesenteric artery
AAS acute aortic syndrome SBP systolic blood pressure
ACEI angiotensin-converting enzyme inhibitor SCI spinal cord injury
AHI aortic height index TAA thoracic aortic aneurysm
AR aortic regurgitation TAAA thoracoabdominal aortic aneurysm
ARB angiotensin receptor blocker TAAD thoracic aortic aneurysm and dissection
ASCA aberrant subclavian artery TAD thoracic aortic disease
ASCVD atherosclerotic cardiovascular disease TAR total arch replacement
ASI aortic size index TEE transesophageal echocardiography
AVR aortic valve replacement TEVAR thoracic endovascular aortic repair
BAAI blunt traumatic abdominal aortic injury TTE transthoracic echocardiography
BAV bicuspid aortic valve VSRR valve-sparing root replacement

Table 1. Associated Guidelines

CLINICAL STATEMENTS
Publication Year
AND GUIDELINES
Title Organization (Reference)

Guidelines
Thoracic endovascular aortic repair for descending thoracic aortic aneurysms SVS 20211
Valvular heart disease ACC/AHA 20202
Large vessel vasculitis EULAR 20203
Blood cholesterol AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/ 20194
APhA/ASPC/NLA/PCNA
Congenital heart disease AHA/ACC 20195
Abdominal aortic aneurysm SVS 20186
High blood pressure ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ 20187
ASPC/NMA/PCNA
Lower extremity peripheral artery disease AHA/ACC 20178
Descending thoracic aorta diseases ESVS 20179
Bicuspid aortic valves statement of clarification ACC/AHA 201610
Vascular graft infections, mycotic aneurysms, and endovascular infections AHA 201611
Endovascular repair of traumatic thoracic aortic injury SVS 201112
Thoracic aortic disease ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/ 201013
SVM
Coronary and other atherosclerotic vascular disease AHA/ACC 200614
Acute type A aortic dissection AATS 202115
Type B aortic dissection STS 202216
AACVPR indicates American Association of Cardiovascular and Pulmonary Rehabilitation; AAPA, American Academy of Physician Assistants; AATS, American As-
sociation for Thoracic Surgery; ABC, Association of Black Cardiologists; ACC, American College of Cardiology; ACCF, American College of Cardiology Foundation;
ACPM, American College of Preventive Medicine; ACR, American College of Radiology; ADA, American Diabetes Association; AGS, American Geriatrics Society;
AHA, American Heart Association; APhA, American Pharmacists Association; ASA, American Society of Anesthesiologists; ASH, American Society of Hematology;
ASPC, American Society for Preventive Cardiology; ESVS, European Society for Vascular Surgery; EULAR, European League Against Rheumatism; NLA, National
Lipid Association; NMA, National Medical Association; PCNA, Preventive Cardiovascular Nurses Association; SCA, Society of Cardiovascular Anesthesiologists;
SCAI, Society for Cardiovascular Angiography and Interventions; SIR, Society of Interventional Radiology; STS, Society of Thoracic Surgeons; SVM, Society for
Vascular Medicine; and SVS, Society for Vascular Surgery.
and distensibility to the aorta, features that are critical to

2. NORMAL ANATOMY, ABNORMAL circulatory function. The adventitia is composed of con-
ANATOMY, AND DEFINITIONS nective tissue, fibroblasts, nerves, and the vasa vasorum,
2.1. Normal Aortic Anatomy which perfuse the outer aortic wall and a substantial por-
tion of the media.
The aorta is the largest artery in the body and can be
divided into 5 main anatomic segments (Figure 1): the
root or sinus segment, which extends from the aortic 2.2. Aortic Landing Zones
valve annulus to the sinotubular junction; the ascend- In addition to the standard anatomic descriptors of the
ing thoracic aorta, which extends from the sinotubular aortic anatomy, there is a more technical classification of
junction to the innominate artery; the aortic arch, which aortic anatomy that is used to plan, guide, and report aor-
extends from the innominate to the left subclavian artery; tic interventions, especially endovascular stent-grafting.
the descending thoracic aorta, which extends from the Because the clinical success of thoracic aortic endovas-
left subclavian artery to the diaphragm; and the abdomi- cular procedures is influenced by the proximal sealing
nal aorta, which extends from the diaphragm to the level zone, in this system the thoracic and abdominal aorta are
of the aortic bifurcation. divided into 11 landing zones, as detailed in Figure 3.
The aortic wall is composed of 3 layers (Figure 2): a Note that Roselli et al2 have proposed a modifica-
thin inner intima, a thicker central media, and a thin outer tion of zone 0, dividing it into 3 subsegments, in which
adventitia. The intima consists of a layer of endothelial 0A extends from the annulus to the distal margin of
cells within a matrix of connective tissue. The media the highest coronary, 0B extends above the coronary to
consists of smooth muscle cells, elastic fibers, collagen the distal margin of the right pulmonary artery, and 0C
proteins, and polysaccharides sandwiched in >50 layers extends from the right pulmonary artery to the distal end
known as elastic lamellae. The media provides strength of the origin of the innominate artery.

Table 2. Applying American College of Cardiology/American Heart Association Class of Recommendation and Level of Evidence to
CLINICAL STATEMENTS
Clinical Strategies, Interventions, Treatments, or Diagnostic Testing in Patient Care* (Updated May 2019)
AND GUIDELINES
2.3. Definitions of Dilation and Aneurysm of the be an aneurysm well below that diameter. Indeed, if this
Aortic Root and Ascending Thoracic Aorta patient had Marfan syndrome or a familial thoracic aor-
tic aneurysm, aortic repair would be recommended at a
The conventional definition of an arterial aneurysm is any diameter of ≤5.0 cm, a size that would not even be large
artery that is dilated to at least 1.5 times its expected enough to be termed an “aneurysm.”
normal diameter.3 This definition applies well to the The most important consideration in deciding the
abdominal and descending thoracic aorta. However, it diameter thresholds at which to call the root and ascend-
has long been recognized that this definition fails when ing aorta dilated or aneurysmal is based on the natural
it comes to defining aneurysms of the aortic root and history of such abnormal aortas. Borger et al4 studied
ascending thoracic aorta. For example, a man in his 40s 201 patients with bicuspid aortic valve (BAV) undergo-
would be expected to have an average aortic root diam- ing aortic valve replacement (AVR) (those undergoing
eter of 3.5 cm; applying the standard definition of ≥1.5 concomitant replacement of the ascending aorta were
times reference diameter, his aortic root would have to excluded) and followed them for 10 to 15 years; they
reach 5.25 cm before it would be considered an aneu- found that those with baseline aortic diameters of 4.5 cm
rysm, whereas most experts would consider his aorta to to 4.9 cm had a significantly increased risk of aneurysm,

CLINICAL STATEMENTS
AND GUIDELINES
Figure 1. The Anatomy of the Aorta and Its Main Branches.
dissection, or sudden death (P<0.001) compared with database. They then analyzed the number of dissections
those with diameters <4.5 cm (Figure 4). (numerator) at each aortic diameter and the population at
To evaluate the risk of type A aortic dissection at various risk at each aortic diameter (denominator). They found that,
diameters below the traditional 5.5 cm threshold for pro- relative to a control aortic diameter of ≤3.4 cm, a diameter
phylactic aortic repair, Paruchuri et al5 plotted a distribu- of 4.0 cm to 4.4 cm conferred an 89-fold increased risk of
tion curve of ascending aortic size in a community sample dissection, and a diameter of ≥4.5 cm conferred a 6000-
from the MESA (Multi-Ethnic Study of Atherosclerosis) fold increased risk (Figure 5), albeit these are only relative

CLINICAL STATEMENTS
AND GUIDELINES
Figure 2. A Simplified Diagram Depicting the Key Histologic
Components of the Aortic Wall.
The medial layer in human aortas contains >50 alternating layers of
elastin and smooth muscle cells (whereas only 5 are shown in this
simplified illustration). Adapted (cropped) from “Illustration of tunics of
the arteries vs veins” by Malgosia Wilk-Blaszczak, used under CC-BY
4.0. “Illustration of tunics of the arteries vs veins” is adapted (cropped)
from figure 20.3 in BC OpenStax Anatomy and Physiology used
under CC-BY 4.0.
risk estimates and do not inform absolute risk. It follows

that the increase in risk at 4.0 cm to 4.4 cm justifies defin-
ing an aorta of this size “dilated,” and the abrupt increase
in risk at a diameter of ≥4.5 cm justifies defining an aorta

of this size as an “aneurysm.” Using this approach, of the
subjects in the MESA database, only 2.6% would be con-
sidered to have a dilated aorta and only 0.2% to have an
aneurysm.
This definition of a dilated ascending aorta being ≥4.0 Figure 3. Classification of Aortic Anatomic Segments by 11
cm is consistent with what was proposed in the 2014 Landing Zones.
European Society of Cardiology guidelines on the diag- Zone 0 (involves the ascending to distal end of the origin of the
nosis and treatment of aortic diseases, in which aortic innominate artery); Zone 1 (involves the origin of the left common
“dilation” was similarly defined as an aorta diameter of carotid; between the innominate and the left carotid); Zone 2 (involves
the origin of the left subclavian; between the left carotid and the
>4.0 cm.6 left subclavian); Zone 3 (involves the proximal descending thoracic
Finally, in the clinical setting, the term “dilation” is aorta down to the T4 vertebral body; the first 2 cm distal to the left
preferred to “ectasia” to describe mild aortic enlarge- subclavian); Zone 4 (the end of zone 3 to the mid-descending aorta –
ment. Historically, there has been a lack of uniformity in T6); Zone 5 (the mid-descending aorta to the celiac); Zone 6 (involves
the use of “ectasia” in image interpretation. Many radi- the origin of the celiac; the celiac to the superior mesenteric); Zone
7 (involves the origin of the superior mesenteric artery; the superior
ologists use “ectatic” rather than “dilated” to describe mesenteric to the renals); Zone 8 (involves the origin of the renal
a mildly enlarged aorta, whereas others use “ectatic” arteries; the renal to the infrarenal abdominal aorta); Zone 9 (the
to describe an abnormal aortic shape, such as a “tor- infrarenal abdominal aorta to the level of aortic bifurcation ); Zone 10
tuous” aorta.7 Even more problematic is the fact that (the common iliac); Zone 11 (involves the origin of the external iliac
some imaging groups use the term “ectasia” to describe arteries). From Czerny et al.1 Copyright 2019, with permission from
Elsevier, Inc., Now Medical Studios, and Oxford University Press on
larger aortas, such as those 4.5 cm to 5.4 cm in diam- behalf of the European Association for Cardio-Thoracic Surgery.
eter,8 which overlaps with what most experts would
consider to be an aneurysm. Lastly, in imaging of the 2.3.1. Normalizing Aortic Root and Ascending Aortic
coronary arteries, “ectasia” is typically used to describe Diameters for Body Size
diffuse (rather than focal) coronary artery dilation,9 As with the aortic diameter thresholds for surgery pre-
which may lead to some clinical uncertainty when “ecta- sented in this guideline, it recognized that the 4.0 cm
sia” is applied to the aorta. and 4.5 cm diameter thresholds discussed previously

CLINICAL STATEMENTS
AND GUIDELINES
Figure 4. Freedom From Ascending

Aortic Complications for Patients
With Bicuspid Aortic Valve Disease.
Patients with moderate dilation of the
ascending aorta (4.5 cm–4.9 cm) had a
significantly increased risk of future aortic
complications (aneurysm, dissection, or
sudden death). Reprinted from Borger et
al.4 Copyright 2004, with permission from
Elsevier, Inc. and the American Association
for Thoracic Surgery.
are intended for those whose height, body surface area correlating BSA and aortic root diameter to generate
(BSA), or both is within 1 to 2 standard deviations of the the z-score. One limitation of the reliance on BSA is that
mean. For male and female patients who are significantly there are multiple formulae to calculate BSA that yield
shorter or taller than average, these diameters need to different results for the same patient. A second limitation
be adjusted downward or upward, accordingly. Several is that multiple z-score calculators exist, each performing
methods to normalize aortic diameter are currently used differently.10 Finally, most of the literature on the natu-
in clinical practice and clinical research. ral history of acute aortic syndromes (AAS) is based on
The Z-Score aortic diameters, whereas reports of outcome based on
The z-score is routinely used to assess aortic dilation z-scores are limited, so the z-score is not typically used to
in the pediatric population, as changes in a child’s age report the degree of aortic dilation in adults.
and body size make it especially challenging to define The Aortic Size Index and Aortic Height Index
normal aortic size and to distinguish normal from patho- Most often, in the clinical care of adult patients, aortic
logic aortic growth. Nomograms have been established diameters are normalized using a ratio of aortic diameter
Figure 5. Relative Risk of Aortic

Dissection by Size Range.
The relative risk of aortic dissection begins
to increase appreciably at a diameter
of 4.0 cm to 4.4 cm and then increases
dramatically at a diameter of ≥4.5 cm.
Reprinted from Paruchuri et al.5 Copyright
2005, with permission from Karger
Publishers, Basel‚ Switzerland.

to BSA or aortic diameter to the patient’s height. In 2006, pler cross-sectional area to height ratio of ≥10 cm2/m
CLINICAL STATEMENTS
Davies et al11 showed that aortic size index (ASI), which is (rather than >10 cm2/m) as the threshold predictive of
AND GUIDELINES
defined as aortic diameter (cm)/BSA(m2), is a better pre- increased risk.14,15
dictor of adverse aortic events than diameter alone, and
that a simple nomogram could be used to stratify those
with aortic aneurysms into low-, medium-, and high-risk 2.4. Definitions and Classification of Acute
groups. However, it is unclear whether the weight of an Aortic Syndrome (AAS)
adult has a significant impact on the expected normal AAS are life-threatening conditions in which there is a
aortic diameter, and one would not expect a patient’s breach in the integrity of the aortic wall. The most com-
aorta to grow or shrink with significant fluctuations in mon AAS are aortic dissection, intramural hematoma
weight. Zafar et al12 therefore examined whether aortic (IMH), and penetrating atherosclerotic ulcer (PAU), all of
height index (AHI), which is defined as aortic diameter which can lead to rupture (Figure 6).
(cm)/patient height (m), might perform better than the
ASI, and they reported that the AHI performed at least as 2.4.1. Aortic Dissection
well as the ASI12 and had the advantage of being simpler Aortic dissection is the most common of the AAS. Aor-
to calculate. tic dissection occurs when there is an intimal tear that
The Cross-Sectional Area to Height Ratio allows the blood to pass through the tear and into the
Another approach to normalizing aortic size to height aortic media, splitting the intima in 2 longitudinally, cre-
was proposed by Svensson et al in 200213 in which they ating a dissection flap that divides the true lumen from
calculated a ratio of the cross-sectional area of the aorta a newly formed false lumen (Figure 6). The dissection
(cm) to the patient’s height (m). The initial studies used flap can propagate in an antegrade or retrograde fashion
a cross-sectional area to height ratio of >10 cm2/m and lead to a number of life-threatening complications,
as a threshold for intervention because of a signifi- including acute aortic regurgitation (AR), myocardial
cant increase in risk of adverse events; notably, in more ischemia, cardiac tamponade, acute stroke, or malperfu-
contemporary reports, this group has shown the sim- sion syndromes. The blood surging in the false lumen
Figure 6. Acute Aortic Syndromes.

In aortic dissection, a tear in the aortic
intima allows blood to penetrate the aortic
media, pushing the dissection flap into
the middle of the aorta, separating the
true from the false lumen. In intramural
hematoma, blood leaks into the aortic
media at low pressure, forming a thrombus
that pushes the outer wall of the aorta
outward, leaving a relatively normal
appearing aortic lumen. A penetrating
atherosclerotic ulcer allows blood to
enter the aortic media, but atherosclerotic
scarring of the aorta typically confines
the blood collection, often resulting in a
localized dissection or pseudoaneurysm.
Adapted with permission from Springer
Nature Customer Service Centre GmbH:
Springer Nature, Clough et al‚1 Copyright
2015.

Table 3. Classification of Aortic Dissection Chronicity Based 2.4.1.1. Definition

CLINICAL STATEMENTS
on the 2020 SVS/STS Reporting Standards Aortic dissection has traditionally been defined as
AND GUIDELINES
Chronicity Time From Onset of Symptoms “acute” during the first 2 weeks after symptom onset and
Hyperacute <24 h
“chronic” when beyond the second week. Investigators
from the International Registry of Acute Aortic Dissec-
Acute 1–14 d
tion (IRAD) proposed that aortic dissection be divided
Subacute 15–90 d
into 4 temporal types: hyperacute (<24 h), acute (2–7
Chronic >90 d d), subacute (8–30 d), and chronic (>30 d).2 The most
Adapted with permission from Springer Nature Customer Service Centre contemporary temporal classification system, proposed
GmbH: Springer Nature, Clough RE, et al.1 Copyright 2015. by the SVS and STS, similarly divides aortic dissection
STS indicates Society of Thoracic Surgeons; and SVS, Society for Vascular
Surgery.
into 4 temporal types, as shown in Table 3, to improve
prognostication and guide decision making about the
timing and types of potential intervention.
may rupture back through the intima into the true lumen, Acute aortic dissection of the ascending aorta is highly
creating a reentry tear. If the blood in the false lumen lethal in symptomatic patients left untreated, with an early
instead tears through the outer media and adventitia, mortality of 1% to 2% per hour after symptom onset.3 The
aortic rupture will result. The incidence of aortic dissec- mortality rate is increased among patients who present
tion is estimated to be 5 to 30 cases per million people with or develop complications of cardiac tamponade (with
per year, with men more commonly affected. Most dis- or without cardiogenic shock), acute myocardial ischemia
sections occur in those between the ages of 50 to 70 or infarction, stroke, or organ malperfusion.3 Patients
years, although patients with Marfan syndrome, BAV, with uncomplicated acute type B aortic dissection have
Loeys-Dietz syndrome, and vascular Ehlers-Danlos syn- a 30-day mortality rate of 10%. However, when patients
drome, present at younger ages. with acute type B aortic dissection develop complica-
Figure 7. Classification of Acute Aortic Dissection.

The DeBakey and Stanford classification systems are used most commonly. The DeBakey system offers greater anatomic detail, whereas the
Stanford system is simpler, essentially distinguishing those dissections that involve the ascending thoracic aorta from those that do not.

CLINICAL STATEMENTS
AND GUIDELINES
Figure 8. Anatomic Reporting of
Aortic Dissection Based on the 2020
SVS/STS Reporting Standards.
STS indicates Society of Thoracic
Surgeons; and SVS, Society for Vascular
Surgery. Reprinted from Lombardi et al.5
Copyright 2020, with permission from
Elsevier, Inc., the Society of Thoracic
Surgeons, and the Society for Vascular
Surgery.
tions, such as malperfusion or rupture, the mortality rate published an expert consensus document4 for the treat-
increases to 20% by day 2 and to 25% by day 30.3 ment of thoracic arch pathologies, in which they added
a third category called “non-A-non-B dissection,” to be
2.4.1.2. Classification used for patients whose proximal dissection flap begins
There are 2 commonly used anatomic classification sys- in the aortic arch.Most recently, in 2020, the SVS and the
tems for aortic dissection (Figure 7): the DeBakey sys- STS proposed an entirely new classification scheme that
tem and the Stanford system. defines the aortic dissection anatomy in more granular
The DeBakey system categorizes dissections into detail5: Dissections are defined anatomically according
types I, II, and III, based on the origin of the intimal tear to the location of intimal tears and the proximal and distal
and the extent of the dissection: extent of the dissection process (Figure 8).
● Type I: Dissection tear originates in the ascending AD indicates type A is used for any dissection with an
aorta and propagates distally to include the aortic entry tear in zone 0 and extends distally the zone denoted
arch and typically the descending aorta by the subscript D (eg, A9); B PD, type B is used for any
● Type II: Dissection tear is confined only to the dissection with an entry tear in zone 1 or beyond; the
ascending aorta proximal and distal extents of the dissection are denoted
● Type III: Dissection tear originates in the descend- by subscripts P and D, respectively (eg, B39). I D, when a
ing thoracic aorta and propagates most often dissection begins in zone 0 but the location of the entry
distally tear has not been identified, it will be considered “Inde-
○ Type IIIa: Dissection tear is confined only to the terminate”; it will be designated with an I and its distal
descending thoracic aorta extent denoted by the subscript D (eg, I9).
○ Type IIIb: Dissection tear originates in the
descending thoracic aorta and extends below 2.4.1.3. Malperfusion
the diaphragm Malperfusion syndrome occurs when there is end-organ
The Stanford classification system divides dissections ischemia related to inadequate perfusion of the aortic
into 2 categories according to whether the ascending branch vessels. The relationship of the true and false
aorta is involved or not, regardless of the site of origin: lumens in an aortic dissection has a critical role in main-
● Type A: All dissections involving the ascending taining stable perfusion of end-organs. Initially, the true
aorta, irrespective of the site of the intimal tear lumen collapses because of the loss of transmural pres-
● Type B: All dissections that do not involve the sure across the dissection flap and the subsequent elas-
ascending aorta (including dissections that involve tic recoil of the medial smooth muscle. Simultaneously,
the aortic arch but spare the ascending aorta) the false lumen expands immediately because of reduced
In 2019, the European Association for Cardio-Thoracic elastic recoil, depth of the dissection plane within the
Surgery and the European Society for Vascular Surgery media, and percentage of the wall circumference involved.

Any of the aortic branches are at risk for malperfusion as

CLINICAL STATEMENTS
the false lumen expands and compresses the true lumen

AND GUIDELINES
and can occur in multiple vascular beds simultaneously

as the dissection propagates distally. Dynamic obstruc-
tion occurs when the septum of the dissected intima pro-
lapses across into the ostia of a branch, usually during
systole, thereby not allowing adequate flow to perfuse
the vessel (Figure 9). The ostia itself remains anatomi-
cally undamaged. When the dissection tear extends into
the vessel proper and creates a stenosis or thrombosis in
the artery, static obstruction occurs (Figure 9).
2.4.2. Intramural Hematoma
IMH describes the presence of blood within the medial
layer of the aortic wall in the absence of an overt inti-
mal tear or patent false lumen. The blood may arise
from either rupture of the vasa vasorum causing bleed-
ing within the media7 or small intimal tears that are not
visualized on standard imaging examinations.8 IMH is
diagnosed by computed tomographic angiography (CTA),
magnetic resonance imaging (MRI), and echocardiogra-
phy by the presence of a circular or crescent-shaped
thickening of the aortic wall of >5 mm in the absence of
detectable blood flow9 (Figure 6). Of patients presenting
with suspected AAS, studies suggest that 5% to 25%
have IMH, a proportion that approaches 30% to 40% in
the Asian literature.8-11
The natural history of IMH is variable. Fewer than
10% of IMH cases resolve spontaneously, whereas 16%
to 47% progress to aortic dissection if the intimal layer

ruptures and creates an entry tear.7,12
2.4.3. Penetrating Atherosclerotic Ulcer
A PAU begins with an ulceration of an atherosclerotic
plaque, which leads to a focal disruption in the aortic
intima that allows blood to penetrate into the medial
layer and is often associated with an IMH of variable
size.10 PAUs most often appear in the middle or distal
descending thoracic aorta, less frequently in the aortic
arch and abdominal aorta, and rarely in the ascending
aorta.8,10 PAUs can vary in size, and often multiple PAUs
are present.10 The true incidence is unknown but is esti-
mated to account for 2% to 7% of all cases of AAS.10
Typically, patients with PAU are older (>70 years of age)
than those with classic aortic dissection and present
more often with extensive and diffuse atherosclerotic
disease involving both the aorta and coronary arteries.10
Additional common comorbidities include hypertension, Figure 9. Mechanisms of Dynamic and Static Obstruction in
Aortic Dissection.
tobacco use, chronic obstructive pulmonary disease, and
(A) Static obstruction occurs when the dissection flap extends from the
renal insufficiency. PAU can occur in younger patients aortic lumen into the ostium of the affected branch vessel, leading to
but often in the setting of a connective tissue disorder, localized thrombosis of the branch false lumen that narrows or colludes
and men are more commonly affected than women.8The the branch true lumen and, consequently, impairs distal branch perfusion.
natural history of PAU is not well defined, as they can (B) Dynamic obstruction occurs when the false lumen becomes
persistently pressurized and compresses the true lumen, in turn pushing
remain stable, enlarge, or progress to either IMH, dis-
the dissection flap up against the ostium of the affected branch vessel,
section, pseudoaneurysm, or aortic rupture.8 The risk significantly reducing or occluding its flow. (C) Sometimes, a branch vessel
of rupture has been reported to be up to 40%.13 The can suffer from both static and dynamic obstruction at the same time.
optimal management strategy must be individualized, Adapted with permission from Grewal et al.6 Copyright 2021, Elsevier, Inc.

CLINICAL STATEMENTS
AND GUIDELINES
Figure 10. Classification of Thoracoabdominal Aortic Aneurysms.

The classification of thoracoabdominal aortic aneurysms according to extent of aortic involvement as originally proposed by Crawford is as
follows3: Extent I, below the left subclavian to above the celiac axis or opposite the superior mesenteric and above the renal arteries; Extent II,
below the left subclavian and including the infrarenal abdominal aorta to the level of the aortic bifurcation; Extent III, below T6 intercostal space,
tapering to just above the infrarenal abdominal aorta to the iliac bifurcation; and Extent IV, below T12, tapering to above the iliac bifurcation. Safi
et al1 proposed expanding the classification with the addition of Extent V, below T6, tapering to just above the renal arteries.
considering the clinical presentation, the imaging fea- blood flow outside the graft and within the aneurysm
tures of the PAU, and the patient’s comorbidities. sac, preventing its complete thrombosis. Consequently,
patients with endografts require lifelong surveillance imag-
ing to monitor for the appearance of endoleaks.1
2.5. Classification of Thoracoabdominal Aortic
Aneurysm (TAAA)
When descending thoracic aortic aneurysms (TAA) 3. IMAGING AND MEASUREMENTS
extend into the abdominal aorta, they are referred to as 3.1. Aortic Imaging Techniques to Determine
thoracoabdominal aortic aneurysms (TAAA). The Craw- Presence and Progression of Aortic Disease
ford classification of TAAA, later modified by Safi et al1
Recommendations for Aortic Imaging Techniques to Determine
(Figure 10), not only describes the extent of an aneu- Presence and Progression of Aortic Disease
rysm but also may predict the morbidity and mortality Referenced studies that support the recommendations are
summarized in the Online Data Supplement.
associated with aneurysm repair.2
COR LOE Recommendations
1. In patients with known or suspected aortic
2.6. Classification of Endoleaks disease, aortic diameters should be mea-
sured at reproducible anatomic landmarks
Endovascular stent-grafting is widely used in the repair of perpendicular to axis of blood flow, and these
aortic aneurysms. One of the limitations of endografting is 1 B-NR measurement methods should be reported
in a clear and consistent manner. In cases of
the occurrence of endoleaks, either early or late following asymmetric or oval contour, the longest diam-
the procedure. There are 5 types of endoleaks, as detailed eter and its perpendicular diameter should be
in Figure 11. An endoleak results in the persistence of reported.3,4

Recommendations for Aortic Imaging Techniques to Determine Recommendation-Specific Supportive Text

CLINICAL STATEMENTS
Presence and Progression of Aortic Disease (Continued )

1. Measurements should be obtained perpendicular
AND GUIDELINES

to the long axis of the aorta at specified segmen-
2. In patients with known or suspected aortic tal locations (Figure 12), with measurements also
disease, episodic and cumulative ionizing radi-
1 C-LD
ation doses should be kept as low as feasible taken at the locations of any abnormalities. If a 3D
while maintaining diagnostic image quality.5-7 data set has been acquired, dedicated multiplanar
3. In patients with known or suspected aortic reformats orthogonal to aortic flow axis should
disease, when performing CT or MR imaging, be created at each level of measurement. This
it is recommended that the root and ascending
approach provides structured, repeatable measure-
aortic diameters be measured from inner-edge
to inner-edge, using an electrocardiographic- ment reporting on serial imaging and avoids oblique
1 C-EO
synchronized technique. If there are aortic imaging that may overestimate the aortic diameter
wall abnormalities, such as atherosclerosis or
at levels of greater curvature and tortuosity.3,4
discrete wall thickening (more common in the
distal aorta), the outer-edge to outer-edge 2. The cancer risk associated with CT scans remains
diameter should be reported (Table 4). a controversial issue; however, the risk is generally
4. In patients with known or suspected aortic agreed to be greatest early in life and substantially
disease, the aortic root diameter should be attenuated later in life.5,6 Consideration of the indi-
recorded as maximum sinus to sinus mea-
surement. In the setting of known asymmetry,
cation for aortic imaging, optimization of the tube
1 C-EO
multiple measurements should be reported, settings for CT protocols, and use of alternative
and both short- and long-axis images of the modalities such as MRI are all valid approaches to
root should be obtained to avoid underestima-
tion of the diameter.
mitigate patient radiation exposure.7
3. On CT and MRI, the root diameter can be mea-
disease, it is reasonable that a dilated root or sured from the commissure to the opposite sinus,
2a C-LD ascending aorta be indexed to patient height or from sinus to sinus, which results in larger
or BSA in the report, to aid in clinical risk dimensions (Figure 12).13 Measuring from sinus
assessment.8-11
to sinus and from inner-edge to inner-edge on
disease, when performing echocardiography,
CT and MRI has shown good correlation with TTE
2a C-EO it is reasonable to measure the aorta from for measurements of the root and ascending seg-
leading-edge to leading-edge, perpendicular ments,14 as well as improved confidence in the
to the axis of blood flow.

determination of aortic root margins on MRI and
Using inner-edge to inner-edge measure- lower interobserver and intraobserver variability.15
2b C-EO ments may also be considered, particularly on
short-axis imaging. Measurement of graft material (eg, interposed
surgical or endostent) may likewise include an
inner-edge to inner-edge measurement for deter-
mination of the functional lumen and potential use
Synopsis in extension treatment planning. The use of elec-
Optimized depiction of aortic anatomy and pathology trocardiographic-gated images decreases motion
requires dedicated aortic imaging protocols. Computed artifact and improves edge depiction in aortic root
tomography (CT), magnetic resonance imaging (MRI), imaging, with diminished measurement variability.16
transthoracic echocardiography (TTE) and transesoph- If there are aortic wall changes (eg, atherosclero-
ageal echocardiography (TEE), and abdominal aortic sis, mural thrombus), as is more commonly noted in
ultrasound all have important roles in these evaluations the arch and distal aorta, or discrete wall thicken-
(Table 5). Selection of an imaging modality may be based ing (eg, aortitis or IMH), the outer margins of the
on patient-specific factors, including hemodynamic sta- abnormal segments are measured.
bility, contrast allergy, renal function, and patient toler- 4. The shape of the aortic root can be asymmetric, and
ance (eg, given relatively longer examination times and the difference between the minimum (short-axis)
the confined space associated with MRI, occasionally and maximum (long-axis) root diameters can be sig-
requiring sedation). The institutional availability of an nificant, particularly in those with bicuspid valves.17
imaging modality or an expert imaging physician may To avoid underestimation, multiple measurements
also direct modality selection. The ubiquity of CT scan- should be reported, with either each of the sinus-to-
ners, combined with rapid acquisition of intuitive, high- sinus diameters or both short- and long-axis diam-
resolution 3-dimensional (3D) imaging data sets, has led eters, to avoid underestimation of the true root size.
to the wide adoption of this modality for the assessment 5. The cross-sectional aortic area to patient height
of suspected aortic pathology and for periprocedural ratio has been shown to be associated with risk
vascular evaluation, in most cases supplanting diagnostic of aortic dissection and death in patients with tri-
catheter angiography.12 cuspid or bicuspid valves9,10 (see Section 2.3.1,

CLINICAL STATEMENTS
AND GUIDELINES
Figure 11. Classification of Endoleak Types.

Endoleaks are classified by 5 types: Type Ia, proximal attachment site endoleak; Type Ib, distal attachment site endoleak; Type II, backfilling
of the aneurysm sac through branch vessels of the aorta; Type III, graft defect or component misalignment; Type IV, leakage through the graft
wall attributable to endograft porosity; and Type V, caused by “endotension,” possibly resulting from aortic pressure transmitted through the graft/
thrombus to the aneurysm sac. Adapted from Rokosh et al.2 Copyright 2021, with permission from Elsevier, Inc., and the Society for Vascular
Surgery.
“Normalizing Aortic Root and Ascending Aortic largest diameter.18,19 These data led to the deter-
Diameters for Body Size”), and both ASI and AHI mination of normal limits adjusted for age, sex, and
have been shown to predict risk of adverse events body size20 and provided insight regarding the prev-
(rupture, dissection, or death).11 alence and prognostic importance of aortic dilation.
6. There is a wealth of historical data regarding using Additionally, measuring from leading-edge to lead-
TTE to measure the aortic root (at end-diastole) ing-edge on TTE has shown good correlation with
from the leading-edge of the anterior wall to the inner-edge to inner-edge measurements obtained
leading-edge of the posterior wall, identifying the on CT and MRI.14 The method of inner-edge to

Table 4. Essential Elements of CT and MRI Aortic Imaging There is a wealth of historical data regarding using TTE
CLINICAL STATEMENTS
Reports to measure the aortic root (at end-diastole) from the

AND GUIDELINES
1. Maximum aortic diameter at each level of dilation, perpendicular to the leading-edge of the anterior wall to the leading-edge of
axis of blood flow. In cases of asymmetric or oval contour, the longest the posterior wall, thus identifying the largest diameter.2,3
diameter and its perpendicular diameter should be reported. Standard
measurement levels may be included, even when normal.
These data allowed for the creation of normal limits
2. Wall changes suggestive of atherosclerosis, diffuse thickening (eg, aor- adjusted for age, sex, and body size4 and provided insight
titis), or mural thrombus. regarding the prevalence and prognostic importance of
3. Evidence of luminal stenosis/occlusion, including location, severity, and
length.
aortic dilation.
4. Findings suggestive of acute aortic syndrome (eg, communicating dis- On CT and MRI, the root diameter can be measured
section, intramural hematoma, penetrating atherosclerotic ulcer, focal from the commissure to the opposite sinus, or from sinus
intimal tear), including proximal/distal extension (Figure 7), suspected
entry tear site (if visible), and complications (eg, active contrast extrava-
to sinus, which results in larger dimensions (Figure 13).5
sation, rupture, contained rupture, rupture including periaortic hemor- Measuring from sinus-to-sinus and from inner-edge to
rhage, pericardial and pleural fluid, mediastinal stranding). inner-edge on CT and MRI has shown good correlation
5. Extension of aortic disease process (acute or chronic) into branch
vessels, findings suggestive of end-organ injury, and suspected
with TTE for measurements of the root and ascending
malperfusion. segments,6 as well as improved confidence in the delin-
6. Direct comparison with previous examinations should be detailed to eation of aortic root margins on MRI and lower interob-
identify pertinent changes.
7. Presence and extent of repair (eg, interposition graft, endovascular
server and interobserver variability.7
stent graft), as well as any evidence of complication. Although aortic dilation as measured by diameter is a
8. Impression regarding disease classification (eg, acute aortic syndrome, well-known risk factor for the occurrence of aortic dis-
aneurysm/pseudoaneurysm, luminal stenosis, atherosclerotic aortic
disease).
section and rupture,8 most dissections occur in aortas
9. Relevant details regarding method of image acquisition (eg, use of with diameters that do not meet the threshold for preven-
electrocardiographic-gating and phase of acquisition) and measure- tive surgery.9 This has led investigators to search for bet-
ment (eg, axial versus double oblique, inner-edge versus outer-edge)
should be included.
ter metrics for risk stratification and treatment guidance.
For instance, research has shown that ascending aortic
CT indicates computed tomography; and MRI, magnetic resonance imaging.
area indexed to height is associated with aortic dissec-
tion and adverse outcomes in patients with tricuspid or
inner-edge measurement on TTE images may also bicuspid valves.10,11 Male sex, age, height, weight, and
be considered, with some experienced investiga- the presence of traditional cardiovascular risk factors
tors showing excellent measurement agreement.15 have also been found to correlate with increased aortic
size in large population-based studies.12 Aortic length is

known to increase over time; spurred by this fact, and
3.2. Conventions of Measurements by the observation that intimal entry tears run in a trans-
Reproducible and accurate measurements of the aorta verse direction, researchers have found that excessive
are critical for characterizing aortic disease and guiding elongation of the ascending aorta may be predictive of
treatment decisions. Measurements should be obtained dissection and thus represents a potentially relevant
perpendicular to the long axis of the aorta at specified measurement.13
segmental locations (Figure 13),1 with measurements Measurements of the arch and further distal seg-
also taken at the location of any abnormality. Unfortu- ments should also be performed perpendicular to the
nately, there is no widely accepted standard for aortic aortic axis, with care taken to avoid oblique imaging that
diameter measurements (eg, inner-edge to inner-edge, may overestimate the aortic diameter at levels of greater
outer-edge to outer-edge) across imaging modalities. curvature and tortuosity. In the setting of wall changes
Table 5. Diagnostic Performance of Aortic Imaging Modalities
Parameter CT MRI TTE TEE US

Availability +++ ++ +++ ++ +++
Portability - - +++ +++ +++
Speed of acquisition +++ + ++ ++ ++
Spatial resolution +++ ++ ++ +++ ++
Temporal resolution + ++ +++ +++ +++
Three-dimensional data set +++ ++ + + +
Arch branch vessel evaluation +++ +++ ++ + NA
Evaluation of valve and ventricular function + ++ +++ +++ NA
CT indicates computed tomography; MRI, magnetic resonance imaging; NA, not applicable; TEE, transesophageal echocar-
diography; TTE, transthoracic echocardiography; US, abdominal aortic ultrasound; +++ excellent results; ++ good results; +
fair results; and -, not available.

of the root and ascending aorta,1 significantly increas-
CLINICAL STATEMENTS
ing the precision of measurements and diagnostic confi-
AND GUIDELINES
dence. When necessary, CT can be performed without the
use of iodinated contrast, and such noncontrast imaging
can still accurately provide diameter assessment of aor-
tic aneurysms that can suffice for surveillance of patients
who cannot tolerate or cooperate with MRI, although aor-
tic wall delineation may be challenging in some instances
(eg, at the aortic root level). The use of iodinated intra-
venous contrast allows for delineation between aortic
lumen and wall and generally improves assessment of
wall changes. In some instances, the potential concern of
patient contrast allergy or renal toxicity may be a consid-
eration. However, according to recent consensus state-
ments from the American College of Radiology and the
National Kidney Foundation,2 the risk of acute kidney
injury developing in patients with impaired renal function
after exposure to intravenous iodinated contrast media
has likely been overestimated given the difficulty distin-
guishing coincident from contrast-induced nephropathy.
CT has a very high sensitivity and specificity for acute
aortic syndromes (AAS, aortic dissection, IMH, PAU)3 and
traumatic aortic injuries. Moreover, CT can identify con-
comitant coronary involvement,4 branch vessel involve-
ment, and hemopericardium, and may aid in identification
of dissection entry tears. In patients whose CT is nega-
tive for AAS, the images may provide insight regarding
other causes of the presenting chest pain.5 When imag-
ing patients with a suspected AAS, a noncontrast series
Figure 12. Aortic Imaging Techniques to Determine the

Presence and Progression of Aortic Disease. of images is typically obtained first, to better distinguish
(A) Schematic shows the leading-edge to leading-edge measurement IMH, if present, from other causes of aortic wall thicken-
technique used in echocardiography, from left to right: measurement ing. Then, a series of arterial phase contrast-enhanced
of the aortic root (sinuses of Valsalva), sinotubular junction, and
proximal tubular ascending aorta. (B) Inner-wall to inner-wall
images is obtained with thin slice to allow for recon-
measurements of the aortic root used in MRI and CT. In addition, structions (computed tomographic angiography [CTA]),
a consistent approach to measuring all 3 sinuses with MRI and extending from the thoracic inlet to the level of the femo-
CT is necessary. The sinus-to-commissure and sinus-to-sinus ral arteries, to define the full extent of any dissection and
measurements can both be used, but consistency is necessary for thereby guide therapy. For consistency in this document,
interval surveillance. (C) Standard measurement locations for MRI and
CT with the inner-wall to inner-wall technique. Adapted from Borger et
CT is used to refer to computed tomography modality
al.21 Copyright 2018, with permission from Elsevier, Inc. CT indicates broadly, with specific imaging techniques chosen depen-
computed tomography; and MRI, magnetic resonance imaging. dent on a given clinical indication and patient history.
*Leading-edge to leading-edge. †Inner-wall to inner-wall.
3.2.2. Magnetic Resonance Imaging
MRI provides coverage of the entire aorta and branch
(eg, discrete thickening from atherosclerosis, aortitis, vessels, can characterize aortic wall changes in the set-
IMH, or other processes), the abnormal wall should be ting of inflammation1 and AAS, and offers physiologic
measured from outer-edge to outer-edge. To assess assessment of ventricular and valve function plus flow
abdominal aortic dimensions, ultrasonographic images quantification. MRI uses no ionizing radiation and can
may be obtained in a dedicated examination or as part of often be performed without intravenous contrast. MRI is
a surface echocardiographic examination. Several stud- therefore often a primary option for assessing congenital
ies have shown that the volume of an AAA may progress aortic abnormalities and is well-suited for serial imaging in
despite a stable diameter.14,15 younger patients. The use of electrocardiographic-gated
imaging decreases motion artifact of the aortic root2 and
3.2.1. Computed Tomography of 3D datasets, critical for achieving precise, repeat-
CT can image the entire aorta and its branches with high able measurements.3 Limitations of MRI include spatial
spatial resolution and fast acquisition. The use of electro- resolution that, although good, is typically inferior to that
cardiographic-gated technique decreases motion artifact of CT, as well as the appearance of artifacts in patients

CLINICAL STATEMENTS
AND GUIDELINES
Figure 13. Reformatted CT Image

Orthogonal to the Aortic Root at the
Level of the Sinuses of Valsalva.
The root diameter can be measured
from sinus-to-sinus (S-S) or sinus-to-
commissure (S-C). The aortic root area
(A) can also be measured. CT indicates
computed tomography; and ROI, region-
of-interest.
with indwelling metallic material or devices. Additionally, sels and the proximal descending aorta and can aid in
MRI is not as widely available as CT for aortic imaging, diagnosis of coarctation of the aorta (CoA) and patent
has a longer acquisition time, and the ability to monitor ductus arteriosus. TTE is portable and can be performed
and treat unstable patients in the scanner is limited. This at the bedside with a high spatial and temporal resolution.
modality is therefore less commonly used in patients It can be useful in the evaluation of patients with AAS to
with suspected acute aortic pathology,4 especially when detect complications, including aortic valve regurgitation,
patients are unstable. Various MRI sequences are avail- left ventricular dysfunction, and cardiac tamponade. TTE
able for aortic depiction, including magnetic resonance is useful in the longitudinal surveillance of aortic root and
angiography (MRA), which involves volumetric acquisi- ascending aortic dilation, provided those aortic segments
tion of aortic anatomy, with slice thickness allowing for are well visualized.
reconstruction of images in multiple planes. Intravenous Transesophageal Echocardiography (TEE)
gadolinium-based contrast media are often used in MRA, TEE provides high-resolution images of most of the
although there is a very small risk of inducing nephro- thoracic aorta, apart from a short segment of the distal
genic systemic fibrosis in patients with underlying kidney ascending aorta just proximal to the innominate artery,
disease, a risk that is particularly low with group II gad- attributable to acoustic shadowing from the trachea. TEE
olinium-based contrast agents.5,6 Additional sequences is also very useful in detailing aortic valve anatomy and
are often used for aortic anatomic depiction that do not function. TEE is particularly useful in the intraoperative
require intravenous contrast media, such as cine gradient evaluation of patients with AAS in guiding both operative
echo bright blood and spin echo dark blood sequences. and endovascular repair strategies and the assessment
For consistency in this document, we use MRI to refer of true and false lumen flows before and immediately
to the modality of magnetic resonance imaging defined after aortic repair.1,2
broadly, which potentially includes many sequences that
are often combined in complementary manner within an 3.2.4. Intravascular Ultrasound
imaging protocol. Intravascular ultrasound is an endovascular technology
designed to provide high-resolution intraluminal imaging
3.2.3. Echocardiography of localized arterial and venous disease.1 Intravascular
Transthoracic Echocardiography (TTE) ultrasound is particularly useful in guiding the endovascu-
TTE is the most common imaging modality used in the lar management of complex pathologies of the thoracic
initial nonemergency assessment of the thoracic aorta.1,2 and abdominal aorta, because it reveals aortic size, tor-
TTE is particularly useful in imaging the aortic root and tuosity, plaque burden, calcification, branch vessel ostia,
ascending aorta and in delineating aortic valve anatomy and intravascular filling defects (eg, thrombus, dissection
and function. Although not ideal for imaging of the aortic flap), in addition to permitting landing zone assessment.1
arch, TTE often does visualize the aortic arch branch ves- Such intravascular ultrasound imaging data may help

to identify patients for whom endovascular treatment is Synopsis
CLINICAL STATEMENTS
high-risk or contraindicated. Intravascular ultrasound is
Evidence-based standards for medical and surgical con-
AND GUIDELINES
especially useful in the setting of aortic dissection2-4 to
ditions recognize the critical relationship among both
distinguish true and false lumen anatomy and thereby
hospital and surgeon case volumes and patient out-
guide endovascular or open repair. Intravascular ultra-
comes. Clinical excellence is further enhanced by collab-
sound may be used to guide deployment of endovascular
orative, multispecialty teams to foster the best treatment
stents and, during final assessment, to reduce the vol-
of patients, especially for complex presentations with
ume of iodinated contrast used.5 Importantly, intravascu-
multiorgan threats. Although there is no agreed on defi-
lar ultrasound requires an operator who is familiar with
nition of a Multidisciplinary Aortic Team, an appropriate
both the acquisition and interpretation of images.
framework might be: A specialized hospital team with
an exceptionally high concentration of expertise in the
3.2.5. Abdominal Ultrasound
evaluation and management of aortic disease, in which
Vascular ultrasound is an effective and rapid imaging
care is delivered in a comprehensive, multidisciplinary
modality and is the recommended diagnostic tool in
manner.7 The concept of comprehensive heart valve
screening for and surveillance of AAA.1-3 The ultrasonic
centers was formally codified in the “2020 ACC/AHA
criterion for AAA is a diameter >3.0 cm, using primar-
Guideline for the Management of the Patient With Val-
ily the outer-edge to outer-edge measurement conven-
vular Heart Disease,”8 which emphasized the numerous
tion in the anterior-posterior or transverse view.4-6 The
essential components of such centers, ranging from phy-
sensitivity of ultrasound to detect the presence of an
sician expertise, experience, and technical skill to data
aneurysm approaches 100%,7 although interobserver
collection, research, and education, to institutional facili-
variability exists, and successful imaging can be limited
ties and resources. Although the specific components of
by obesity and superimposed bowel gas.8
such teams may differ from center to center, the most
Using B-mode imaging, color Doppler, and spectral
common features that distinguish Multidisciplinary Aortic
waveform analysis, a comprehensive ultrasound evalu-
Teams include: Having cardiac surgical, vascular surgical,
ation of the abdominal aorta can quickly detect other
and endovascular specialists with extensive experience
aortic pathologies, such as plaque or mobile atheroma
managing complex aortic disease at a center with a high
formation, arterial stenoses, mural thrombus, inflamma-
volume of aortic interventions; having imaging specialists
tion, dissection, pseudoaneurysm, contained rupture, and
with expertise in aortic disease to perform and interpret
aortocaval fistulae, and these findings may prompt the
CT, MRI, and echocardiography; anesthesiologists expe-

need for further imaging with CT or MRI. Abdominal ultra-
rienced in the management of acute aortic disease and
sound can also be used for surveillance of patients who
cerebrospinal fluid drainage; and an intensive care unit
have undergone endovascular repair of AAA (EVAR);
(ICU) experienced in the management of acute aortic
it can detect aneurysm sac expansion, which may indi-
disease.
cate the presence of an endoleak (Figure 11), defined
as abnormal flow outside of the aortic endograft, a find-
ing that typically warrants confirmation by CT. The use
Recommendation-Specific Supportive Text
of contrast-enhanced color duplex ultrasound has shown
promising results in enhanced sensitivity in detection of 1. In cardiovascular care, we have long recognized
endoleaks,9 although its use requires ongoing study. the critical value of collaborative multidisciplinary
expertise in cardiac transplantation and mechani-
cal circulatory support conducted only at centers
4. MULTIDISCIPLINARY AORTIC TEAMS of excellence. More recently, we have seen the
Recommendations for Multidisciplinary Aortic Teams rise in multidisciplinary heart teams focused on
the care of patients with complex coronary artery
disease and patients with complex heart valve
1. For patients with acute aortic disease that
requires urgent repair, a multidisciplinary team disease; indeed, the important role of multidisci-
1 C-EO
should determine the most suitable interven- plinary heart valve teams was emphasized in the
tion. “2020 ACC/AHA Guideline for the Management
2. For patients who are asymptomatic with of the Patient With Valvular Heart Disease.”8 There
extensive aortic disease, or who may benefit
from complex open and endovascular aortic
is ample evidence that patients with complex aor-
repairs, or with multiple comorbidities for tic disease may similarly benefit from treatment
2a C-LD
whom intervention is considered, referral to by such multidisciplinary teams.6 Andersen et al1
a high-volume center (performing at least
30-40 aortic procedures annually) with expe-
compared the outcomes of patients with acute
rienced surgeons in a Multidisciplinary Aortic type A aortic dissection undergoing open surgical
Team is reasonable to optimize treatment repair before and after implementation of a mul-
outcomes.1-6
tidisciplinary thoracic aortic surgery program and

found that operative mortality declined dramati- analyzed >13 000 elective aortic root and aortic
CLINICAL STATEMENTS
cally after implementation of the multidisciplinary valve-ascending aortic procedures performed at

AND GUIDELINES
team and that the significant mortality advantage 741 North American hospitals from 2004 to 2007.
persisted over a 5-year follow-up (P=0.002). They found a negative association between the
Likewise, in a report from England,2 hospitals with hospital volume and the adjusted odds ratio (OR)
multidisciplinary thoracic aortic programs reported for mortality (P<0.001), particularly at a hospital
significant reductions in mortality compared with volume of <30 to 40 procedures annually (Figure
hospitals without such programs. 14). The inverse relationship between center
2. In a study of 230 736 Medicare beneficiaries case volume and mortality was shown again
undergoing AAA repair between 2001 and 2006, in a more contemporary series by Mori et al9 of
in which hospital procedural volume for both open >53 000 proximal thoracic aortic surgeries in the
and endovascular repair was divided into quin- United States from 2011 to 2016 in which the
tiles, the adjusted mortality decreased as hospital risk of operative mortality decreased significantly
volume increased, by quintile, especially among when the annual center volume exceeded 20 to
the group undergoing open surgical repair.3 The 25 cases (only 116 US centers performed >20
benefits of high case volume on surgical outcome cases/y), and decreased significantly further still
apply similarly to patients with TAA. Hughes et al4 at an annual center volume of >50 cases (only 24
Figure 14. Observed Relationship

Between Annual Institutional Case
Volume and Risk-Adjusted Odds
Ratio for Operative Mortality ±2
Standard Deviations as Assessed
With Regression Analysis.
The odds ratio for operative mortality
decreased as institutional case volume
increased. Adapted from Hughes et al.4
Copyright 2013, with permission from
Elsevier Inc.

CLINICAL STATEMENTS
AND GUIDELINES
Figure 15. Predicted Risk of Mortality
Derived From the Logistic Regression
Model Without Center Case Volume
as a Covariate.
Actual mortality and the ratio of actual
mortality to predicted mortality (A/P ratio,
the risk-adjusted mortality rate) are also
shown. A similar predicted risk of mortality
across the case volume strata and a
decrease in the actual mortality at higher
center case volume are seen. Reprinted
from Mori et al.9 Copyright 2018, with
permission from Elsevier Inc.
US centers performed >50 cases/y) (Figure 15). endovascular aortic repairs be performed by expe-
Perhaps the most consistent correlation between rienced operators in centers with Multidisciplinary
case volume and mortality rate is among patients Aortic Teams.
with acute aortic dissection. In a retrospective
review of 232 patients with acute type A aortic
dissection who underwent urgent surgery in a 5. SHARED DECISION-MAKING
single center in the United Kingdom, the 30-day Recommendations for Shared Decision-Making
mortality rate was significantly lower among those
operated on by a surgeon with aortic expertise
1. In patients with aortic disease, shared deci-
versus a nonaortic expert, at 10% versus 26%, sion-making is recommended when determin-
respectively (P=0.02). Moreover, aortic special- ing the appropriate thresholds for intervention,
ists performed aortic root procedures significantly 1 C-LD deciding on the type of surgical repair, choos-
ing between open surgical versus endovascu-
more often (43.0% versus 17.3%; P=0.001), lar approaches; and in medical management
and their cross-clamp times were significantly and surveillance.1-6
shorter.5 Finally, Umana-Pizano et al10 found that 2. In patients with aortic disease who are contem-
the mortality rate of acute type A aortic dissection plating pregnancy or who are pregnant, shared
repair was 14% versus 24% for high-volume and 1 C-EO
decision-making is recommended when con-
sidering the cardiovascular risks of pregnancy,
low-volume surgeons, respectively. Clearly not all the diameter thresholds for prophylactic aortic
patients with thoracic aortic disease (TAD) can be surgery, and the mode of delivery.
treated by Multidisciplinary Aortic Teams, espe-
cially in the setting of AAS. Nevertheless, when
patients are referred for elective aortic interven- Synopsis
tion, especially at aortic diameter thresholds that Shared decision-making is increasingly used in patient-
are borderline, the lower surgical mortality rate
centered care as advocated by the National Academy of
with expert aortic surgeons at high-volume cen-
Medicine.7 Although no randomized trials have evaluated
ters may justify early aortic repair. Similarly, when
aortic procedures are relatively new or complex, the value and effectiveness of shared decision-making,
the best outcomes are likely to be at centers with multiple position papers advocate strongly for the incor-
high-volume operators who have experience with poration of shared decision-making in the care of patients
such novel techniques. Consequently, throughout with thoracic and AAAs.2-5 Decision aids have been devel-
this guideline is a number of recommendations in oped for shared decision-making in patients with AAAs
which it is specified that certain open surgical or to help improve the patient understanding of the disease

and treatment options.1 Shared decision-making is espe- Table 6. Cause of TAA

CLINICAL STATEMENTS
cially useful when considering the diameter thresholds HTAD (see Table 7): syndromic
AND GUIDELINES
for and the timing of intervention in addition to having an Marfan syndrome

important role in considering the risks of pregnancy in Loeys-Dietz syndrome
Vascular Ehlers-Danlos syndrome
patients with underlying aortic disease. Smooth muscle dysfunction syndrome
Others: attributable to pathogenic variants in FLNA, BGN, LOX
Recommendation-Specific Supportive Text HTAD (see Table 7): nonsyndromic

ACTA2, MYH11, PRKG1, MYLK, and others
1. Shared decision-making is an active process in Familial thoracic aortic aneurysm without identified pathogenic variants
in a known gene for HTAD
which patients and families are encouraged to
Congenital conditions
share their values and preferences regarding qual-
Bicuspid aortic valve
ity of life, goals of care, and desired procedural Turner syndrome
outcomes. Formally recognizing those prefer- Coarctation of the aorta
Complex congenital heart defects (tetralogy of Fallot, transposition of
ences helps physicians to better frame the risks
the great vessels, truncus arteriosus)
and benefits of intervention versus conservative
Hypertension
management. Actively involving patients in the
decision-making process is especially important Atherosclerosis
in situations in which there is clinical equipoise, Degenerative

such as: an aortic aneurysm with a diameter at the Previous aortic dissection
borderline of the threshold for repair; performing Inflammatory aortitis
valve-sparing root repair rather than valved-conduit Giant cell arteritis
aortic root replacement; performing thoracic endo- Takayasu arteritis
Behçet disease
vascular aortic repair (TEVAR) in a patient with Immunoglobulin G4-related disease, antineutrophil cytoplasmic anti-
an uncomplicated type B aortic dissection who is body-related, sarcoidosis
at increased risk of complications; or treating an Infectious aortitis
AAA with open surgical versus endovascular repair. Bacterial, fungal, syphilitic
Shared decision-making may be used for nonin- Previous traumatic aortic injury
terventional issues as well, such as the choice of HTAD indicates heritable thoracic aortic diseases; and TAA, thoracic aortic
medical therapies or the imaging modality used for
aneurysms.
surveillance.
2. Shared decision-making has an important role
in pregnancy among those with aortic disease Hypertension, smoking, hypercholesterolemia, and heri-
to determine whether to consider conception, table genetic variants are risk factors for TAA disease.
an appropriate diameter threshold for prophy- Patients with TAA have a modestly increased incidence
lactic aortic repair, and the mode of delivery. This of AAA4 and cerebral aneurysms.5
has particular relevance in patients with Marfan Causes of TAA include heritable disorders, congeni-
syndrome and Loeys-Dietz syndrome, and other tal conditions, multifactorial degenerative conditions,
heritable aortic disorders who are planning a previous aortic dissection, inflammatory diseases, and
pregnancy. infectious diseases (Table 6). Aneurysms of the aortic
root and ascending thoracic aorta tend to have a heri-
table influence and present at younger ages, whereas
6. ANEURYSMS aneurysms of the descending thoracic aorta tend to
be degenerative and present at older ages.6 Moreover,
6.1. Thoracic Aortic Aneurysm (TAA) Causes aneurysms of the aortic root and ascending thoracic
TAAs occur in 5 to 10 per 100 000 person years.1 The aorta are also commonly associated with BAV, although
natural history and treatment vary depending on the the genetic basis of BAV and why some but not all
cause and location of the TAA. The size of a given seg- patients have a concomitant aortopathy are not well
ment of the thoracic aorta is influenced by age, sex, understood. Finally, many aneurysms of the root and
height, and body size.2 Aortic z-scores and other diam- ascending thoracic aorta are sporadic and idiopathic.
eter indexing methods (see Section 2.3, “Definitions of Because the management of patients with aneurysms
Dilation and Aneurysm of the Aortic Root and Ascend- of the aortic root and ascending thoracic aorta may dif-
ing Thoracic Aorta”) may assist with risk assessment.3 Of fer depending on the underlying cause or family history,
all TAA, aneurysms of the aortic root, ascending aorta, the recommendations for medical and surgical therapy
or both are most common (∼60%), followed by those are grouped accordingly in the document, as shown in
of the descending aorta (∼30%) and arch (<10%). Figure 16.

CLINICAL STATEMENTS
AND GUIDELINES
Figure 16. Recommendations for Management of Aneurysms of the Aortic Root and Ascending Aorta According to Known
Causative Factors.
Table 7.
11

Table 7.
CLINICAL STATEMENTS
AND GUIDELINES
Figure 17. Evaluation and Genetic Testing Protocol for Patients With TAD.
Genetic testing is recommended for individuals with syndromic features, family history of TAD, and/or early age of disease onset. Thoracic aortic imaging is
recommended for first-degree relatives of all individuals with TAD, regardless of age of onset, to detect asymptomatic aneurysms. Positive genetic testing
should trigger gene-based management and cascade testing of at-risk relatives. When testing is negative or reveals variants of unknown significance,
first-degree relatives should undergo screening aortic imaging. Modified with permission from Milewicz et al.6 Copyright 2021, Minerva Medica. Blue (+)
indicates positive; green (–), negative; LDS, Loeys-Dietz syndrome; MFS, Marfan syndrome; TAAD, thoracic aortic aneurysm and dissection; TAD, thoracic
aortic disease; vEDS, vascular Ehlers-Danlos syndrome; and VUS, variants of unknown significance. *Aneurysms are typically asymptomatic.

Table 8. Risk Factors for Familial TAD atherosclerotic cause. Although sometimes referred to
CLINICAL STATEMENTS
TAD and syndromic features of Marfan syndrome, Loeys-Dietz syndrome,
as atherosclerotic aneurysms, more often aneurysms
AND GUIDELINES
or vascular Ehlers-Danlos syndrome of descending thoracic aorta (not related to connective
TAD presenting at age <60 y tissue disorders) are referred to as “degenerative.” The
A family history of either TAD or peripheral/intracranial aneurysms in a
medical management and surgical and endovascular
first- or second-degree relative management of sporadic and degenerative aneurysms
A history of unexplained sudden death at a relatively young age in a first- are discussed in Sections 6.4, “Medical Management of
or second-degree relative Sporadic and Degenerative Aortic Aneurysm Disease,”
TAD indicates thoracic aortic disease.
and 6.5, “Surgical and Endovascular Management of
Aortic Aneurysms,” respectively.
Approximately 20% of TAA are related to a genetic
or heritable condition (also referred to as heritable tho- 6.1.2. Genetic Aortopathies
racic aortic disease [HTAD]), some of which associ-
6.1.2.1. HTAD: Genetic Testing and Screening of Family
ate with multisystem features (considered syndromic
Members for TAD
HTAD) and others with abnormalities limited to the aorta
Recommendations for HTAD: Genetic Testing and Screening of Family
with or without its branches (known as nonsyndromic Members for TAD
HTAD)7 (Table 7). HTAD most commonly involves the Referenced studies that support the recommendations are
aortic root, ascending aorta, or both but may also summarized in the Online Data Supplement.
present with distal aortic disease and aortic dissec- COR LOE Recommendations
tion.8 Pathogenic variants in multiple genes can lead 1. In patients with aortic root/ascending aortic
to TAA, cerebral aneurysms, and AAA.7,8 Up to 20% of aneurysms or aortic dissection, obtaining a
individuals with a TAA or aortic dissection have a fam- 1 B-NR multigenerational family history of TAD, unex-
plained sudden deaths, and peripheral and
ily history of TAD, with at least 1 affected first-degree intracranial aneurysms is r ecommended.1-3
relative.8 Population studies have shown the familial 2. In patients with aortic root/ascending
nature of TAAs and dissections, with familial cases aortic aneurysms or aortic dissection and
having a significantly increased risk of TAA and aortic 1 B-NR
risk factors for HTAD (Table 8, Figure 17),
genetic testing to identify pathogenic/
dissection8,9 compared with sporadic cases. Therefore, likely pathogenic variants (ie, mutations) is
among patients with aortic root and ascending aortic recommended.4-6
aneurysm or those with aortic dissection, screening 3. In patients with an established pathogenic or
of first-degree relatives with imaging is essential to likely pathogenic variant in a gene predispos-
detect unrecognized, asymptomatic TAD.8,10 ing to HTAD, it is recommended that genetic
1 B-NR
counseling be provided and the patient’s clini-
cal management be informed by the specific
6.1.1. Sporadic and Degenerative TAA gene and variant in the gene.7-9
Although there is a well-recognized anatomic distinc- 4. In patients with TAD who have a pathogenic/
tion between aneurysms of the thoracic versus abdomi- likely pathogenic variant, genetic testing of at-
risk biological relatives (ie, cascade testing) is
nal aorta, this should not imply that all TAA are similar
recommended.6,10,11 In family members who are
in cause or natural history. Aneurysms of the aortic root 1 B-NR found by genetic screening to have inherited
and ascending aorta are typically diagnosed at younger the pathogenic/likely pathogenic variant, aortic
imaging with TTE (if aortic root and ascending
patient ages than aneurysms of the descending thoracic
aorta are adequately visualized, otherwise with
aorta (60 versus 72 years, respectively).1 Even when CT or MRI) is recommended.4,5,12
considering just the “sporadic” aneurysms (ie, aneurysms 5. In a family with aortic root/ascending aor-
in which there is no evidence of a syndromic, familial, tic aneurysms or aortic dissection, if the
or known genetic etiology), a significant difference in disease-causing variant is not identified
1 B-NR with genetic testing, screening aortic imag-
the ages between the 2 groups (64 versus 72 years, ing (as per recommendation 4) of at-risk
respectively) persists.1 In addition, typical atherosclero- biological relatives (ie, cascade testing) is
sis risk factors (ie, hypertension, diabetes, smoking) are recommended.13-15
significantly less common in sporadic root and ascend- 6. In patients with aortic root/ascending aor-
ing versus descending aortic aneurysms.2 Moreover, the tic aneurysms or aortic dissection, in the
absence of either a known family history of
prevalence of aortic calcification or atheroma (by CT or 1 C-LD TAD or pathogenic/likely pathogenic variant,
MRI) is quite low in sporadic aneurysms of the root and screening aortic imaging (as per recommen-
ascending thoracic aorta but quite high in aneurysms of dation 4) of first-degree relatives is recom-
mended.13
the descending aorta, at 8% to 9% versus 80% to 88%,
7. In patients with acute type A aortic dissection,
respectively.1 Collectively, these findings suggest that the diameter of the aortic root and ascending
aneurysms of the aortic root and ascending aortic tend 1 C-EO aorta should be recorded in the operative note
to have a congenital if not hereditary cause, whereas and medical record to inform the management
of affected relatives.
aneurysms of the descending aorta tend to have an

Synopsis that overlap with Marfan syndrome, Loeys-Dietz

CLINICAL STATEMENTS
syndrome, or vascular Ehlers-Danlos syndrome.

A major risk factor for aortic root aneurysms, ascend-
AND GUIDELINES
Clinical genetic testing is integral to the diag-

ing aortic aneurysms, and aortic dissection is a patho-
nostic evaluation of patients with TAD who have
genic variant in genes predisposing to TAD. Although the
clinical indicators suggestive of an underlying
recommendations focus on individuals at high risk for a
single gene disorder (Table 8).5,20 In patients who
single gene mutation (Table 8), genetic testing may have
meet the clinical diagnostic criteria for Marfan
a role in many TAD patients. A multigene panel compris-
syndrome but do not have ectopia lentis (ie, dis-
ing all genes suspected to cause HTAD is the most cost-
located lens), genetic testing is reasonable to
effective and clinically useful approach to testing. Only
exclude an alternative diagnosis of Loeys-Dietz
pathogenic or likely pathogenic variants are disease-
syndrome. Genetic testing laboratories categorize
causing and should be used for cascade genetic test-
rare variants in HTAD genes into these classes:
ing all relatives at risk for inheriting the disease-causing
pathogenic, likely pathogenic, variant of uncer-
variant.15,16 tain/unknown significance, benign, and likely
In families with HTAD in which the causative gene benign. Variants of unknown significance have
has not been identified, the clinical features in affected not been confirmed to cause TAD and therefore
family member should dictate management of other should not be used either to identify which fam-
family members, including location of aneurysms; rel- ily members are at risk or to guide clinical man-
evant clinical features include the diameter of the aortic agement. Because a subset of these variants of
root and ascending aorta in affected family members unknown significance may, nevertheless, be dis-
who have had a type A dissection (noting that the aortic ease-causing, families with the potential to help
root typically is not distorted by the dissection, whereas further classify the variant of unknown signifi-
the ascending aorta may acutely enlarge17) and other cance should be evaluated in collaboration with
vascular disease or features segregating with TAA in the genetic testing company.
the family. 3. FBN1, TGFBR1, TGFBR2, SMAD3, and TGFB2
mutations have been identified in approximately
6% to 8% of HTAD families whose members
do not have syndromic features of Marfan syn-
1. Current data indicate that 13% to 20% of drome or Loeys-Dietz syndrome.12,20-23 Mutations
patients with TAD and without Marfan syndrome in ACTA2, MYH11, MYLK, LOX, and PRKG1 have
or Loeys-Dietz syndrome features have similarly been confirmed to cause HTAD in the absence of
affected first-degree relatives.1,2 TAD in these significant features of Marfan syndrome or Loeys-
families is typically inherited in an autosomal Dietz syndrome.16,24 Through clinical characteriza-
dominant manner, with decreased penetrance, tion of HTAD families with pathogenic variants in
particularly in women. These data suggest that novel genes, data have emerged that the underly-
heterozygous pathogenic variants in single ing gene predicts not only who in the family is at
genes are responsible for HTAD in most fami- risk for thoracic aortic aneurysm and dissection
lies.3,18 In families with HTAD, testing in an indi- (TAAD) but also the aortic disease presentation,
vidual diagnosed with TAD should be initiated. risk for aortic dissection at a given range of aor-
Patients with a family history of the disease tic diameters as described previously, and risk for
present at younger ages (average 57 years).3 and type of additional vascular diseases.7-9 For
These families with HTAD show variable expres- example, TGFBR2 mutations predispose to TAAD
sion of TAD, including varying age of disease but also to intracranial aneurysms and aneurysms
onset, frequency of aortic dissection at a diame- and dissections of other arteries, whereas ACTA2
ter <5.0 cm, risk for type B aortic dissection, and mutations lead to TAAD and occlusive vascular
frequency with which dilation involves the aortic disease, including early onset stroke and coro-
root, the tubular ascending aorta, or both.8,14 In nary artery disease. Genetic counseling is useful
addition, the specific altered gene impacts the to explain to patients and families the genetic risk
risk for associated vascular conditions. and how it is inherited, to assess the family his-
2. The HTAD genetic testing panels include (at the tory to determine TAD risk, to assist in cascade
time of this writing) 11 genes that are confirmed genetic testing and/or imaging for TAD in family
to confer a highly penetrant risk for TAD: FBN1, members, and to offer psychosocial and ethical
LOX, COL3A1, TGFBR1, TGFBR2, SMAD3, guidance.10
TGFB2, ACTA2, MYH11, MYLK, and PRKG1.19 4. Cascade screening is the process of extend-
These panels also include genes that increase ing imaging to identify asymptomatic thoracic
the risk for TAD and/or lead to systemic features aortic enlargement to individuals at risk within a

family for inheriting the pathogenic variant caus- 6.1.2.1.1. Surgical Considerations for Nonsyndromic
CLINICAL STATEMENTS
ing HTAD in the family; the process is repeated as Heritable TAAs and No Identified Genetic Cause
AND GUIDELINES
family members are identified with thoracic aortic
Recommendations for Surgical Considerations for Nonsyndromic
enlargement or as carriers of the pathogenic vari-
Heritable TAA and No Identified Genetic Cause
ant are identified.10 Pathogenic variants in genes
for HTAD confer a high risk for TAD, so individuals
found to have these pathogenic variants should 1. In asymptomatic patients with aneurysms
of the aortic root or ascending aorta with
be screened with aortic imaging for asymptomatic nonsyndromic heritable thoracic aortic dis-
TAD.16,24 ease (nsHTAD) and no identified genetic
5. Among patients undergoing genetic testing, 1 C-LD cause, determining the timing of surgical
repair requires shared decision-making and
many will not have a pathogenic variant identified, is informed by known aortic diameters at the
despite other clinical evidence that the disease time of aortic dissection, TAA repair, or both in
is likely genetically triggered (eg, extensive family affected family members.1-4
history of TAD or early onset sporadic TAD with 2. In asymptomatic patients with aneurysms of
the aortic root or ascending aorta with
no risk factors). Despite the absence of a patho-
nsHTAD and no identified genetic cause but
genic variant among the currently known genes no information on aortic diameters at the time
that were tested, TAD could still be inherited in 1 C-LD of dissection or aneurysm repair in affected
family members and who have no high-risk
the family attributable to a causative genetic vari-
features for adverse aortic events (Table 9) it
ant that has yet to be identified. Consequently, is recommended to repair the aorta when the
multiple studies have confirmed the utility of maximal diameter reaches ≥5.0 cm.1
screening aortic imaging of at-risk relatives of 3. In patients with aneurysms of the aortic root
all TAD patients with a positive family history.13-15 or ascending aorta with nsHTAD and no
identified genetic cause and a maximal aortic
If negative, repeat screening imaging might be diameter of ≥4.5 cm, who have high-risk fea-
worthwhile in 5 years of younger family members 2a C-LD tures for adverse aortic events (Table 9), or
or 10 years in older family members, informed who are undergoing cardiac surgery for other
indications, aortic repair is reasonable when
by the family history. Additionally, it is critical to performed by experienced surgeons in a Mul-
obtain relevant clinical data from affected fam- tidisciplinary Aortic Team.5
ily members, including the location of the aortic
dilation (ie., the aortic root versus the ascending
aorta), current aortic diameter or diameter at the Synopsis

time of surgical repair or diameter at the time HTAD refers to TAD caused by a highly penetrant rare
of type A aortic dissection, and the presence of variant (or mutation) in a single gene. A diagnosis of
other vascular diseases (eg, aneurysms in other HTAD is based on ≥2 members of a family with TAD, the
arteries, early onset occlusive vascular diseases), identification of a pathogenic variant in the gene known
as these will inform management of all affected to cause TAD in a family member, or clinical diagnosis of
family members. The HTADs vary in terms of the syndrome that confers a risk for TAD (eg, Marfan syn-
risk of other clinical cardiovascular complications drome) in a family member. Syndromic HTAD typically
that segregate with TAD; therefore, surveillance has systemic features with multiorgan phenotype, posi-
for such conditions is best guided by the family tive family history of aortic aneurysm or dissection, and is
history.22,25-27 often caused by mutations involving extracellular matrix
6. Although the data are more limited, studies also proteins or involved in transforming growth factor-β
support the screening of first-degree relatives of pathway. Such patients, including Marfan syndrome and
patients with TAD who do not have a family history Loeys-Dietz syndrome, are predisposed to developing
of the disease.13 If negative, aortic imaging may be aneurysms of the aortic root and ascending aorta at an
repeated years later, depending on the relative’s early age, and have a faster rate of aortic growth than
age and aortic size. It should be recognized that do those with sporadic aneurysms. Consequently, these
there is no upper limit to the age at which patients
present with TAD that precludes an underlying
genetic cause of the disease. Table 9. Features Associated With an Increased Risk of
7. Because the size at which the aortic root or ascend- Aortic Dissection in Patients With Heritable Thoracic Aortic
ing aorta dissects impacts the risk of aortic dissec- Aneurysms
tion in other affected family members, the specific Heritable Thoracic Aortic Aneurysms and No Identified Genetic Cause
aortic diameters should be recorded in the medical
Family history of aortic dissection at an aortic diameter <5.0 cm
record (ie, operative report, discharge summary), so
Family history of unexplained sudden death at age <50 y
that the information can be readily retrieved when
needed in the future. Rapid aortic growth (0.5 cm in 1 y or 0.3 cm/y in 2 consecutive y)

patients have a higher risk of acute aortic dissection or elective surgery or experienced aortic dissection.
CLINICAL STATEMENTS
rupture, resulting in a shorter life expectancy than those However, the GenTAC study suggested a higher
AND GUIDELINES
patients whose aneurysms are not genetically medi- risk of aortic dissection, with a large proportion
ated. Prophylactic surgery to replace the aortic root and of patients not having met the 5.5-cm threshold
ascending aorta has dramatically improved the overall for elective repair at the time of their aortic dis-
life expectancy of HTAD patients. Prophylactic elec- section. Given that aortic dissection in this popu-
tive surgery in these young patients requires a very low lation with familial TAAs may occur at younger
operative mortality with a multidisciplinary approach for ages and with worse outcomes and the more
genetic testing and lifelong surveillance. Surgeons in frequent need for reoperations, prophylactic sur-
Multidisciplinary Aortic Teams have shown sufficiently gery is warranted when the maximal diameter of
low operative mortality to safely treat these patients at the aortic root or ascending aorta reaches ≥5.0
smaller aortic sizes. Similar to what is seen with sporadic cm.1-4,8
aneurysms, aortic dissection in HTAD can occur at aortic 3. For patients with a family history of aortic dissection
diameters smaller than the surgical thresholds recom- at a known maximal aortic root or ascending aortic
mended in guidelines. diameter <5.0 cm but with no known pathogenic
nsHTAD refers to a genetic predisposition to TAD variant, it is reasonable to perform prophylactic aor-
running in families in the absence of systemic features. tic repair at a maximal aortic diameter of ≥4.5 cm,
NsHTAD may be present in up to 20% of patients with because their affected relative experienced an
TAD (based on family history), is typically inherited in an aortic dissection at the relatively small diameter
autosomal dominant manner, with a pathogenic genetic of <5.0 cm. Similarly, patients with relatives whose
variant identified in up to 20%. When no pathogenic aortic dissection or unexplained sudden death
variant is identified in families with nsHTAD, it has often occurred at an age <50 years are themselves at
been referred to as “familial thoracic aortic aneurysm and increased risk of such adverse events at ages <50
dissection.” It tends to be more penetrant and of earlier years as well. Similarly, nsHTAD patients who have
onset in men than women within affected families. The documented rapid aneurysm growth are increased
diagnosis is often delayed until midlife but occurs earlier risk of untoward aortic events at younger ages
than for sporadic aneurysms; aneurysm growth is also and smaller aneurysm sizes, so prophylactic aor-
typically faster than for sporadic aneurysms. Because tic surgery is reasonable when performed by
the initial presentation is commonly acute aortic dissec- experienced surgeons in Multidisciplinary Aortic
tion, screening family members is important to guide pro- Teams, with shown excellent short- and long-term
phylactic surgery to prevent potential aortic dissection. outcomes.1-4,8
Clearly, elective surgery before aortic dissection yields
better long-term survival with fewer aortic reinterventions
6.1.2.2. Marfan Syndrome
than surgery after aortic dissection.4-7
6.1.2.2.1. Diagnostic and Surveillance Aortic Imaging
in Marfan Syndrome
Recommendations for Diagnostic and Surveillance Aortic Imaging in
1. The GenTAC (National Registry of Genetically Marfan Syndrome
Triggered Thoracic Aortic Aneurysms and Referenced studies that support the recommendations are
Cardiovascular Conditions) study found a higher
risk of dissection, with most of dissection patients COR LOE Recommendations
not having met the size criteria for prophylactic sur- Initial Diagnosis and Surveillance Imaging
gery.1,6 For patients with a family history of TAA, 1. In patients with Marfan syndrome, a TTE
aortic dissection, or both, but with no known patho- is recommended at the time of initial
diagnosis, to determine the diameters of
genic variant, it is useful to determine the size at the aortic root and ascending aorta, and 6
which the aorta dissected (if known) or the size months thereafter, to determine the rate
at which elective aortic surgery was performed, 1 C-EO of aortic growth; if the aortic diameters
are stable, an annual surveillance TTE is
as well as the age of the affected relative at time recommended.1 If the aortic root, ascending
of the aortic event. It is appropriate to offer aortic aorta, or both are not adequately visualized
repair based on the family member’s aortic size at on TTE, a CT or MRI of the thoracic aorta is
recommended.2
dissection or elective surgery.
2. Patients with a family history of TAAs but with 2. In adults with Marfan syndrome, after the
initial TTE, a CT or MRI of the thoracic aorta
no known pathogenic variant may not have 2a C-EO is reasonable to confirm the aortic diameters
information regarding the aneurysm size at and assess the remainder of the thoracic
which the family members underwent either aorta.

Recommendations for Diagnostic and Surveillance Aortic Imaging in the diameter exceeds 4.5 cm. Patients with Marfan
CLINICAL STATEMENTS
Marfan Syndrome (Continued) syndrome are at greatest risk for aneurysmal dila-
AND GUIDELINES
COR LOE Recommendations tion of the aortic root, followed by involvement of
Imaging After Aortic Root Replacement the ascending aorta. Patient-specific factors, such
3. In patients with Marfan syndrome who have
as pectus deformities and lung disease, may limit
undergone aortic root replacement, surveil- the evaluation of the aortic root on TTE. When the
lance imaging of the thoracic aorta by MRI aortic root and ascending aorta are not adequately
1 C-LD (or CT) is recommended to evaluate for distal
TAD, initially annually and then, if normal in
visualized by TTE, CT or MRI should be performed
diameter and unchanged after 2 years, every to measure the aortic diameters,2 although TEE is
other year.3-6 another alternative to measure the aortic root and
4. In patients with Marfan syndrome who have ascending aorta.
2a C-LD
undergone aortic root replacement, surveil- 2. Patients with Marfan syndrome may develop dis-
lance imaging every 3 to 5 years for potential
AAA is reasonable.2,6
ease of the descending aorta.9,10 In some indi-
viduals, a thorough TTE may accurately assess
the diameters of aortic root, ascending aorta,
Synopsis aortic arch, proximal descending aorta, and distal
Marfan syndrome is an autosomal dominant connec- descending aorta. For patients undergoing an ini-
tive tissue disorder caused by pathogenic variants in the tial evaluation in whom the aortic segments distal
FBN1 gene affecting 1 in 5 000 individuals.1 Phenotypic to the ascending aorta are not adequately visual-
features in the skeletal, ocular, pulmonary, cutaneous, ner- ized on TTE, a CT or MRI can be used to assess
vous, and cardiovascular systems may be recognized. The the more distal aortic segments.
modified Ghent criteria for diagnosis incorporate genetic 3. Surgical aortic root replacement can prevent
testing, the systemic score, ectopia lentis, and the family type A aortic dissection and improve longevity for
history.1 Patients with Marfan syndrome develop aneu- patients with Marfan syndrome and aortic root
rysms involving the aortic root (sinuses of Valsalva) and aneurysms.3,9,10 Long-term complications after aor-
are at risk for aortic dissection.1 Descending aortic and tic root replacement may include graft infections,
AAAs are less common.6,7 Type B aortic dissection is the pseudoaneurysms, aneurysms in the distal aorta,
initial aortic event in about 10% of patients and may also and aortic dissection distal to the graft.4,13
4. In patients with Marfan syndrome, distal TAA and
occur despite previous root replacement.4 Imaging sur-

veillance of the aorta is typically performed annually, with AAA (in the absence of aortic dissection) may
the frequency dependent on age, aortic diameter, rate occur but are much less common than aortic root
of aortic growth, and family history.8 Prophylactic aortic disease. Most individuals with aortic disease distal
root replacement for aneurysm disease prevents type A to the root have had previous root replacement or
dissection and improves survival in Marfan syndrome.3,9,10 smoke cigarettes.7,13
6.1.2.2.2. Medical Therapy in Marfan Syndrome

Recommendations for Medical Therapy in Marfan Syndrome
1. Aortic root dilation and type A aortic dissection Referenced studies that support the recommendations are
are the leading causes of morbidity and mortality summarized in the Online Data Supplement.
in Marfan syndrome.9,10 Aortic dilation involves the COR LOE Recommendations

aortic root, but effacement of the sinotubular junc- 1. In patients with Marfan syndrome, treatment
tion with enlargement of the proximal ascending with either a beta blocker or an ARB, in maxi-
1 A mally tolerated doses (unless contraindicated),
aorta is often present.11 The aortic root and ascend- is recommended to reduce the rate of aortic
ing aorta are measured by TTE and are observed dilation.1,2
annually. Nomograms accounting for age, sex, and 2. In patients with Marfan syndrome, the use of
body size (and height) assist with determining the both a beta blocker and an ARB, in maximally
degree to which the diameter deviates from normal 2a C-LD tolerated doses (unless contraindicated), is
reasonable to reduce the rate of aortic dila-
in the general population.12 In patients with Marfan tion.3,4
syndrome participating in trials of beta block-
ers versus angiotensin receptor blockers (ARBs),
the mean growth of the aortic root was 1 mm to Synopsis
1.5 mm over 3 years and 4 mm to 5 mm over 5 Beta blockers have long been recommended for patients
years. The rate of aortic dilation is faster in patients with Marfan syndrome to reduce heart rate and myocar-
with larger aortic aneurysms. More frequent imag- dial contractility and to slow aortic root growth.5-7 More
ing is performed in patients with rapid aortic growth, recently, ARBs have also been found to be efficacious in
in those approaching surgical thresholds, or when Marfan syndrome.1-4,8

Recommendation-Specific Supportive Text Table 10. Features Associated With Increased Risk of Aortic
CLINICAL STATEMENTS
Complications in Marfan Syndrome

1. In an open-label study of patients with Marfan
AND GUIDELINES
Family history of aortic dissection

syndrome who were observed for >10 years, pro-
pranolol treatment was associated with a reduc- Rapid aortic growth (0.3 cm/y)
tion in aortic root growth rate and fewer clinical Diffuse aortic root and ascending aortic dilation14
events5 compared with control (no treatment). More Marked vertebral arterial tortuosity15
recently, in a retrospective evaluation of children
with Marfan syndrome, beta-blocker treatment
was associated with a reduced aortic growth rate.6 Synopsis
Losartan was shown to prevent aneurysm forma- Prophylactic aortic root replacement for aneurysm dis-
tion in mouse models of Marfan syndrome9 and, in ease prevents type A aortic dissection and improves
a small, nonrandomized open label study of children survival in Marfan syndrome.6-8 The size threshold for
with Marfan syndrome who had previously had rapid elective surgery to replace the dilated aortic root in Mar-
aortic root growth, ARBs were shown to dramati- fan syndrome is dependent on many factors, including
cally slow aortic root growth.10 However, random- the patient’s age, height and weight, family history, rate
ized trials comparing an ARB to a beta blocker in of aortic growth, and other patient-specific factors.1,3-5,9
patients with Marfan syndrome found no significant In patients with Marfan syndrome who are managed with
difference in the rate of either aortic root growth optimal medical therapy and whose aortic diameters are
or clinical events (including aortic surgery or aortic <5.0 cm, the risk of aortic dissection is low.3,4,10 However,
dissection) between the 2 treatment groups.1,2 the risk of aortic dissection increases when the aortic
2. Multiple trials have compared the addition of diameter is >5.0 cm and is greater in patients with a fam-
an ARB to beta-blocker therapy in patients with ily history of aortic dissection or rapid aortic growth.3,4,10
Marfan syndrome3,4,8; in 2 studies, the addition of
an ARB led to a reduction of aortic root growth
rates over a 3- to 5-year follow-up,3,4 and a meta- Recommendation-Specific Supportive Text
analysis confirmed slower aortic growth rates with
1. In patients with Marfan syndrome and a dilated
combination therapy.11
aortic root, elective aortic root and ascending aortic
6.1.2.2.3. Marfan Syndrome Interventions: replacement before aortic dissection improves sur-
Replacement of the Aortic Root in Patients With vival.6-8 A landmark report in 1995 documented the
Marfan Syndrome marked improvement in lifespan among patients
Recommendations for Marfan Syndrome Interventions: Replacement of with Marfan syndrome treated with elective aortic
the Aortic Root in Patients With Marfan Syndrome
Referenced studies that support the recommendations are
repair compared with historical controls from previ-
summarized in the Online Data Supplement. ous eras.6,10 Although risk of aortic dissection is low
in patients with Marfan syndrome who are receiv-
ing appropriate medical care and lifestyle modifica-
1. In patients with Marfan syndrome and an
aortic root diameter of ≥5.0 cm, surgery to tions, the risk of aortic dissection increases when
1 B-NR
replace the aortic root and ascending aorta is the aortic diameter is >5.0 cm.3,4,11 When pro-
recommended.1-4
phylactic surgical aortic repair is performed, both
2. In patients with Marfan syndrome, an aortic the aortic root and ascending aorta are replaced;
root diameter of ≥4.5 cm, and features associ-
ated with an increased risk of aortic dissection
although some centers have advocated including
2a B-NR (see Table 10), surgery to replace the aortic hemiarch replacement in patients at the time of
root and ascending aorta is reasonable, when elective root/ascending aorta replacement, data to
performed by experienced surgeons in a Mul-
tidisciplinary Aortic Team.1,3,4
support this approach are lacking.
2. In large series of patients with Marfan syndrome,
3. In patients with Marfan syndrome and a maxi-
mal cross-sectional aortic root area (cm2) about 20% have undergone elective surgery when
2a C-LD
to patient height (m) ratio of ≥10, surgery to aortic root diameters are <5.0 cm.3,4,11 Predictors
replace the aortic root and ascending aorta is of aortic dissection and other adverse aortic out-
reasonable, when performed by experienced
surgeons in a Multidisciplinary Aortic Team.5 comes in Marfan syndrome are listed in Table 10.
4. In patients with Marfan syndrome and an aor-
Indications for earlier aortic surgery may include
tic diameter approaching surgical threshold, rapid aortic growth (≥0.3 cm/y), family history of
who are candidates for valve-sparing root aortic dissection, desire for pregnancy, severe valve
replacement (VSRR) and have a very low
2b C-LD
surgical risk, surgery to replace the aortic root
regurgitation, and patient preference.3,9,12 For most
and ascending aorta may be reasonable when patients with Marfan syndrome, aortic growth rates
performed by experienced surgeons in a Mul- are relatively slow, but the growth rate increases
tidisciplinary Aortic Team.2-4
with aortic size.12

3. Aortic diameters vary depending on age, sex, dissection or rupture in patients with Marfan syndrome
CLINICAL STATEMENTS
height, and body size. Aortic event rates, includ- with primary (nondissected) aneurysms of the aortic arch,
AND GUIDELINES
ing aortic dissection, increase as the aortic size descending, or abdominal aorta, so using a 5.0-cm diam-
indexed to height (or body size) increases. When eter threshold for surgery, as is used for the aortic root,
the maximal cross-sectional area in square (cm2) is reasonable.
of the aortic root or ascending aorta divided by the
patient’s height (m) is ≥10 cm2/m, prophylactic Recommendation-Specific Supportive Text
aortic root replacement is reasonable; when this
cross-sectional area to height ratio was used to 1. Although uncommon, aortic segments distal to
guide prophylactic surgery, patients had favorable the aortic root and ascending aorta may dilate in
outcomes. Marfan syndrome, and this occurs more often after
4. Aortic root replacement is associated with a very elective aortic root replacement or after a previ-
low surgical risk3,4,11 when performed by experi- ous aortic dissection involving these segments.5
enced surgeons in Multidisciplinary Aortic Teams. In patients at acceptable risk for operative repair
The 2 aortic root replacement procedures per- or with a long life expectancy, operative interven-
formed most commonly in the United States are tion to resect primary (nondissected) aneurysms
a composite valved graft conduit and a VSRR.13 involving the arch, descending, or abdominal aorta
The composite valved graft conduit consists of a is reasonable at an aortic diameter threshold of
prosthetic aortic valve (typically mechanical but ≥5.0 cm, depending on the patient’s age, rate of
may be bioprosthetic) and aortic graft, with reim- aortic growth, family history, and surgical risk. Type
plantation of the coronary arteries (often referred B aortic dissection occurs in about 10% of Marfan
to as the modified Bentall procedure). The VSRR patients, often in the absence of significant dila-
uses the David procedure, in which the native tion of the descending aorta, and is sometimes
aortic valve is reimplanted into a prosthetic aortic associated with prior elective aortic root replace-
graft that is attached to the left ventricular outflow ment,1 a previous aortic dissection elsewhere,6 or
tract proximally and to the ascending aorta distally. pregnancy.7
The advantage of the VSRR is that, if successful,
patients can potentially avoid the lifelong risks and 6.1.2.3. Loeys-Dietz Syndrome
complications associated with prosthetic valves. 6.1.2.3.1. Imaging in Loeys-Dietz Syndrome
Consequently, early prophylactic surgery can be Recommendations for Imaging in Loeys-Dietz Syndrome
considered when both the procedural and late risks COR LOE Recommendations
are low. However, durability of the spared native 1. In patients with Loeys-Dietz syndrome, a
aortic valve is a potential concern; in one series of baseline TTE is recommended to determine
239 patients with Marfan syndrome undergoing the diameters of the aortic root and ascending
1 C-EO aorta, and 6 months thereafter to determine
VSRR, 7% developed at least moderate AR at 1 the rate of aortic growth; if the aortic diam-
year follow-up.13 eters are stable, annual surveillance TTE is
recommended.1-3
6.1.2.2.4. Marfan Syndrome Interventions:
2. In patients with Loeys-Dietz syndrome and
Replacement of Primary (Nondissected) Aneurysms a dilated or dissected aorta and/or arterial
of the Aortic Arch, Descending, and Abdominal Aorta 1 C-EO branches at baseline, annual surveillance
in Patients With Marfan Syndrome imaging of the affected aorta and arteries with
MRI or CT is recommended.1
Recommendation for Replacement of Primary (Nondissected)
Aneurysms of the Aortic Arch, Descending, and Abdominal Aorta in 3. In patients with Loeys-Dietz syndrome, a
Patients With Marfan Syndrome baseline MRI or CT from head to pelvis is
1 C-LD recommended to evaluate the entire aorta
COR LOE Recommendation and its branches for aneurysm, dissection, and
tortuosity.1-4
1. In patients with Marfan syndrome and a
nondissected aneurysm of the aortic arch, 4. In patients with Loeys-Dietz syndrome with-
descending thoracic aorta, or abdominal out dilation of the aorta distal to the aortic
2a C-EO
aorta of ≥5.0 cm, surgical intervention root or ascending aorta and without dilated
to replace the aneurysmal segment is or dissected arterial branches, surveillance
2a C-EO
reasonable. imaging from chest to pelvis with MRI (or
CT) every 2 years is reasonable, but imaging
may be more frequent depending on family
Synopsis history.
5. In patients with Loeys-Dietz syndrome with-
Marfan syndrome most commonly leads to aneurysms out dilation of the cerebral arteries on initial
of the aortic root and ascending aorta but may also 2a C-EO screening, periodic imaging surveillance for
affect the distal aorta and its branches.1-4 Unfortunately, cerebral aneurysms with MRI or CT every 2 to
3 years is reasonable.
there are no large datasets to inform the risk of aortic

Synopsis aneurysm disease involved the ascending aorta

CLINICAL STATEMENTS
in 78%, arch in 10%, descending aorta in 10%,

Loeys-Dietz syndrome is characterized by aortic and
AND GUIDELINES
abdominal aorta and branches in 17%, thoracic

branch vessel aneurysms and dissections, arterial tortuos-
aortic branches in 21%, and head and neck arterial
ity, and skeletal features similar to those seen in Marfan
branches in 10%.1 Among patients with SMAD3-
syndrome but with unique craniofacial and cutaneous fea-
related disease, aneurysms from head to pelvis are
tures.1 Pathogenic variants in 5 genes cause Loeys-Dietz
also described.9,12 Individuals with Loeys-Dietz syn-
syndrome, also termed transforming growth factor-β vas-
drome can be at risk for TAD and other vascular
culopathies.1-3,5,6 Some pathogenic variants in Loeys-Dietz
diseases in the absence of other systemic features
syndrome genes, in particular TGFBR1 and TGFBR2, may
characteristic of Marfan syndrome or Loeys-Dietz
have earlier onset TAD.7 All the Loeys-Dietz syndrome
syndrome. Although there is phenotypic overlap
genes confer a risk for aortic involvement distal to the
among the genes, there is also distinct vascular
aortic root along with branch vessel and intracranial aneu-
disease and systemic complications associated
rysms.1,8-11 Most clinical information is available in patients
with each gene. The physician should be cognizant
with TGFBR1 and TGFBR2 pathogenic variants.1,8 Patho-
of the particular gene variant in monitoring and
genic variants in SMAD3 are associated with premature
managing patients with Loeys-Dietz syndrome.
osteoarthritis and later onset of TAD.9,12 There is much less
4. In patients with Loeys-Dietz syndrome and aortic
information about the aortic and branch vessel disease in
disease limited to segments that are well-visualized
patients with variants in TGFB2 and TGFB3.13-16 Imaging
by TTE and without branch vessel disease, surveil-
with CT or MRI, from head to pelvis, is indicated to evalu-
lance of the distal aorta and its branches is needed
ate for aneurysms and arterial tortuosity.
to evaluate for the possible interval occurrence of
dilation (or dissection); the frequency of surveil-
lance imaging may be influenced by the patient’s
Recommendation-Specific Supportive Text age and family history.2
1. Aortic root and ascending aortic aneurysm and 5. Cerebral aneurysms are described in 10% to 18%
aortic dissection are leading causes of morbidity of patients with Loeys-Dietz syndrome.1,9,11,20 The
and mortality in Loeys-Dietz syndrome.1,8,9,12 Aortic frequency of follow-up screening for cerebral aneu-
dissection may occur at relatively small aortic rysm disease in patients without aneurysms on initial
diameters in Loeys-Dietz syndrome when related screening will depend on the patient’s age and may
to pathogenic variants in TGFBR1, TGFBR2, and be informed by phenotype or other features.11

SMAD3.1,2,6 The specific genetic variant and sever- 6.1.2.3.2. Medical Therapy in Loeys-Dietz Syndrome
ity of extra-aortic phenotypic features, including
Recommendation for Medical Therapy in Loeys-Dietz Syndrome
craniofacial features, degree of arterial tortuosity,
cutaneous findings, and family history inform the COR LOE Recommendation
risk of aortic events.1,2,6 The aortic root and ascend- 1. In patients with Loeys-Dietz syndrome, treat-
ment with a beta blocker or an ARB (unless
ing aortic diameters are typically measured by TTE. 2a C-EO
contraindicated), or both, in maximally toler-
BAV is more common in Loeys-Dietz syndrome and ated doses, is reasonable.
can be diagnosed by TTE.17 Patients with Loeys-
Dietz syndrome attributable to certain pathogenic
variants are at risk for aortic dissection at relatively Synopsis
small aortic diameters.1,8 In patients with Loeys- The management of individuals with Loeys-Dietz syn-
Dietz syndrome, the stability of the aortic size 6 drome includes medical therapy, lifestyle modification,
months after the initial diagnosis should be deter- imaging surveillance, and surgical intervention. To lessen
mined, and then, once stability is confirmed, moni- hemodynamic stress on the aorta, beta blockers are
tored with annual surveillance imaging.1,2 used.1 Based on studies of mouse models, ARBs have
2. Patients with Loeys-Dietz syndrome may have vari- also been used.2
able aortic and branch vessel involvement and vari-
able rates of dilation of involved arterial segments
over time. In Loeys-Dietz syndrome patients with Recommendation-Specific Supportive Text
aortic aneurysm or previous dissection, relatively 1. There are no randomized trials of medications
rapid arterial enlargement may occur.2,18,19 to reduce aortic growth or the risk of aortic dis-
3. Patients with Loeys-Dietz syndrome are at risk section in patients with Loeys-Dietz syndrome.
for widespread aortic and branch vessel aneurys- Consequently, the approach to medical therapy is
mal disease and dissections.1,12 In a series of 90 similar to that used for treating patients with Marfan
patients with Loeys-Dietz syndrome attributable syndrome, based on the similarities between the
to pathogenic variants in TGFBR1 and TGFBR2, 2 connective tissue disorders and on data from

mouse models of Loeys-Dietz syndrome.2 Thus, the SMAD3 variants.1,2,4,15,16 The size threshold for elective
CLINICAL STATEMENTS
use of beta blockers, ARBs, or both is reasonable.1 surgery to replace the dilated aortic root and ascending
AND GUIDELINES
aorta in Loeys-Dietz syndrome depends on multiple fac-
6.1.2.3.3. Loeys-Dietz Syndrome Surgical tors and is informed by the specific pathogenic variant,
Interventions: Replacement of the Aorta in Patients phenotypic features, patient age, aortic growth rates, and
With Loeys-Dietz Syndrome family history (Table 11).1,2,10-12,17
Recommendations for Replacement of the Aorta in Patients With
There is little information about size thresholds for
Loeys-Dietz Syndrome prophylactic surgery in Loeys-Dietz syndrome to lessen
COR LOE Recommendations the risk of aortic dissection or rupture when there are
intact aneurysms involving the aortic arch, descending, or
1. In patients with Loeys-Dietz syndrome and
aortic dilation, the surgical threshold for abdominal aorta, or involving aortic branch vessels.11,12,18
prophylactic aortic root and ascending aortic After aortic dissection, progressive aneurysmal dilation
replacement should be informed by the spe-
1 C-LD commonly occurs and often requires multiple operative
cific genetic variant, aortic diameter, aortic
growth rate, extra-aortic features, family his- interventions.11,12,18
tory, patient age and sex, and physician and
patient preferences (Table 11).1-9
2. In patients with Loeys-Dietz syndrome attrib- Recommendation-Specific Supportive Text
utable to a pathogenic variant in TGFBR1,
TGFBR2, or SMAD3, surgery to replace 1. Pathogenic variants in TGFBR1, TGFBR2, SMAD3,
the intact aortic arch, descending aorta, or TGFB2, and TGFB3 lead to Loeys-Dietz syndrome
2b C-EO abdominal aorta at a diameter of ≥4.5 cm or may cause aortopathy with few outward fea-
may be considered, with the specific genetic
variant, patient age, aortic growth rate, family tures. Most information is available for TGFBR1
history, presence of high-risk features (Table and TGFBR2 pathogenic variants.1,2 Patients
11), and surgical risk informing the decision. with TGFBR1 and TGFBR2 variants are at risk
of type A aortic dissection at younger ages and
smaller aortic root diameters than in Marfan syn-
Synopsis drome.1,17,19 This aggressive aortopathy, especially
In patients with Loeys-Dietz syndrome, prophylactic aor- in those with severe craniofacial features, previ-
tic root replacement for aneurysm disease prevents type ously led to a recommendation for surgery at an
A aortic dissection and improves outcomes.1,2,10-12 Aortic aortic root diameter of >4.0 cm.1 The “2010 ACC/
dissection in Loeys-Dietz syndrome that is attributable to AHA Guidelines for the Management of Thoracic
pathogenic variants in TGFBR1, TGFBR2, and SMAD3 Aortic Disease” recommended aortic surgery at a
may occur at smaller aortic diameters than in Marfan diameter between 4.2 cm and 4.6 cm, depending
syndrome.1-3,13 Based on limited data, Loeys-Dietz syn- on imaging modality.20 SMAD3-related Loeys-Dietz
drome attributable to pathogenic variants in TGFB25,6,14 syndrome variants may lead to aortic dissection at
and TGFB38,9 may have a less aggressive aortic pheno- variable diameters.4,15,16,20 Aortic dissection risk is
type than disease attributable to TGFBR1, TGFBR2, or higher in women with TGFBR2 variants who have
Table 11. Surgical Thresholds for Prophylactic Aortic Root and Ascending Aortic Replacement
in Loeys-Dietz Syndrome Based on Genetic Variant
COR LOE (references) Genetic Variant Presence of High-Risk Features* Aortic Diameter (cm)
1 C-LD 2
TGFBR1 No 4.5
1 C-LD2 TGFBR2 No 4.5
2b C-EO 2
TGFBR1 Yes 4.0
2a C-LD1,2 TGFBR2 Yes 4.0
2a C-EO 13,16
SMAD3 – 4.5†
2b C-EO5-7 TGFB2‡ – 4.5†
2b C-EO 9,23
TGFB3 – 5.0†
*Aortic surgery may be recommended at smaller aortic diameters in Loeys-Dietz syndrome attributable to TGFBR1 and
TGFBR2 pathogenic variants when there are features that associate with a higher risk of aortic dissection, including: certain
specific pathogenic variants; women with TGFBR2 and small body size; severe extra-aortic features (ie, craniosynostosis,
cleft palate, hypertelorism, bifid uvula, marked arterial tortuosity, widened scars, and translucent skin); family history of aortic
dissection (especially at young age or relatively small aortic diameter); and aortic growth rate >0.3 cm/y.
†Family history, age, and aortic growth rate also inform surgical thresholds.
‡Pathogenic variants in the TGFB2 gene are different than variants in the TGFBR2 gene.
COR indicates class of recommendation; and LOE, level of evidence.
Colors correspond to COR and LOE in Table 2.

certain extraaortic features.2 Limited data have not with some physicians choosing alternative beta blockers
CLINICAL STATEMENTS
suggested higher aortic dissection risk at smaller with vasodilatory properties. There are no studies showing
AND GUIDELINES
aortic size in those with TGFB25,6,14 or TGFB3 vari- a benefit of ARBs in vascular Ehlers-Danlos syndrome.
ants.8,9 Marked intrafamilial variability exists for Surgical repair in vascular Ehlers-Danlos syndrome
aortic disease in Loeys-Dietz syndrome.17,21,22 A carries an increased risk because of vascular fragility
shared decision about timing of prophylactic sur- and associated bleeding complications.1-3,5 Rapid arte-
gery to prevent type A aortic dissection in Loeys- rial aneurysm growth or the occurrence of dissection are
Dietz syndrome should include consideration of indications for treatment,1-3,5 but no data are available to
the specific genetic variant, aortic diameter, aor- guide diameter thresholds for prophylactic surgical inter-
tic growth rate, age, sex, body size, family history, vention for aortic and arterial branch vessel aneurysms
patient preferences, and surgical expertise. in vascular Ehlers-Danlos syndrome.1-5 Consequently, the
2. Aneurysms of the distal ascending aorta, arch, decision to intervene for aortic and branch vessel aneu-
descending aorta, and abdominal aorta may occur rysms and dissections involves a Multidisciplinary Aor-
in Loeys-Dietz syndrome.1,2,5,8,9,11-14,16,17 At the time tic Team and shared decision-making.3,6 Open surgery
of aortic root replacement, the entire ascending requires meticulous technique to lessen vascular and
aorta is also to be replaced because distal ascend- tissue trauma, and interventional techniques may involve
ing aortic aneurysm and dissection may occur arterial embolization and endovascular therapy, depend-
after isolated aortic root replacement.10-12,19 There ing on individual circumstances.1,3,5
is little information about aortic size thresholds at Guidelines for management of pregnancy in vascular
which the risk of aortic dissection warrants elec- Ehlers-Danlos syndrome are limited, given the lack of data
tive surgery in the intact aortic arch, descending, and the rarity of the condition.9 The decision to proceed with
or abdominal aorta in Loeys-Dietz syndrome. A pregnancy in vascular Ehlers-Danlos syndrome is complex;
shared decision should consider the pathogenic for some women with specific genetic variants, null muta-
variant, aortic diameter, rate of aortic growth, age, tions, and normal vascular imaging, the risk may be lower,
sex, body size, patient preference, and the sur- but shared decision-making is essential.9 Of 38 women
geon’s preference and surgical expertise. Aortic with vascular Ehlers-Danlos syndrome completing 82 deliv-
interventions in Loeys-Dietz syndrome are espe- eries, only 13% were aware of their diagnosis before preg-
cially common after aortic dissection.10-12 nancy.9 Tissue fragility complicates labor and delivery and
poses risks for vascular events and wound complications.9,10
6.1.2.4. Vascular Ehlers-Danlos Syndrome: Imaging, Complications may occur after vaginal or cesarean deliver-
Medical Therapy, and Surgical Intervention ies, but most women known to have vascular Ehlers-Danlos
Vascular Ehlers-Danlos syndrome, affecting 1 in 50 000 syndrome undergo cesarean delivery.9-12
to 100 000 individuals, is attributable to pathogenic vari-
ants in COL3A1 and leads to spontaneous aortic and 6.1.2.5. Turner Syndrome
arterial dissections, aneurysms, and rupture at young
Recommendations for Diagnostic Testing, Surveillance, and Surgical
ages.1,2 The onset and severity of arterial pathology cor- Intervention for Aortic Dilation in Turner Syndrome
relates with the specific COL3A1 pathogenic variant.2 Referenced studies that support the recommendations are
Imaging the aorta and branches may identify arterial seg- summarized in the Online Data Supplement.
ments at risk, but the frequency of screening surveillance COR LOE Recommendations
is uncertain.1-4 Typical protocols include baseline MRI or 1. In patients with Turner syndrome, TTE and
CT from head to pelvis to evaluate the entire aorta and cardiac MRI are recommended at the time of
1 B-NR diagnosis to evaluate for BAV, aortic root and
its branches, with annual surveillance imaging thereafter
ascending aortic dilation, aortic coarctation,
to monitor any dilated or dissected aortic or arterial seg- and other congenital heart defects.1-9
ments and imaging every 2 years when the initial imaging 2. In patients with Turner syndrome who are
is normal.1,2,5 Notably, the aorta and arterial branches in ≥15 years old, the use of the ASI (ratio
vascular Ehlers-Danlos syndrome may rupture (or dis- 1 B-NR
of aortic diameter [cm] to BSA [m2]) is
recommended to define the degree of
sect) even without significant dilation.1-3 aortic dilation and assess the risk of aortic
Medical therapy of vascular Ehlers-Danlos syndrome dissection.9,10,11
includes education, lifestyle modification, and avoidance 3. In patients with Turner syndrome without risk
of invasive procedures when possible.3,6 Studies of celip- factors for aortic dissection (Table 12), sur-
rolol, a beta blocker with vasodilatory properties, have sug- veillance imaging with TTE or MRI to evalu-
1 C-LD
ate the aorta is recommended every 5 years
gested a benefit in patients with vascular Ehlers-Danlos in children and every 10 years in adults, as
syndrome,7,8 but data were considered to be insufficient for well as before planning a p regnancy.9,10,11
US Food and Drug Administration (FDA) approval. In the 4. In patients with Turner syndrome and an ASI
absence of data showing efficacy in vascular Ehlers-Dan- 1 C-EO >2.3 cm/m2, surveillance imaging of the aorta
is recommended at least annually.9
los syndrome, other beta blockers are often prescribed,

Recommendations for Diagnostic Testing, Surveillance, and Surgical underestimate aortic dissection risk.9-11,13 Type A
CLINICAL STATEMENTS
Intervention for Aortic Dilation in Turner Syndrome (Continued ) aortic dissection in Turner syndrome may occur at
AND GUIDELINES
COR LOE Recommendations relatively small aortic diameters, likely reflecting the
5. In patients with Turner syndrome and risk typical patient’s short stature, so indexing of aortic
factors for aortic dissection (Table 12), surveil- diameter to body size (by calculating the ASI) is
1 C-EO lance aortic imaging at an interval depending performed when evaluating patients with Turner
on the aortic diameter, ASI, and aortic growth
rate is recommended (Figure 18).9 syndrome who are ≥15 years old.9,10 The ASI is cal-
culated by dividing the maximal aortic diameter, in
6. In patients with Turnery syndrome who are
≥15 years old and have an ASI of ≥2.5 cm/m2 centimeters, by the BSA, in meters squared. An ASI
2a C-LD plus risk factors for aortic dissection (Table >2.0 cm/m2 is considered to be abnormal, and an
12), surgical intervention to replace the aortic
ASI ≥2.5 cm/m2 is associated with an increased
root, ascending aorta, or both is reasonable.9,10
risk of aortic disection.9-11 Using a Turner syn-
In those without risk factors for aortic
dissection, surgical intervention to replace the
drome-specific z-score to assess for aortic dilation
2b C-EO is preferred in children <15 years old.
aortic root, ascending aorta, or both may be
considered. 3. Lifelong surveillance imaging of the aorta is used
to monitor for aortic dilation: For children with
Turner syndrome and no additional risk factors for
Synopsis
aortic dissection, reevaluation at 5-year intervals
Turner syndrome, which affects 1 in 2 500 liveborn girls, is appropriate; for adults with Turner syndrome
results from complete or partial loss of the second X and no additional risk factors for aortic dissec-
chromosome in all or some of the cells of an individual.9,12 tion, surveillance imaging of the aorta with TTE or
Approximately 50% of patients with Turner syndrome have MRI every 10 years is appropriate.9 Surveillance
cardiovascular defects that include BAV (15%–30%), imaging should also be performed before planned
aortic coarctation (7%–18%), and ascending aortic dila- pregnancy.9
tion (33%).9,12 Patients with Turner syndrome require car- 4. In Turner syndrome, the risk of aortic dissection
diac imaging to evaluate for congenital heart and aortic correlates with ASI,9 and an ASI ≥2.5 cm/m2 is
defects and to determine aortic diameters. Patients with associated with a significantly increased risk of
Turner syndrome are at increased risk of aortic dissec- aortic dissection. When the ASI approaches this
tion, with 85% occurring in the ascending and 15% in threshold, more frequent surveillance imaging is
the descending aorta.10,11,13 Risk factors for aortic dissec- appropriate to monitor aortic diameters.9
tion include aortic dilation, hypertension, BAV, and aortic 5. In Turner syndrome, risk factors for aortic dissec-
coarctation.9-11,13 Because Turner syndrome patients are tion include aortic dilation, BAV, aortic coarctation,
of short stature, type A aortic dissection may occur at and hypertension.9-11,13 When these risk factors
relatively small aortic diameters; consequently, indexing are present, surveillance imaging of the aorta is
the aortic diameter to body size (ie, calculating an ASI) is performed more frequently. For the patients with
recommended in monitoring the aorta.9,12,14 Turner syndrome who are ≥15 years old and have a
stable ASI of ≤2.3 cm/m2, surveillance imaging with
Recommendation-Specific Supportive Text TTE or MRI is performed every 2 to 3 years.9 In the
patients with Turner syndrome who are >15 years
1. Turner syndrome may be recognized in infancy or old with an ASI >2.3 cm/m2, at least annual sur-
childhood or, alternatively, go unrecognized until veillance imaging of the aorta is appropriate.9 The
adolescence or adulthood. On the diagnosis of frequency of imaging should be informed by aortic
Turner syndrome, a TTE and cardiac MRI are per- diameter, aortic growth rate, severity of hyperten-
formed to evaluate for associated congenital car- sion, and aortic valve function (Figure 18).9,12
diovascular abnormalities (BAV, aortic coarctation, 6. In patients with Turner syndrome, diameter thresh-
and others) and to measure aortic diameters.9,12 olds for prophylactic surgical replacement of
2. Because patients with Turner syndrome have short aneurysms of the aortic root/ascending aortic
stature, using absolute aortic diameters alone may replacement are based on retrospective series
and case studies.10,11,13 Data from registries of aor-
Table 12. Risk Factors for Aortic Dissection in Patients With tic dissection in Turner syndrome report that the
Turner Syndrome risk of dissection is significantly increased when
Aortic coarctation the ASI is ≥2.5 cm/m2.9-11,13 In addition to aortic
Aortic dilation
size, risk factors for aortic dissection in Turner syn-
drome include BAV, aortic coarctation, and hyper-
Bicuspid aortic valve
tension.9,11,13 However, decisions using indexed
Hypertension
calculations alone for aortic risk determination in

CLINICAL STATEMENTS
AND GUIDELINES
Figure 18. Suggested Aortic Monitoring Protocol for Girls and Women With Turner Syndrome Who Are ≥15 Years of Age.
*Surveillance frequency may vary depending on disease severity (ie, aortic valve dysfunction, severity of coarctation obstruction, hypertension,
and left ventricular hypertrophy). Color corresponds to Class of Recommendations in Table 2. ASI indicates aortic size index; BAV, bicuspid aortic
valve; CoA, coarctation of the aorta; HTN, hypertension; MRI, magnetic resonance imaging; and TTE, transthoracic echocardiography. Modified
from Silberbach et al.9 Copyright 2018, with permission from American Heart Association, Inc. Modified from Gravholt et al.12 Copyright 2017, with
permission from Bioscientifica Limited.
short-statured but obese patients with Turner syn- inherited in an autosomal dominant manner.1-4 In these
drome or those with low body weight relative to nsHTADs, baseline imaging of the thoracic aorta with
height may be less accurate. In such Turner syn- TTE, or with CT or MRI if the ascending aorta is not ade-
drome patients who are ≥15 years old, an absolute quately visualized by TTE, is recommended; surveillance
aortic diameter of >4.0 cm may be more accurate imaging is then performed annually, if stable. The arch
than ASI in determining the risk of aortic disection.9 and descending aorta may dilate, in which case surveil-
For patients with Turner syndrome who are <15 lance imaging of these segments is also performed. Less
years old, a Turner syndrome-specific z-score cal- frequent imaging may be considered when the aorta is
culation is appropriate to determine aortic risk and normal, depending on gene variant, age, and family his-
assess for surgical intervention.9,14 For patients with tory. Beta-blocker therapy is used to lessen hemody-
Turner syndrome without additional risk factors for namic stress on the aorta.
aortic dissection, few data exist on the degree of Specific features associated with each gene include:
aortic dilation that warrants surgical intervention.9 Patients with ACTA2 mutations primarily present with
type A or B aortic dissection, have aneurysms that
6.1.2.6. Pathogenic or Likely Pathogenic Variants involve the root and ascending aorta, and a subset of
in ACTA2, PRKG1, MYH11, MYLK, and LOX: pathogenic variants predispose to occlusive vascu-
Recommendations for Surveillance of Aorta, Medical lar diseases.2,5-7 Screening for coronary artery disease
Therapy, and Aortic Surgical Intervention and cerebrovascular disease is performed in individu-
Pathogenic variants in ACTA2, PRKG1, MYH11, MYLK, als with specific pathogenic variants.5,6,8,9 Patients with
and LOX confer a highly penetrant risk for TAD that is ACTA2 mutations can suffer type A aortic dissection at

Table 13. Surgical Thresholds for Prophylactic Aortic Root and Ascending Aortic Re-
CLINICAL STATEMENTS
placement in Nonsyndromic Heritable Thoracic Aortic Disease Based on the Genetic
AND GUIDELINES
Variant and Additional Risk Factors for Aortic Dissection
COR* LOE* Genetic Variant Risk Factors Aortic Diameter (cm)

2a C-LD ACTA2 No 4.5
2b C-EO ACTA2 Yes† 4.2
2b C-LD PRKG1 No 4.2
2b C-EO PRKG1 Yes† 4.0†
*Patient has risk factors for aortic dissection (family history of type A aortic dissection with minimal aortic en-
largement, aortic growth rate 0.3 cm/y) or significant valve disease requiring surgery.
†Earlier surgery may be considered in patients with a family history of type A aortic dissection in the setting of
no or minimal aortic dilation, aortic growth rate 0.3 cm/y, or at the patient’s request.
Colors correspond to COR and LOE in Table 2.
COR indicates class of recommendation; and LOE, level of evidence.
aortic diameters <4.5 cm, and consideration of surgery Recommendations for BAV Aortopathy (Continued )
at diameters <4.5 cm is informed by the presence of COR LOE Recommendations
additional risk factors.10 PRKG1-related HTAD can pres-
4. In patients with a BAV and a dilated aortic
ent in the late teens with type A or B aortic dissection root or ascending aorta, screening of all first-
without previous aortic enlargement11-13; patients with degree relatives by TTE is recommended to
MYH11 mutations primarily present with type A or B evaluate for the presence of a BAV, dilation
of the aortic root and ascending aorta, or
aortic dissection (type A aortic dissection may present at 1 C-LD
both; if the diameter and morphology of the
aortic diameters <5.0 cm), have aneurysms that involve aortic root, ascending aorta, or both cannot be
the root and ascending aorta, and may have peripheral assessed accurately or completely by TTE, a
cardiac-gated CT or MRI of the thoracic aorta
arterial disease4,14; patients with MYLK mutations pres- is indicated.7
ent at age >40 years with type A aortic dissection with
5. In patients with a BAV, screening of all first-
little previous enlargement of the aorta (median aortic degree relatives by TTE is reasonable to
diameter, 4.25 cm)3,15,16; patients with LOX mutations 2a B-NR evaluate for the presence of a BAV, dilation of
the aortic root and ascending aorta, or

can present with aortic root aneurysms, fusiform dilation
both.7-10
of the root and ascending aorta that can extend into the
aortic arch, or type A aortic dissection, and they may
have mild systemic features of Marfan syndrome.1,17,18 Synopsis
The decision regarding the timing of aortic repair in nsH-
TAD is based on the aortic diameter, age, family history, BAV is a common congenital valve condition affecting
and the presence or absence of additional risk factors approximately 1% of the population, with a 2 to 3:1 male-
(Table 13). to-female predominance.3 BAV most often occurs spo-
radically but may be inherited in an autosomal dominant
pattern with variable penetrance.5 A BAV may be iso-
6.1.3. BAV Aortopathy lated or associated with other congenital cardiovascular
Recommendations for BAV Aortopathy defects or aortopathy conditions.5 BAV is often associ-
Referenced studies that support the recommendations are ated with aortic valve dysfunction (stenosis or regurgita-
tion) and is at risk of infective endocarditis. Patients with a
COR LOE Recommendations BAV often have aortic dilation or aneurysms affecting the
1. In patients with a BAV, TTE is indicated to aortic root, ascending aorta, or both, with the prevalence
evaluate valve morphology and function, to
of aortic aneurysm increasing with age.11 Distinct aortic
evaluate the diameter of the aortic root and
1 B-NR
ascending aorta, and to evaluate for aortic dilation phenotypes have been described.1,12 Those with
coarctation and other associated cardiovascu- BAV and a dilated aorta are at risk for type A aortic dis-
lar defects.1-4
section.11,13,14 Patients with BAV require lifelong surveil-
2. In patients with a BAV, CT or MRI of the tho- lance imaging of the aorta, even after AVR.
racic aorta is indicated when the diameter and
1 C-LD morphology of the aortic root, ascending aorta,
or both cannot be assessed accurately or
completely by TTE.1
3. In patients with a BAV and either HTAD or 1. Aortic dilation in BAV may affect the aortic root,
1 C-LD
phenotypic features concerning for Loeys- the ascending aorta, or both. The ascending aorta
Dietz syndrome, a medical genetics evaluation is most commonly involved, and the dilation some-
is recommended.5,6
times extends up into the arch.1-3 The prevalence

of aortic dilation in BAV is reported from 20% to 5. The prevalence of a BAV in the relatives of a
CLINICAL STATEMENTS
84%, depending on the population studied and patient with a BAV ranges from 9% to 20%.8-10
AND GUIDELINES
the definition of aortic dilation.3,12 Patients with Family members of individuals with a BAV may also
BAV and aortic dilation are at risk for aortic dissec- have aortic dilation. A recent analysis found that
tion.3,11,13 The aortic root, ascending aorta, arch, and TTE screening of first-degree relatives of affected
proximal descending aorta should be imaged by patients, to detect both BAV and aortopathy, proves
TTE to evaluate for aortic valve function, aortic dila- to be cost-effective.18
tion, and aortic coarctation.1-4 Conversely, in other
patients undergoing TTE, a finding of unexplained 6.1.3.1. Routine Follow-Up of BAV Disease Aortopathy
aortic root, ascending aortic dilation, or both should
Recommendations for Routine Follow-Up of BAV Disease Aortopathy
prompt suspicion of an underlying BAV15; if TTE Referenced studies that support the recommendations are
of the aortic valve is inconclusive for BAV, cardiac summarized in the Online Data Supplement.
magnetic resonance, cardiac CTA, and TEE can be COR LOE Recommendations
used to better visualize the aortic valve and thereby
1. In patients with a BAV who have undergone
diagnose BAV. previous aortic valve repair or replacement
2. Cardiac-gated CT or MRI provides superior images and have a diameter of the aortic root,
ascending aortic, or both of ≥4.0 cm, lifelong
of the aortic root and ascending aorta when TTE is 1 B-NR
surveillance imaging of the aortic root and
inadequate to visualize the full extent of the proxi- ascending aorta by TTE, CT, or MRI is recom-
mal aorta. The choice between CT or MRI depends mended at an interval dependent on aortic
diameter and rate of growth.1-3
on patient characteristics, institutional expertise,
renal function, affordability, and radiation exposure 2. In patients with a BAV and a diameter of the
aortic root, ascending aorta, or both of ≥4.0
concerns.16 cm, lifelong surveillance imaging of the aortic
1 C-LD
3. Certain types of HTAD have an increased preva- root and ascending aorta by TTE, CT, or MRI
lence of BAV. For example, BAV is present in ∼10% is recommended at an interval dependent on
aortic diameter and rate of growth.4,5
of patients with Loeys-Dietz syndrome (attribut-
able to pathogenic variants in TGFBR1, TGFBR2,
SMAD3, TGFB2, and TGFB3),6 and HTAD attrib- Synopsis
utable to pathogenic variants in NOTCH1, ACTA2, Patients with BAV, with or without aortic dilation, require
MAT2A, SMAD6, and LOX also have an increased
lifelong surveillance of the aortic root and ascending

prevalence of BAV.5,6 Importantly, most patients with aorta because of risk of late aortic growth. The degree of
BAV and TAAs who undergo genetic testing will aortic dilation and the progression of aortopathy may be
not be found to have a pathologic genetic variant, greater in patients with aortic root phenotype and those
even when their condition is familial. Nevertheless, with predominant AR.3,6 Progressive aortic growth may
when the condition is familial, a medical geneticist occur after AVR.3,7
or specialist in genetic aortopathy should evaluate,
counsel, and genetically test patients with BAV and
aortopathy.17 Recommendation-Specific Supportive Text
4. Both BAV and aortic root and ascending aortic dila- 1. Patients with BAV who have undergone previous
tion may be familial,7 and the inheritance patterns for isolated AVR or aortic valve repair remain at risk
familial BAV and aortopathy are consistent with an for future aortic dilation and dissection. In a series
autosomal dominant pattern with incomplete pen- of 1 286 patients who underwent isolated AVR for
etrance.8-10 In families with BAV and aortic root and BAV from 1960 to 1995, the 15-year freedom
ascending aortic dilation, obligate carriers may have from aortic events (aortic dissection, aortic aneu-
BAV, aortic dilation, both, or neither.7 In families with rysm of >5.0 cm, or aortic aneurysm surgery) was
BAV and aortic root and ascending aortic dilation, 89% but was lower for those with documented
screening of the first-degree relatives (parents, sib- aortic dilation at baseline compared with those with
lings, and children) with TTE to evaluate for BAV and normal diameters (85% versus 93%; P=0.001).8
aortic dilation identifies affected members. If a family Patients with BAV who have undergone isolated
member is discovered to have a BAV, aortic dilation, AVR for aortic stenosis and have only mild-to-mod-
or both, cascade evaluation of other related family erate aortic dilation are at low risk for adverse aor-
members is then indicated. Because families with tic events at 15-year follow-up,3,9 whereas those
BAV and aortic dilation may have members with aor- who underwent AVR for predominant AR and
tic root and ascending aortic dilation in the absence those with predominant dilation of the aortic root
of a BAV, if the ascending aorta is not adequately (“root phenotype”) are at higher risk for adverse
assessed by TTE, a CT or MRI should be performed aortic events during follow-up. Among 56 patients
to fully evaluate the size of the ascending aorta. with BAV who underwent isolated AVR for AR and

had concomitant aortic root dilation (4.0–5.0 cm), Synopsis
CLINICAL STATEMENTS
adverse aortic events occurred in 34% of patients
The timing of surgery to replace the aorta in BAV disease
AND GUIDELINES
during follow-up.4 Patients with BAV who undergo
depends on the morphology and diameter of the aorta,
isolated AVR for AR are at higher risk for late aortic
aortic valve function, rate of aortic growth, family history,
dissection than patients who underwent AVR for
patient characteristics, patient wishes, and the expertise
aortic stenosis.10
of the surgeon and institution.1,7
2. In a prospective study of 90 adults with BAV, the
mean increase in ascending aortic diameter was 0.47
mm/y (range, 0.2–2.3 mm/y) over a 4.8-year follow- Recommendation-Specific Supportive Text
up.11 Surveillance imaging can document current aor-
1. Patients with a BAV without significant aortic dila-
tic diameters and permit calculation of aortic growth
tion are at low risk for type A aortic dissection,3,8
rates.2,6 Among a cohort of adult patients with BAV
whereas those patients with BAV and aneurysmal
(mean age, 55±17 years) without a TAA at baseline
dilation of the aortic root, ascending aorta, or both
(ie, the baseline aortic diameter was <4.5 cm), 13%
have a significantly increased risk of aortic dis-
went on to develop a TAA at 14±6 years after diag-
section.5,8 The risk of aortic dissection rises with
nosis, and the 25-year risk of TAA was 26%.12 For
increasing aortic diameter, and there are “hinge
many adults, an aortic root, ascending aortic, or both
points” when the ascending aorta reaches diam-
diameter ≥4.0 cm is considered dilated and should
eters >5.25 cm to 5.75 cm.9
therefore be monitored over time with surveillance
2. Indexing the maximal aortic root or ascending aortic
imaging to detect progressive dilation.
diameter to height is predictive of aortic dissection
6.1.3.2. BAV Aortopathy Interventions: Replacement of risk and therefore informs surgical thresholds.3,4
the Aorta in Patients With BAV Moreover, when comparing long-term outcomes
in patients with BAV and aortic root or ascending
Recommendations for BAV Aortopathy Interventions: Replacement of
the Aorta in Patients With BAV
aortic dilation, survival was significantly better for
Referenced studies that support the recommendations are those with an aortic cross-sectional area (in cm2)
summarized in the Online Data Supplement. to height (in meters) ratio of ≥10 who underwent
COR LOE Recommendations elective prophylactic aortic repair compared with
1. In patients with a BAV and a diameter of those who did not undergo elective repair.3
the aortic root, ascending aorta, or both 3. There are additional risk factors for aortic dissec-
1 B-NR of ≥5.5 cm, surgery to replace the aortic
tion that may inform aortic surgical thresholds in
root, ascending aorta, or both is recom-
mended.1-3 patients with a BAV. A family history of aortic dis-
2. In patients with a BAV and a cross-sectional
section10 and rapid aortic growth of ≥0.3 cm/y
aortic root or ascending aortic area (cm2) to (when measured similarly with same technique) are
height (m) ratio of ≥10 cm2/m, surgery to both risk factors for aortic dissection. Patients with
2a B-NR replace the aortic root, ascending aorta, or
both is reasonable, when performed by expe-
BAV and aortic coarctation have been reported to
rienced surgeons in a Multidisciplinary Aortic be at increased risk of aortic dissection,11 although
Team.3,4 in a recent report of 499 patients with BAV (mean
3. In patients with a BAV, a diameter of the aortic age, 40±16 years), of whom 24% also had aortic
root or ascending aorta of 5.0 cm to 5.4 cm, coarctation, there was no difference in adverse aor-
and an additional risk factor for aortic dissec-
2a B-NR tion (Table 14), surgery to replace the aortic tic events between those with or without coarcta-
root, ascending aorta, or both is reasonable, tion.12 Patients with dilation of the aortic root (“root
when performed by experienced surgeons in a phenotype”) represent 10% to 20% of patients
Multidisciplinary Aortic Team.1,5
with BAV and aortopathy and may have more rapid
4. In patients with a BAV who are undergoing
surgical aortic valve repair or replacement,
aortic growth and an increased risk of aortic com-
and who have a diameter of the aortic root plications.13,14 Because surgical aortic root replace-
2a B-NR
or ascending aorta of ≥4.5 cm, concomitant ment (and VSRR) is more complex than ascending
replacement of the aortic root, ascending
aorta, or both is reasonable, when performed
aortic replacement, shared decision-making is
by experienced surgeons in a Multidisciplinary often used when evaluating the risks and benefits
Aortic Team.1,6
5. In patients with a BAV, a diameter of the aortic
root or ascending aorta of 5.0 cm to 5.4 cm, Table 14. Risk Factors for Aortic Dissection
no other risk factors for aortic dissection
Family history of aortic dissection
(Table 14), and at low surgical risk, surgery
2b B-NR
to replace the aortic root, ascending aorta, or Aortic growth rate 0.3 cm/y
both may be reasonable, when performed by
Aortic coarctation
experienced surgeons in a Multidisciplinary
Aortic Team.1,2,5 “Root phenotype” aortopathy

of elective aortic root replacement at aortic diam- Table 15. Risk Factors for Abdominal Aortic Aneurysm
CLINICAL STATEMENTS
eters <5.5 cm.1,2,5,6 Strong Risk Factors Additional Risk Factors

AND GUIDELINES
4. In patients with a BAV and indications for aortic

Smoking history Hypertension
valve intervention for stenosis or regurgitation,
Older age Hyperlipidemia
the data are limited regarding the degree of aor-
tic dilation that warrants replacement of the aortic Male sex White race
root, ascending aorta, or both at the time of AVR. Family history of abdominal aortic Inherited vascular connective
aneurysm tissue disorder
Patients with a long life expectancy, low surgical
risk, or with the root phenotype and predominant Atherosclerotic cardiovascular
disease
AR may benefit from concomitant prophylactic aor-
tic repair. Conversely, for patients at higher surgi-
cal risk, especially those with aortic stenosis and Lifetime risk for AAA is 8.2% in men and 10.5% in cur-
only moderate ascending aortic dilation, the risks rent smokers.11 At least 10% to 25% of patients with
of concomitant aortic repair may not be warranted. AAA have a family member with the same condition,2
5. Limited data are available on the risk of aortic and AAA may occur concomitantly with thoracic aortic
dissection among those with a BAV and aortic aneurysmal disease, especially in some genetic aortop-
aneurysm diameter of 5.0 cm to 5.4 cm.5,15 Patient- athies.11 Inflammatory aortitis is a rare cause of AAA13,14
related characteristics and surgical expertise may (see Section 9.1, “Inflammatory Aortitis – Diagnosis and
inform the timing of surgery, especially in low-risk Treatment of Takayasu Arteritis and Giant Cell Arteritis
patients with BAV and aortic aneurysms of 5.0 cm (GCA)”). The growth of AAA is nonlinear, with a mean
to 5.4 cm.1,2,5,6 rate of 2.6 mm/y for AAA <5.0 cm,15 and may accel-
erate in the setting of smoking or a family history of
6.2. AAA: Cause, Risk Factors, and Screening AAA,16,17 and smoking may have a greater impact on
growth in women than in men.4 Ultrasound screening
Recommendations for AAA: Cause, Risk Factors, and Screening
should be targeted toward those at the greatest risk for
summarized in the Online Data Supplement. AAA and growth (Figure 19), with the goal of prevent-
COR LOE Recommendations ing rupture and associated mortality.1,5,6
1. In men who are ≥65 years of age who have
1 B-R ever smoked, ultrasound screening for

detection of AAA is recommended.1
2. In men or women who are ≥65 years of 1. Older age, male sex, and smoking are independent,
1 C-LD
age and who are first-degree relatives of strong risk factors for the development of AAA.9-11
patients with AAA, ultrasound screening for
detection of AAA is recommended.2,3
Smoking history is defined as lifetime use of ≥100
cigarettes, but risk attributable to smoking varies
3. In women who are ≥65 years of age who
2a C-EO have ever smoked, ultrasound screening for
significantly depending on use, with lowest risk of
detection of AAA is reasonable.4,5 AAA in those who have lower versus higher pack-
4. In men or women <65 years of age and year history.9 Based on a meta-analysis of ran-
2b C-LD
who have multiple risk factors (Table 15) or domized clinical trials inclusive of nearly 125 000
a first-degree relative with AAA, ultrasound mostly male patients, screening of men ≥65 years
screening for AAA may be considered.5,6
of age reduced long-term AAA-related mortal-
5. In asymptomatic men or women >75 years
3: No who have had a negative initial ultrasound
ity (4 RCTs: OR, 0.65; 95% CI, 0.57–0.74) and
B-NR AAA-related ruptures (4 RCTs: OR, 0.62; 95% CI,
Benefit screen, repeat screening for detection of
AAA is not recommended.1 0.55–0.70) over 12 to 15 years.1 In a recent pop-
ulation-based study (of both men and women) in
the United Kingdom, two-thirds of the acute AAA
Synopsis events occurred in those ≥75 years of age; con-
Although AAA share risk factors with typical athero- sequently, screening to elderly patients should be
sclerosis, AAA are histopathologically distinct and char- offered, provided they would benefit from potential
acterized by medial degeneration of the aortic wall.7 aortic repair.18
Most AAA develop an intraluminal thrombus that con- 2. Having a first-degree relative with AAA is a
tributes to ongoing wall degradation via oxidative stress, well-known and well-established risk factor for
smooth muscle cell apoptosis, proteolysis of the extra- development of AAA.2,3 Small cohort studies of
cellular matrix, and adventitial inflammation.8 A complex ultrasound screening in relatives of those with
interplay of hereditary and environmental risk factors AAA have identified an overall prevalence of new
contributes to AAA, most notably older age, male sex, AAA of 10% to 20%, with the highest prevalence
smoking, and a positive family history (Table 15).2,3,9-12 of 25% found among brothers. Indeed, the overall

CLINICAL STATEMENTS
AND GUIDELINES
Figure 19. Algorithm for Identifying Patients to Screen for Abdominal Aortic Aneurysm.
Colors correspond to Class of Recommendations in Table 2. AAA indicates abdominal aortic aneurysm.
lifetime prevalence of AAA is estimated to be 32% of age.5 Notably, in patients <65 years, data are
in brothers of those with AAA,2 suggesting the lacking on the mortality benefit of AAA screening.
need for a targeted and individualized screening 5. Some patients may develop AAA after the age
approach for those who already meet age criteria of 75 years even if they had an initial negative
within families. screen between the ages of 65 and 75 years.
3. Select women may be at risk for AAA and related Although somewhat limited, data from cohort stud-
complications.5 Randomized trials and large obser- ies suggest long-term AAA-related mortality is low
vational studies that evaluate outcomes of screen- among patients with an initial negative screening
ing for AAA by ultrasound in women are lacking, ultrasound who had a subsequent AAA detected
as female sex has not been proven an indepen- on repeat screening after the age of 75 years.1
dent risk factor for AAA,11 and overall prevalence However, select patients at low surgical risk who
of AAA in women is lower than in men. However, may have had borderline enlarged abdominal aorta
the risk of AAA may be potentiated by smoking in measurements on initial screening and who have
women; in 1 study, smoking was associated with a significant AAA risk factors (Table 15) may be con-
15-fold increased risk of AAA among women (rel- sidered for repeat screening on an individualized
ative risk, 15.0; 95% CI, 13.2–17.0) versus 7-fold basis.
among men (relative risk, 7.3; 95% CI, 6.4–8.2).4
Practical implementation and outcomes of screen-
6.3. Growth and Natural History of Aortic
ing in women remain uncertain and warrant further
study. Aneurysms
4. Select patients <65 years of age may be at Aortic aneurysm growth and natural history is variable
increased risk of AAA rupture, and data suggest and dependent on the underlying etiology, such as HTAD
a significant proportion of those undergoing repair (eg, Marfan syndrome and Loeys-Dietz syndrome), BAV,
for ruptured AAA did not meet the standard criteria or sporadic aortic disease without a known genetic basis.
for screening based on age.5,6 In a large study from There is significant evidence that aortic diameter cor-
the National Inpatient Sample, 10 603 of 25 777 relates with aortic dissection, aortic rupture, and mortal-
patients with ruptured AAA (24%) were <65 years ity.1-3 In patients with Marfan syndrome, the mean rate

of growth of the aortic root has been reported to be Recommendations for BP Management in TAA (Continued )
CLINICAL STATEMENTS
0.26 cm/y (range, 0.13–0.35 cm/y), with a tendency for COR LOE Recommendations
AND GUIDELINES
larger aneurysms (>6.0 cm) to grow faster (0.46 cm/y).4 2. In patients with TAA, regardless of cause and
Patients with BAV have a slower rate of aortic growth, with in the absence of contraindications, use of
2a C-LD
a root predominant phenotype growing at 0.06 cm/y (0.6 beta blockers to achieve target BP goals is
reasonable.1,4,5
mm/y) and the more common ascending aortic pheno-
3. In patients with TAA, regardless of etiology
type at 0.03 cm/y (0.3 mm/y).5 Moreover, among those and in the absence of contraindications,
2a C-EO
with tricuspid aortic valves and sporadic ascending aortic ARB therapy is a reasonable adjunct to beta-
blocker therapy to achieve target BP goals.6
dilation, the mean rate of growth is even slower, as low as
0.01 cm/y (0.1 mm/y).6 Aortic arch aneurysm growth has
been reported to be 0.25 cm/y.7 The mean growth rate Synopsis
of descending and TAAA has been reported to be 0.19
The goal of BP control in TAA is to slow growth and
cm/y, with rates increasing as the diameter increases.8
prevent aortic dissection, as well as to reduce nonaor-
The mean rate of growth of AAA is 0.26 cm/y, with larger tic cardiovascular events, such as myocardial infarction
aneurysms growing as fast as 0.5 cm/y.9 and stroke. Uncontrolled hypertension increases the risk
for aortic dissection,7 so achieving a SBP goal of ≤130
6.4. Medical Management of Sporadic and mm Hg and a DBP goal of ≤80 mm Hg, with the use of
antihypertensive therapy in those with hypertension and
Degenerative Aortic Aneurysm Disease
TAA, is advised. Although data are limited, achieving a
The primary goals of medical therapy in sporadic and more intensive SBP goal of <120 mm Hg, if tolerated,
degenerative thoracic and abdominal aneurysmal dis- may have added benefit in selected patients and who
ease are to reduce growth rates, the risk of aortic-related are not undergoing surgical repair.4 There has been sig-
mortality, and the need for aortic repair; a secondary goal nificant progress in understanding the molecular basis of
is to decrease the risk of nonaortic cardiovascular events, aneurysmal development and growth,8 and a number of
given the multiple shared risk factors between aneurys- clinical trials have explored the effects of beta-blocker
mal and atherosclerotic disease.1,2 Lifestyle modification, and ARB therapy.9 A summary of these trials specific
including smoking cessation and blood pressure (BP) to genetic aortopathies is covered in detail in Section
control, improves overall cardiovascular health and may 6.1.2, “Genetic Aortopathies.” However, as the molecular
be beneficial to patients with aortic aneurysmal disease. mechanisms of aneurysm formation may have similari-
Pharmacotherapy specific to the treatment of aortic ties between aneurysm patients with and without Marfan
syndrome, data from these studies may be extrapolated
disease includes the use of selected antihypertensives
in guiding the treatment of aortic disease of other causes.
(especially beta blockers and ARBs) that may mitigate
Further clinical trials are clearly needed.
the proteolysis pathways, leading to medial degenera-
tion and reducing of sheer stress on the aortic wall, as
well as the use of statins, which may target inflammatory
and atherosclerotic pathways.3 Outcomes data from clini-
cal trials of medical therapy in aortic aneurysms broadly 1. No randomized clinical trials have evaluated the
are limited, as most trials have focused on cohorts of optimal threshold to which BP should be lowered
patients with either Marfan syndrome or AAA. Conse- in patients with TAA to reduce the risk of aortic
quently, correlations may be imprecise when applied to complications (aortic growth, aortic dissection,
other populations. or aortic rupture). Updated hypertension guide-
lines from the ACC and AHA suggest all patients
6.4.1. Medical Therapy and Risk Factor Modification with clinical cardiovascular disease should have a
in Sporadic TAA target SBP <130 mm Hg, DBP <80 mm Hg, or
both.1 Evidence supports aggressive BP lower-
6.4.1.1. BP Management in Sporadic TAA ing to reduce vascular-related adverse events and
Recommendations for BP Management in TAA all-cause mortality.2,3 Data from SPRINT (Systolic
Blood Pressure Intervention Trial) showed that
intensive BP control to a SBP <120 mm Hg, if
tolerated, reduced cardiovascular events by 25%
1. In patients with TAA and an average systolic
BP (SBP) of ≥130 mm Hg or an average
and all-cause mortality rate by 27% in patients
1 B-NR diastolic BP (DBP) of ≥80 mm Hg, the use of without diabetes over a median of 3.3 years, com-
antihypertensive medications is recommended pared with a control with a SBP target of <140
to reduce risk of cardiovascular events.1-3
mm Hg.10,11

2. Prospective data on the positive effects of beta reduction in low-density lipoprotein (LDL) for patients
CLINICAL STATEMENTS
blockers in TAA based on cause are limited, with <75 years of age with clinical atherosclerotic cardio-
AND GUIDELINES
the most robust evidence derived from cohort vascular disease was recommended to prevent adverse
studies of those with Marfan syndrome (see events (eg, myocardial infarction and stroke). If a high-
Section 6.1.2.2, “Marfan Syndrome”). In a small, intensity statin cannot be used, a moderate-intensity
open-label, randomized clinical trial of prophylac- statin can be initiated.1 According to evidence from
tic propranolol (mean dose, 212±68 mg/d) versus animal studies in nonatherosclerotic-related TAA, statin
placebo in adolescents and adults with Marfan therapy may prevent growth and adverse remodeling.7
syndrome, beta-adrenergic blocking drugs slowed However, its use in clinical practice at this time is not
aortic root growth and reduced aortic complica- fully understood.
tions.5 In a study of 155 children <12 years of age
with Marfan syndrome, beta blockers decreased
the rate of aortic root growth by 0.16 mm/y, on Recommendation-Specific Supportive Text
multivariate analysis.4 In the “2017 Hypertension 1. Atherosclerotic aortic aneurysms increase risk
Clinical Practice Guideline,” beta-blocker therapy is of stroke and myocardial infarction and thus are
the recommended first-line antihypertensive drug considered a coronary artery disease equivalent
therapy for patients with hypertension and TAD.1 according to NCEP ATP III (National Cholesterol
3. A meta-analysis of 1 510 randomized patients Education Program Adult Treatment Panel III), with
evaluating the effect of ARBs on TAA associated a >20% risk of an event within 10 years.8 The
with Marfan syndrome showed slower growth of “2016 AHA/ACC Guideline on the Management
the aortic root with the use of ARBs compared with of Patients With Lower Extremity Peripheral Artery
placebo; in a direct comparison with beta-blocker Disease”9 gave a COR 2a recommendation for use
therapy, there was no difference in aortic growth; of high-intensity statin in patients with noncoronary
and the combination of beta blocker plus ARB led atherosclerotic disease to achieve an LDL goal
to slower aortic growth than beta blockers alone.6 of <70 mg/dL. From the Cholesterol Treatment
In the Jikei Heart Study,12 which supported the use Trialists’ Collaboration, when combining data from
of ARBs in the 2010 ACC/AHA thoracic aortic 5 RCTs of 39 612 patients over median 5.1 years,
disease guidelines, Japanese patients on an anti- more intensive cholesterol lowering in patients with
hypertensive drug regimen that included valsartan ASCVD reduced major cardiovascular events by an
had a lower rate of adverse cardiovascular events, additional 15% beyond what was achieved with
including mortality and, in particular, a reduction less intensive cholesterol lowering.1,2 In patients
was showed in the incidence of aortic dissection. with sporadic or genetically mediated aneurysms, if
However, this study was subsequently retracted13 there is concomitant atherosclerotic disease else-
and, consequently, the LOE for use of ARBs has where, then statin therapy is still reasonable.
been downgraded to C from B. 2. It has long been hypothesized that the pleiotropic
effects of statins may be beneficial in preventing
6.4.1.2. Treatment of TAA With Statins
the adverse vascular wall remodeling associated
Recommendations for Treatment of TAA With Statins with TAAs, thereby slowing growth, regardless of
COR LOE Recommendations cause and whether associated atherosclerosis is
1. In patients with TAA and imaging or clinical present. Animal studies have shown a reduction in
2a C-LD evidence of atherosclerosis, statin therapy at thoracic aneurysm growth with statin therapy, possi-
moderate or high intensity is reasonable.1,2
bly via regulation of MMP activity.7,10 A study of 1348
2. In patients with TAA who have no evidence of
patients with thoracic aortic ectasia showed, in a
2b C-LD atherosclerosis, the use of statin therapy may
be considered.3-6 propensity-matched analysis, a possible benefit with
statin therapy in the reduction of aortic growth rate
as well as aortic complications.3,11 In a retrospective
Synopsis study that included 2267 patients who underwent
Clinical atherosclerotic cardiovascular disease (ASCVD) TEVAR for aneurysmal disease, 1148 (64%) of
encompasses aortic aneurysms of atherosclerotic ori- whom had been treated with a statin preoperatively,
gin. For the purpose of this guideline, we also define preoperative statin therapy was associated with sig-
aortic aneurysm with concomitant PAU or visualized nificantly lower perioperative complication rates and
atheroma as atheromatous aortic disease, even in the 5-year mortality.12 A possible benefit of statins in
presence of a genetic syndrome, given some causes prevention of adverse aortic-related outcomes was
have shared risk factors with ASCVD. Based on the also showed in a small cohort study, and slowing
AHA/ACC “2018 Guideline on the Management of of aortic growth is suggested by 2 small studies in
Blood Cholesterol,”1 a high-intensity statin for >50% patients with BAV and aortopathy.6,8

6.4.1.3. Smoking Cessation in TAA Other Atherosclerotic Vascular Disease”1 recommend

CLINICAL STATEMENTS
Recommendation for Smoking Cessation in TAA use of low-dose aspirin (75–162 mg/d) in patients with
AND GUIDELINES
COR LOE Recommendation

atherosclerotic aortic disease. Even in the absence of
TAA, this remains true in other atherosclerotic aortic dis-
1. In patients with TAA who smoke cigarettes,
1 C-LD
smoking cessation efforts are recommended.1,2 eases, such as high-grade atheroma, PAU, or both.
Synopsis Recommendation-Specific Supportive Text

Smoking cessation and avoidance of secondhand smoke 1. In the SPARC (Stroke Prevention: Assessment of
exposure is considered a healthy lifestyle modification in Risk in a Community) study, aortic atherosclerosis
patients with TAAs, regardless of cause. Many patients was associated with coronary artery disease (OR,
cared for in cardiovascular clinical practices have interest 2.99; 95% CI, 1.47–6.10; P=0.003).2 In turn, in
in smoking cessation; thus, implementation of an effec- the presence of coronary artery disease, aspirin
tive strategy using the 5 A’s (Ask, Advise, Assess, Assist, has long been recommended to reduce the risk
and Arrange) is worthwhile, along with a referral to dedi- of cardiovascular events, including stroke, death
cated programs, use of app-based tools, pharmacother- caused by coronary artery disease, and myocardial
apy (which includes nicotine replacement, bupropion, or infarction.1
varenicline), or both.1-3 Although the use of e-cigarettes
6.4.2. Medical Therapy and Risk Factor Modification
has been shown to be an effective strategy in smoking
in AAA
cessation,4 the efficacy and, importantly, safety of e-cig-
arette use in patients with TAA is not well understood. 6.4.2.1. BP Management in AAA
Recommendation for BP Management in AAA
Referenced studies that support the recommendation are summarized
Recommendation-Specific Supportive Text in the Online Data Supplement.
1. There are many validated options for smoking ces- COR LOE Recommendation
sation for patients who continue to smoke and 1. In patients with AAA and an average SBP of
≥130 mm Hg, or an average DBP of ≥80 mm
have TAA.1-3 Although no randomized clinical trials 1 B-NR Hg, the use of antihypertensive medication is
have evaluated the effect of smoking cessation on recommended to reduce risk of cardiovascular
outcomes in TAA, smoking is a risk factor for TAA events.1-3

expansion and, among those with atherosclerotic
aortic disease, smoking cessation reduces the rates
Synopsis
of myocardial infarction and death.5,6 The use of
e-cigarettes, although an effective smoking cessa- Reducing cardiovascular events such as myocardial
tion tool, has not been shown to be safe when used infarction and stroke, as well as preventing aneurysm
in patients with vascular disease, including TAA; fur- growth and rupture, are the main goals in antihyper-
ther, small studies suggest that the flavoring chemi- tensive therapy in AAA. Uncontrolled hypertension is a
cals in e-cigarettes may have an adverse effect known risk factor for aortic rupture and dissection; there-
on vascular endothelial function and relaxation via fore, achieving an SBP goal of <130 mm Hg, and a DBP
nitric oxide and cyclic guanosine monophosphate- goal of <80 mm Hg with the use of antihypertensive
mediated signaling.7,8 therapy in those with hypertension and AAA can reduce
adverse clinical outcomes, and some patients may ben-
6.4.1.4. Antiplatelet Therapy in TAA efit from more intensive lowering with an SBP goal of
<120 mm Hg.4 The most robust evidence of antihyper-
Recommendation for Antiplatelet Therapy in TAA
tensive therapy in AAA is for beta blockers and agents
that alter the renin angiotensin system; however, in pro-
1. In patients with atherosclerotic TAA and con- spective clinical trials in humans, no specific agent has
comitant aortic atheroma or PAU, the use of
2a C-EO
low-dose aspirin is reasonable, unless contra- been proven to inhibit AAA growth.
indicated.1,2

Synopsis 1. Updated hypertension guidelines from the ACC
Aortic aneurysms of atherosclerotic origin are consid- and AHA suggest all patients with clinical cardio-
ered a coronary artery disease equivalent according to vascular disease have a target SBP of <130 mm
the NCEP ATP III, with a >20% risk of an event within Hg and/or DBP <80 mm Hg.1 Evidence supports
10 years.3 The 2006 updated “AHA/ACC Guidelines for aggressive BP lowering to reduce vascular-related
Secondary Prevention for Patients With Coronary and adverse events and all-cause mortality.2,3 A more

intensive SBP goal of <120 mm Hg, if tolerated, ruptured AAA (46.1% versus 59.3%, respec-
CLINICAL STATEMENTS
may have added benefit in select patients without tively; adjusted mortality rate, 0.80; 95% CI, 0.68–
AND GUIDELINES
diabetes and who are not undergoing surgical aor- 0.95).10 Retrospective data from 5 892 patients
tic repair. However, data are limited to the single enrolled in the EUROSTAR (EUROpean collabo-
randomized SPRINT,4 which showed that intensive rators on Stent-graft Techniques for abdominal
BP control to SBP <120 mm Hg reduced cardio- aortic Aneurysm Repair) registry showed improved
vascular events by 25% and all-cause mortality by survival over 5 years of follow-up associated with
27% in patients without diabetes over a median of statin use (81% for statin users versus 77% for
3.3 years, compared with a control with an SBP tar- nonusers; P=0.005).11 Additionally, in a large reg-
get of <140 mm Hg.4,5 istry-based study of 37 950 patients undergoing
repair of AAAs, those not previously on statin ther-
6.4.2.2. Treatment of AAA With Statins apy who were started on statin before discharge
Recommendations for Treatment of AAA With Statins had improved 1- and 5-year survival compared with
Referenced studies that support the recommendations are those who remained off statin therapy.12
2. In a recent meta-analysis, in broad cohorts of
patients with AAA, statin therapy was associ-
1. In patients with AAA and evidence of aortic ated with slower aneurysm growth, reduced risk
1 B-NR atherosclerosis, statin therapy at moderate or
high intensity is recommended.1-3
of rupture, and lower 30-day mortality after aor-
tic repair4; because atherosclerosis is so preva-
2. In patients with AAA but no evidence of
2b C-LD atherosclerosis, statin therapy may be lent among patients with AAA, it was not possible
considered.4,5 to distinguish whether statin therapy benefited
those without atherosclerosis equally. The mech-
anisms by which statins improve survival in AAA
Synopsis warrant further study, as in 1 single prospective
ASCVD includes noncoronary atherosclerotic disease cohort study of patients undergoing long-term
such as peripheral artery disease (PAD) and AAA.6 For surveillance, statins had not been shown to slow
the purpose of this guideline, we define abdominal AAA the growth rate of AAA or have direct effect
of atherosclerotic cause as those with visualized aortic on matrix metalloproteinase-9 or interleukin-6
wall atheroma, penetrating aortic ulceration either within concentrations.5
the aneurysm or at another site along the aorta, or both,

with a limitation being that many patients with geneti-
cally mediated AAA (see Section 6.2, “AAA: Cause, Risk 6.4.2.3. Smoking Cessation in AAA
Factors and Screening”) may have concomitant ASCVD. Recommendation for Smoking Cessation in AAA
The AHA/ACC “2018 Guideline on the Management of COR LOE Recommendation

Blood Cholesterol,”9 using evidence from the Cholesterol 1. In patients with AAA who smoke cigarettes,
Treatment Trialists’ Collaboration, recommended a high- 1 C-LD smoking cessation efforts are recom-
mended.1-4
intensity statin or, in some cases, moderate-intensity, for
patients with clinical ASCVD. A 50% reduction in LDL
for such patients <75 years of age can prevent adverse
events, such as myocardial infarction and stroke.7 Ongo-
Synopsis
ing study is needed to evaluate clinical outcomes of Cigarette smoking is a major risk factor for the develop-
statin therapy in patients with nonatherosclerotic AAA. ment, growth, and complications of AAA (see Section 6.2,
“AAA: Cause, Risk Factors, and Screening”) and increases
the risk for adverse clinical outcomes in the perioperative
Recommendation-Specific Supportive Text setting for AAA repair. Healthy lifestyle modifications in
1. AAA of atherosclerotic cause is considered a ASCVD, such as atherosclerotic AAA and PAU, include
coronary artery disease equivalent, with a >20% smoking cessation and avoidance of secondhand smoke.
risk of a cardiovascular event within 10 years.8 Effective strategies in those patients motivated to quit
Intensive cholesterol lowering in patients with smoking use the 5 A’s (Ask, Advise, Assess, Assist, and
ASCVD reduces major cardiovascular events by Arrange) and may include dedicated multidisciplinary pro-
an additional 15% beyond what is achieved with grams, app-based tools, or pharmacotherapy with nicotine
less intensive cholesterol lowering.7,9 From a large replacement, bupropion, varenicline, or all 3.1-3 Although
Danish case-control study, current, but not a his- e-cigarette use has been shown to be an effective strat-
tory of previous, statin use was associated with egy in smoking cessation,4 the efficacy and safety of its
decreased 30-day mortality rates in patients with use in patients with AAA has not been shown.

Recommendation-Specific Supportive Text repair at diameters of 4.0 cm to 4.9 cm but not for
CLINICAL STATEMENTS
aneurysms <4.0 cm. However, evidence from the

1. No randomized clinical trials have assessed the
AND GUIDELINES
Danish National Registry of Patients study of 4010

effect of smoking cessation on clinical outcomes in
age- and sex-matched subjects with AAA1 showed
patients with AAA, given the inherent design limi-
an increased case-fatality rate associated with
tations of such an intervention. Current guidelines
preadmission aspirin use in ruptured AAA (66%
and recommendations that encourage counseling
in users versus 57% in nonusers; adjusted mor-
and pharmacological interventions in patients moti-
tality rate ratio, 1.16; 95% CI, 1.06–1.27); there
vated to quit are derived from the fact that cigarette
was no association between aspirin use and the
smoking is considered the largest modifiable risk
risk of AAA rupture (adjusted OR, 0.97; 95% CI,
factor for AAA. The use of e-cigarettes is effective
0.86–1.08).
in smoking cessation; however, given its association
with adverse vascular remodeling, more evidence 6.4.3. Surveillance for Medical Management
on its safety in patients with AAA is needed.
6.4.3.1. Surveillance of Thoracic Aortic Dilation and
Aneurysm
6.4.2.4. Antithrombotic Therapy in AAA
Recommendations for Surveillance of Thoracic Aortic Dilation and
Recommendation for Antithrombotic Therapy in AAA Aneurysm
COR LOE Recommendation COR LOE Recommendations
1. In patients with AAA with concomitant ather- 1. In patients with a dilated thoracic aorta, a
2b C-LD oma and/or PAU, the use of low-dose aspirin TTE is recommended at the time of diag-
may be considered, unless contraindicated.1 1 C-LD nosis to assess aortic valve anatomy, aortic
valve function, and thoracic aortic diam-
eters.1-4
Synopsis 2. In patients with a dilated thoracic aorta, a CT
or MRI at the time of diagnosis is reasonable
Atherosclerotic AAA are associated with a >20% risk of 2a C-LD
to assess thoracic aortic anatomy and diam-
cardiovascular events within 10 years, as they are con- eters.1,3,5-7
sidered a coronary artery disease equivalent according 3. In patients with a dilated thoracic aorta,
to the NCEP ATP III.2 To reduce risk of cardiovascular follow-up imaging (with TTE, CT, or MRI, as
appropriate based on individual anatomy) in
events and mortality, aspirin at 75 mg to 162 mg daily
6 to 12 months is reasonable to determine

for secondary prevention has been incorporated into the 2a C-LD
the rate of aortic enlargement; if stable,
2006 updated “AHA/ACC Guidelines for Secondary surveillance imaging every 6 to 24 months
(depending on aortic diameter) is reason-
Prevention for Patients With Coronary and Other Athero- able.1,3,4
sclerotic Vascular Disease.”3 Most AAA contain an intra-
luminal thrombus (see Section 6.2, “AAA: Cause, Risk
Factors, and Screening”) made up of a complex matrix Synopsis
of platelets, inflammatory cells, and fibrin, which contrib-
In patients with TAD, a detailed baseline assessment of
utes to growth and progression, and thus antithrombotic
all the segments of thoracic aorta, aortic valve anatomy,
therapy has been hypothesized to have a potential ben-
and aortic valve function is important. TTE, CT, and MRI
efit in AAA. However, clinical outcomes data are limited,
are all commonly used for assessment of the thoracic
and further study of the efficacy of antiplatelet therapy in
aorta.
AAA is warranted.

Recommendation-Specific Supportive Text 1. In patients with TAD not at surgical threshold, a
1. Low-dose aspirin monotherapy in patients with detailed assessment with a TTE to evaluate aortic
noncoronary atherosclerosis is considered a treat- valve anatomy and aortic valve function is impor-
ment to mitigate risk of cardiovascular events, tant for establishing a baseline. TTE usually pro-
including stroke, death caused by coronary artery vides clear images of the aortic root and ascending
disease, and myocardial infarction.3 Data are lim- aorta, is safe and reproducible, and can be used for
ited on aortic-specific clinical outcomes in AAA. longitudinal surveillance. In select patients with dif-
Use of low-dose aspirin has been hypothesized ficult echocardiographic imaging windows, a TEE
to reduce growth and progression of AAA attrib- is an alternative for evaluating aortic valve anatomy
utable to the detrimental effects of platelet acti- and aortic dimensions.1-3
vation within the intraluminal thrombus. In 1 small 2. Cross-sectional imaging with CT or MRI has been
cohort study, low-dose aspirin was associated with established as the gold standard for assessment
a reduced AAA growth rate and need for aneurysm of all segments of thoracic aorta including arch

branch vessels.5,6 Electrocardiographic-gated Synopsis
CLINICAL STATEMENTS
techniques minimize motion artifact and thus allow
In patients with AAA, imaging assessment of the abdom-
AND GUIDELINES
precise measurement of aortic root and ascending
inal aorta is important for establishing baseline diameter
aortic dimensions.5,6
and determining the timing of surveillance imaging. Ultra-
3. Patients with stable aortic dimensions can be
sound imaging has been the standard for surveillance
observed longitudinally with TTE, CT, or MRI. The
imaging of the abdominal aorta and is widely used. CT
frequency of surveillance imaging should be indi-
provides superior visualization of the abdominal aorta
vidualized and informed by the aneurysm cause,
and its branches and is therefore used for preopera-
aortic diameter, historical rate of aortic growth, how
tive planning. MRI is a reasonable alternative to CT in
close the diameter is to the surgical threshold, and
selected patients. Figure 20 shows a proposed general
the patient’s age.8,9 In general, in patients with non-
algorithm for surveillance imaging of AAA, recognizing
genetic and syndromic causes, the rate of aortic
that surveillance intervals should be individualized.
growth is relatively slow, so the interval for surveil-
lance imaging may be increased.
6.4.3.2. Surveillance of Abdominal Aortic Dilation and
Aneurysm 1. Multiple studies have established that ultrasound
surveillance of AAAs helps to prevent rupture and
Recommendations for Surveillance of Abdominal Aortic Dilation and
Aneurysm
mortality.2-7,11 The risk of rupture increases at an
Referenced studies that support the recommendations are AAA diameter of >5.5 cm for men and >5.0 cm for
summarized in the Online Data Supplement. women; accordingly, surveillance imaging should
COR LOE Recommendations be more frequent at larger AAA diameters that
1. In patients with an AAA of 3.0 cm to 3.9 cm, approach these thresholds. Conversely, at AAA
1 B-NR
surveillance ultrasound is r ecommended diameters of 3.0 cm to 3.9 cm, longer surveillance
every 3 years to assess for interval
imaging intervals have been shown to be safe.
change.1-8
2. In patients with AAA of 4.0 cm to 4.9 cm, rates
2. In men with an AAA of 4.0 cm to 4.9 cm
and in women with an AAA of 4.0 cm to 4.4
of aortic growth are higher, so annual surveillance
1 B-NR
cm, surveillance ultrasound is recommended ultrasound is recommended. Even shorter intervals
annually to assess for interval change.1-8 are often used in those who smoke, have diabetes,
3. In men with an AAA of ≥5.0 cm and women or both because of their increased risk of growth.
with an AAA of ≥4.5 cm, surveillance ultra- 3. Once the size of the AAA reaches ≥5.0 cm in men
1 B-NR
sound is recommended every 6 months to
assess for interval change.1-8 and ≥4.5 cm in women, the screening interval is
4. In patients with an AAA that is inadequately
shortened to every 6 months given the potential of
1 C-EO defined with ultrasound, surveillance CT is larger aneurysms to grow more rapidly and reach
recommended. the thresholds for intervention. CT provides superior
In such patients, when there is a contraindica- visualization of the abdominal aorta and its branches
2a C-LD tion to CT or to lower cumulative radiation risk, and is an excellent alternative when ultrasound is
surveillance MRI is reasonable.9,10
inadequate. MRA is a reasonable alternative to CT.
5. In patients with an AAA that meets criteria for
1 C-EO repair, CT is recommended for preoperative
Non-IV contrast MRI techniques have also been
planning. shown to be useful in defining AAAs.9,10
Figure 20. The

Frequency of
Surveillance Imaging
of Abdominal Aortic
Aneurysms Based on
Current Aortic Diameter.
Color corresponds to Class
of Recommendations in
Table 2.

4. CT is generally preferred when an AAA reaches Recommendations for Surgery for Sporadic Aneurysms of the Aortic
CLINICAL STATEMENTS
the threshold for intervention, both to confirm aor- Root and Ascending Aorta (Continued)
AND GUIDELINES
tic diameters and to detail the anatomy of the aorta COR LOE Recommendations
and its branches for preoperative planning. 5. In patients undergoing repair or replace-
ment of a tricuspid aortic valve who have
a concomitant aneurysm of the ascending
6.5. Surgical and Endovascular Management of 2a B-NR aorta with a maximum diameter of ≥4.5 cm,
ascending aortic replacement is reasonable
Aortic Aneurysms when performed by experienced surgeons in
a Multidisciplinary Aortic Team.18-21
Most patients with TAA and AAA are asymptomatic, so
In patients undergoing repair or replacement
the purpose of surgical or endovascular intervention is of a tricuspid aortic valve who have a concom-
to reduce the risk of adverse aortic events (ie, aortic dis- 2a B-NR itant aneurysm of the ascending aorta with
section, rupture, and aortic-related death). Consequently, a maximum diameter of ≥5.0 cm, ascending
aortic replacement is reasonable.18-21
determining the optimal timing of intervention requires a
careful anatomic assessment, followed by weighing the In patients undergoing cardiac surgery for
indications other than aortic valve repair
risk of future adverse aortic events against the risk of or replacement who have a concomitant
2b C-LD
intervention.The goal of open surgery is to replace the aneurysm of ascending aorta with a maxi-
aneurysmal aortic segment with a prosthetic graft anas- mum diameter of ≥5.0 cm, ascending aortic
replacement may be reasonable.18
tomosed to nonaneurysmal aortic tissues while maintain-
6. In patients with a height >1 standard devia-
ing critical aortic branch vessels. Endovascular repair tion above or below the mean who have an
leverages contiguous nonaneurysmal aortic or iliac seg- asymptomatic aneurysm of the aortic root
ments for fixation of endovascular stent grafts to exclude 2a C-LD
or ascending aorta and a maximal cross-
sectional aortic area/height ratio of ≥10
blood flow from the aneurysmal sac. To date, the FDA cm2/m, surgery is reasonable when performed
has approved individual stent grafts for the treatment of by experienced surgeons in a Multidisciplinary
aneurysms involving the descending thoracic, juxtarenal, Aortic Team.14,15,22
and infrarenal aortic segments. Stent graft devices to 7. In asymptomatic patients with aneurysms of
address the ascending aorta, aortic arch, and thoracoab- the aortic root or ascending aorta who have
either an ASI of ≥3.08 cm/m2 or AHI of ≥3.21
dominal aorta are available under investigational use in 2b C-LD
cm/m, surgery may be reasonable when per-
the United States, currently in physician- and industry- formed by experienced surgeons in a Multidis-
sponsored clinical trials. Long-term studies have shown ciplinary Aortic Team.23
that use of endovascular stent grafts outside of the ana-

tomic criteria tested in their pivotal trials is associated Synopsis
with increased risk of aneurysm sac enlargement, under-
scoring the need for appropriate patient selection and for Elective surgery for aneurysms of the aortic root and
long-term surveillance after endovascular repair.1 ascending aorta is ideally performed when the risk of
adverse events—dissection, rupture, or sudden death—
outweighs the risks of surgery. No prospective multi-
6.5.1. Surgery for Sporadic Aneurysms of the Aortic
center observational studies have evaluated the myriad
Root and Ascending Aorta
of parameters (eg, aortic diameter, length, or area, alone
Recommendations for Surgery for Sporadic Aneurysms of the Aortic
Root and Ascending Aorta
or indexed to height or BSA, wall stress, shear stress)
Referenced studies that support the recommendations are proposed for predicting the risk of aortic adverse events.
summarized in the Online Data Supplement. From a purely mechanical perspective, aortic dissection
COR LOE Recommendations or rupture can be considered a failure event, where an
1. In patients with aneurysms of the aortic root imbalance exists between stresses on the aneurysm wall
1 C-LD and ascending aorta who have symptoms attrib- and the inherent strength of its tissue.24 Whether the aor-
utable to the aneurysm, surgery is indicated.1,2
tic dissection is precipitated by increased wall stress or
2. In asymptomatic patients with aneurysms of the decreased wall strength, or a combination of both, is an
1 B-NR aortic root or ascending aorta who have a maxi-
mum diameter of ≥5.5 cm, surgery is indicated.3-9
area of active research.25-29 Maximal aortic diameter has
logically been the primary criterion for elective aneurysm
3. In patients with an aneurysm of the aortic root
or ascending aorta of <5.5 cm, whose growth repair because, per LaPlace’s law, wall stress increases
1 C-LD rate confirmed by tomographic imaging is proportionally with aortic radius and inversely to thick-
≥0.3 cm/y in 2 consecutive years, or ≥0.5 cm ness.30 The original natural history studies examined
in 1 year, surgery is indicated.10-13
the risk of rupture or aortic dissection versus diameter
4. In asymptomatic patients with aneurysms of
the aortic root or ascending aorta who have
and the hinge point for dissection generated the 5.5-
2a B-NR a maximum diameter of ≥5.0 cm, surgery is cm threshold that has long governed clinical practice.7,8
reasonable when performed by experienced Although a significant proportion of patients with type
surgeons in a Multidisciplinary Aortic Team.14-17
A aortic dissection present with diameters <5.5 cm,31,32

this surgical threshold still effectively reduces adverse relative to a control aortic diameter of ≤3.4 cm, a
CLINICAL STATEMENTS
events.17,33 diameter of 4.0 cm to 4.4 cm conferred an 89-fold
AND GUIDELINES
increased risk of aortic dissection, and a diameter
of ≥4.5 cm conferred a 6300-fold increased risk
Recommendation-Specific Supportive Text (Figure 5). Consequently, many experienced sur-
1. Large aneurysms can compress nearby structures geons in a Multidisciplinary Aortic Team choose to
as they expand, resulting in symptoms of chest or operate selectively on patients with aneurysms of
back pain. Alternatively, pain is sometimes associ- 5.0 cm to 5.4 cm,17 provided the patient’s surgical
ated with rapid aortic growth. Consequently, the risk is low,38 and they have had excellent results14-16
appearance of such symptoms raises concern for in doing so. However, there is an ongoing prospec-
an increased risk of aneurysm rupture,1,2 and surgi- tive multicenter RCT of patients with ascending
cal repair is therefore indicated. TAAs of 5.0 cm to 5.4 cm that will compare out-
2. A maximum aortic diameter of ≥5.5 cm has been comes of early elective surgery vs. medical surveil-
the primary criterion for elective surgical repair of lance,39 the results of which could provide further
aneurysms of the aortic root or ascending thoracic guidance.
aorta,4,6 based on natural history studies that exam- 5. For patients undergoing aortic valve surgery with
ined diameter (without centerline analysis) at the concomitant ascending aortic aneurysm of ≥4.5
time of adverse event and an assumed operative cm, guidelines have previously recommended
mortality of <5%.4,7-9 The mortality rate for elective simultaneous aortic replacement in those with BAV.
surgery is low, whereas the risk of adverse events On the other hand, in patients who have undergone
is high when such surgery is recommended but not valve surgery without concomitant aortic aneurysm
performed because of patient noncompliance or surgery, whether for an underlying bicuspid or tri-
comorbidities.33 The same 5.5-cm diameter thresh- cuspid aortic valve, the associated aneurysms have
old applies regardless of whether patients have tri- been shown to grow slowly and have low rates
cuspid or BAVs.5 of aortic complications over time. Still, data have
3. One meta-analysis and limited observational stud- also shown the safety of performing concomitant
ies have found ascending aortic aneurysm growth aneurysm repair at a diameter of ≥4.5 cm by expe-
to be slower than previously reported, and fre- rienced surgeons working in a Multidisciplinary
quently <0.5 mm/y, in patients with a tricuspid Aortic Team.16,18-21,40-43 Nevertheless, until there are
aortic valve and without a genetic aortic disor- better predictors for aortic complications, in general
der.11,12,17,34 The meta-analysis suggested that rapid it is reasonable in patients undergoing aortic valve
aneurysm growth is associated with an increased repair or replacement to offer concomitant aneu-
risk of rupture.12 Because of the inherent error in rysm surgery for those with aneurysms of ≥5.0 cm,
measurement as well as interobserver variability, because of the faster rate of growth and higher
1 mm to 2 mm growth per year would be difficult risk of aortic dissection. Aortic root replacement
to document consistently on surveillance imaging. should be individualized based on the type of aortic
Discrepancies in measurement can occur when valve surgery (ie, valve repair with or without valve-
comparing different imaging modalities or even sparing root versus valve replacement, mechani-
when using the same modality when comparing cal versus bioprosthetic root replacement), patient
images obtained with and without contrast. Ideally, condition, patient age, and comorbidities. In those
growth rates are most accurate when assessed undergoing cardiac surgery for indications other
using cardiac-gated CT or MRI with centerline than aortic valve repair, concomitant prophylactic
measurement techniques.35 Confirmed growth aortic replacement at a diameter of 5.0 cm may be
of ≥0.5 cm in 1 year has been, and remains, an reasonable, because it would provide a margin of
indication for surgery.3-6 Moreover, growth of even safety against future aortic dissection, particularly
0.3 cm in 1 year still substantially exceeds the because cardiac surgery itself becomes an addi-
expected growth rate for aneurysms of the root tional risk factor for subsequent aortic dissection.
and ascending aorta, so if that rate of growth rate 6. Data from the IRAD showed that ∼60% of patients
is sustained for 2 consecutive years, intervention is with acute type A aortic dissection had maximal
also recommended.13 aortic diameters of <5.5 cm32 at presentation, a
4. The risk of aortic dissection or rupture correlates finding that has been corroborated by others.31,44
with increasing aneurysm diameter,7,8,16 as does the Conversely, most patients with aneurysms <5.5
rate of aortic growth.12,36 As such, aneurysms of cm who are managed medically do not suffer aor-
≥5.0 cm would be expected to have a greater risk tic dissection or rupture. Therefore, absolute aor-
of complications or rapid growth than would smaller tic diameter is far from an ideal predictor of risk.
aneurysms. Indeed, in a report by Paruchuri et al,37 Parameters proposed to improve risk prediction

include the ratio of aortic diameter to either patient Synopsis

CLINICAL STATEMENTS
height or BSA,23 the ratio of aortic area to height,14,15

The goal of prophylactic repair of aneurysms of the aortic
AND GUIDELINES
the ascending aortic length (centerline, from annu-

root and ascending aorta is to prevent life-threatening
lus to innominate artery takeoff),14,15,45-47 aortic
complications from acute aortic events such as aortic dis-
stiffness, and peak aortic wall stress.25,48-50 All are
section, aortic rupture, or sudden death. This goal is best
retrospectively promising, but none has been pro-
achieved when the risk of future adverse aortic events is
spectively validated. A cross-sectional aortic area
greater than the expected surgical mortality (considering
to patient height ratio of ≥10 cm2/m was found to
both the surgeon’s and institutional experience). The STS
correlate with increased mortality among unoper-
database has clearly shown that proximal thoracic aortic
ated patients with root or ascending aortic aneu-
surgery has a lower operative mortality when performed
rysms and either a tricuspid15 or BAV.14 The use
electively rather than emergently (2.2% versus 17.2%,
of the cross-sectional aortic area to height ratio is
respectively).1
most appropriate in patients whose height is >1
standard deviation above or below the mean.
7. A single-center large database of TAA has grown Recommendation-Specific Supportive Text
considerably and was reevaluated with indexing
of aortic diameter to BSA (ASI) or height (AHI), to 1. Proximal thoracic aortic operations in the United
improve the prediction of adverse aortic events.23 States, including ascending thoracic aortic replace-
Height was preferred because the variable nature ment and aortic root replacement, have an over-
of weight and the underlying genetic contribu- all elective mortality rate of 2.2%. Consequently,
tion to height. Recommendations for prophylactic patients who meet criteria for aneurysm repair and
repair at aortic diameters of <5.5 cm have been have low operative risk can undergo prophylactic
proposed but not systematically tested in large- resection and graft placement with low operative
scale multicenter trials. Experienced surgeons in mortality risk.1 Similar results were obtained when
a Multidisciplinary Aortic Team38 may consider the examining the Nationwide Inpatient Sample in
use of such ratios when determining the optimal which operative mortality rate for proximal thoracic
timing of intervention. This may be particular useful aortic surgery was 2.5%.2
for female patients, but more studies are required to 2. Single-institution studies have shown that the
further evaluate surgical thresholds in women with addition of ascending TAA repair to AVR does not
aneurysms of the aortic root or ascending aorta. increase operative mortality in experienced aor-
tic centers.22-24 However, such results may not be
6.5.1.1. Surgical Approach for Patients With Sporadic reproducible at low-volume centers that have a
Aneurysms of the Aortic Root and Ascending Aorta higher operative mortality rate for isolated proximal
Meeting Criteria for Surgery thoracic aortic surgery.1,2 Root-sparing AVR with
Recommendations for Surgical Approach for Patients With Sporadic concomitant ascending aneurysm repair is accept-
Aneurysms of the Aortic Root and Ascending Aorta Meeting Criteria for
able, because data suggest the aortic root dilates
Surgery
Referenced studies that support the recommendations are at a slower rate than does the ascending aorta,
summarized in the Online Data Supplement. and studies of root-sparing surgery have shown no
COR LOE Recommendations increase in long-term adverse aortic events.3-6,25
1. In patients with an aneurysm isolated to the 3. Surgical approaches to replace the aortic root
1 B-NR
ascending aorta who meet criteria for surgery, should be guided by the aortic valve anatomy. If the
aneurysm resection and replacement with an aortic valve is unsuitable for sparing or repair (eg,
interposition graft should be performed.1,2
large fenestrations, calcification), a mechanical- or
2. In patients undergoing aortic valve repair or
replacement with a concomitant ascending
biological-valved conduit aortic root replacement
1 B-NR aortic aneurysm, a separate aortic valve inter- should be performed because, when elective,
vention and ascending aortic graft is recom- this procedure has an operative mortality rate of
mended.3-6
2.2% in the United States based on the STS data-
3. In patients undergoing aortic root replace- base.2,26 Single-institution series from centers with
ment with an aortic valve that is unsuitable for
1 B-NR sparing or repair, a mechanical or biological Multidisciplinary Aortic Teams have also shown
valved conduit aortic root replacement is indi- excellent results both with and without concomi-
cated.1,2,7,8 tant hemiarch replacement.7 Long-term outcomes
4. In patients undergoing aortic root replace- are similar with mechanical- versus biological-
ment, valve-sparing aortic root replacement
is reasonable if the aortic valve is suitable
valved conduit aortic root replacements, even in
2a B-NR patients <70 years old.8
for sparing or repair and when performed by
experienced surgeons in a Multidisciplinary 4. In younger patients with an aortic valve that is ame-
Aortic Team.9-21
nable to sparing or repair, elective valve-sparing

aortic root replacement has been performed safely and specialized branched grafts to aid in reconstruction.
CLINICAL STATEMENTS
by experienced surgeons in a Multidisciplinary Endovascular techniques also continue to evolve.
AND GUIDELINES
Aortic Team.9-11,21 In patients with aortic root aneu-
rysms without an underlying genetic disorder,
valve-sparing aortic root replacement has been Recommendation-Specific Supportive Text
performed by either the reimplantation or remodel- 1. Because of the juxtaposition of the aortic arch to
ing technique with comparable survival and valve other vascular structures, nerves, trachea, and the
durability.12 esophagus, symptoms may develop because of the
mass effect from encroachment on adjoining struc-
6.5.2. Aortic Arch Aneurysms tures. Ortner’s syndrome is unilateral hoarseness
Recommendations for Aortic Arch Aneurysms secondary to inflammation or stretching of the left
recurrent laryngeal nerve.8 Dysphagia aortica can
be caused by extrinsic compression of the esopha-
gus by either fusiform or saccular aneurysms of
1. In patients with an aortic arch aneurysm
who have symptoms attributable to the
the arch. Likewise, extrinsic compression of the
1 C-EO aneurysm and are at low or intermediate trachea may result in dyspnea, and compression
operative risk, open surgical replacement is of the innominate vein or superior vena cava may
recommended.
cause superior vena cava syndrome. Nonspecific
2. In patients with an isolated aortic arch symptoms include chest pain or pressure, fatigue,
aneurysm who are asymptomatic and have
2a B-NR a low operative risk, open surgical replace- and neck, jaw, or back pain.
ment at an arch diameter of ≥5.5 cm is 2. Open replacement of the aortic arch requires the
reasonable.1-3 use of cardiopulmonary bypass, hypothermia, and
3. In patients undergoing open surgical repair of other adjuncts for neurologic and systemic pro-
an ascending aortic aneurysm, if the aneurys-
tection. Various randomized and nonrandomized
2a C-LD mal disease extends into the proximal aortic
arch, it is reasonable to extend the repair with trials have compared different cannulation strate-
a hemiarch replacement.4,5 gies (ie, axillary, femoral, innominate),9-12 levels of
4. In patients undergoing open surgical repair hypothermia, and variations in cerebral perfusion
of an aortic arch aneurysm, if the aneurysmal (antegrade, select antegrade, retrograde),13-15 with
2b C-LD disease extends into the proximal descending
no one technique dominating or being shown con-
thoracic aorta, an elephant trunk procedure

may be considered.6,7 clusively to be superior to another.
5. In patients with an aortic arch aneurysm who 3. When proximal aneurysmal aortic disease extends
are asymptomatic but meet criteria for inter- to the level of the innominate artery or further into
2b C-EO vention, but have a high risk from open surgi- the arch, but not necessarily the whole arch, a hemi-
cal repair, a hybrid or endovascular approach
may be reasonable. arch procedure may be able to effectively address
the distal pathology. Open distal anastomosis will
require the same adjuncts and approaches used in
Synopsis open arch replacement (as described previously),
Aortic arch aneurysms are the least common of the TAA, including neuroprotective strategies. Modified
because <10% of aneurysms involve the arch only; in approaches have been described that eliminate
most cases, arch aneurysms are associated with adja- the need for open repair whereby the ascending
cent pathology.1 Previous aortic dissection is the most aorta is replaced first with a trifurcated side-branch
common cause of arch aneurysms; in a large meta- for debranching of the arch in a sequential manner
analysis, only 28.3% of patients undergoing intervention to a level that is accessible for clamping; however,
on the arch had de novo aneurysmal disease, with the no studies have yet shown the benefit of such an
remainder resulting from acute or chronic aortic dissec- approach. Although it does add to the cardiopulmo-
tion. The risk of dissection or rupture, as related to aor- nary bypass time and blood loss,4,5 the addition of a
tic diameter, is presumed to be similar in the arch as in hemiarch has been shown not to increase the risk
other thoracic locations, although no large reports con- of the procedure. However, this noninferiority is lost
sider arch dimensions alone. Additionally, because of the when the proximal arch is disease free, with excep-
proximity of the aortic arch to other thoracic structures, tion of an underlying aortopathy in which the nor-
dilation may result in symptoms before the diameter mal-sized arch will predictively enlarge or dissect at
reaches a threshold typically considered for intervention. a later time; in this setting, a hemiarch is justified.16,17
Intervention to treat arch aneurysms carries an increased 4. The elephant trunk procedure, as originally
risk given the need to manage the great vessels and described, extends the aortic arch graft into the
protect the brain. Various techniques have been devel- proximal descending aorta, thereby facilitating the
oped, including the use of hypothermic circulatory arrest subsequent repair of diseased descending thoracic

aorta (by either open repair or TEVAR).18 Either a with endovascular experience who have access to
CLINICAL STATEMENTS
traditional elephant trunk (an extension graft anas- the appropriate devices, investigational devices, or
AND GUIDELINES
tomosed to the distal end of an aortic arch graft at both.

the time of arch repair that projects into the proximal
6.5.3. Descending TAA
descending aorta with a free distal end) or a frozen
elephant trunk (a combined open aortic arch graft 6.5.3.1. Size Thresholds for Repair of Descending TAA
with an extension endovascular stent-graft extend- Recommendations for Size Thresholds for Repair of Descending TAA
ing into the descending thoracic aorta to treat Referenced studies that support the recommendations are
extensive TAD involving both arch and descending summarized in the Online Data Supplement.
segments via a median sternotomy) can be used.6,19 COR LOE Recommendations

With adjunctive procedures (ie, debranching), the 1. In patients with intact descending TAA,
distal anastomosis can be moved more proximally 1 B-NR repair is recommended when the diameter is
≥5.5 cm.1,2
into aortic zones 2 or 3 (Figure 3), while still pro-
ceeding with an elephant trunk and with the poten- 2. In patients with intact descending TAA
and risk factors for rupture (Table 17),
tial of decreasing morbidity, but data are limited 2b B-NR
repair may be considered at a diameter of
on the benefits of moving the anastomotic site. A <5.5 cm.2-6
qualifying factor for considering open versus frozen 3. In patients at increased risk for perioperative
elephant trunk is whether the primary distal seal will 2b B-NR
morbidity and mortality (Table 18), it may be
reasonable to increase the size threshold for
be achieved with the frozen elephant trunk. In the surgery accordingly.7
absence of a distal seal, the conventional approach
would provide the same considerations for the sec-
ond-stage procedure. Synopsis
5. Various hybrid and endovascular techniques have The current aortic size threshold for repair of descend-
been developed to address the aneurysmal arch in ing TAA is primarily based on single-center series where
the setting of a high-risk patient, including open patients have been observed with surveillance imag-
extra-anatomical bypasses (eg, left carotid-to- ing and clinical follow-up to determine the incidence of
left subclavian artery bypass) and endovascular aortic-related events and deaths. Such series indicate
approaches. The Next-gen Fenestrated TEVAR that a descending aortic diameter of >6 cm is associ-
trial showed the feasibility of proximal landing ated with an increased risk of adverse aortic events
zone coverage, with most endografts being placed and mortality,1,2 as shown in Table 16. Moreover, certain
in zones 0 or 1 (Figure 3), although a landing zone patient and aneurysm features are associated with an
of <15 mm was associated with an increased risk increased risk for aortic dissection or rupture, as shown
of a type I endoleak (Figure 11).20 The midterm in Table 17, which may prompt consideration of earlier
follow-up showed 5-year survival of 71% with an surgery. Conversely, some patients are at increased risk
aneurysm-related event-free survival of 77%. The of perioperative morbidity or mortality, in which case
most frequent reason for reoperation was type Ia the size threshold for aortic repair might be increased.
endoleak (5%).21 Nonrandomized comparisons of Specifically, if the patient does not have ideal anatomy
open versus hybrid endovascular approaches have for endovascular repair, or has otherwise increased risk
not shown significant differences in outcomes.22-24 for contemplated open repair, close monitoring until
Complete endovascular approaches have been a higher surgical threshold is reached would be justi-
described25,26 and may be considered by those fied. Advanced age,8 preoperative renal insufficiency or
Table 16. Adverse Aortic Events at 1 Year, Based on Base- Table 17. Risk Factors for Aortic Rupture Among Patients
line Aortic Diameter, Among Patients With Descending TAA With Descending TAA
Aortic Diameter Definite Aortic Probable Aortic High-Risk Features for Rupture
(cm) Event* (%) Event† (%)
Aneurysm growth of 0.5 cm/y3
5.0 5.5 8.0
Symptomatic aneurysm4
5.5 7.2 11.2
Marfan, Loeys-Dietz, or vascular Ehlers-Danlos syndrome, or HTAD (see
6.0 9.3 15.6 Section 6.1.2, “Genetic Aortopathies”)2
7.0 15.4 28.1 Saccular aneurysm5
*Definite aortic event includes aortic dissection or rupture confirmed with Female sex2
imaging or intraoperative findings. Infectious aneurysm6
†Probable aortic event includes definite aortic events as well as sudden
unexplained death. HTAD indicates heritable thoracic aortic disease; and TAA, thoracic aortic
TAA indicates thoracic aortic aneurysm. Based on data from Kim JB, et al.1 aneurysm.

hemodialysis, chronic obstructive pulmonary disease, predictive of poor outcomes after TEVAR.7 When
CLINICAL STATEMENTS
and previous stroke are harbingers of adverse outcomes contemplating either approach, special attention
AND GUIDELINES
or perioperative mortality after open repair (Table 18).9 to these risk factors will allow appropriate consid-
Markers of frailty, pulmonary disease, thoracoabdominal eration of the risks to benefits in deciding on the
extent, need for iliac access, and zone 1/2 deployment merits of intervention.
were associated with major adverse events after TEVAR
(Table 18).7 A nuanced approach and detailed discus- 6.5.3.2. Endovascular Versus Open Repair of
sion with the patient can help guide the most reason- Descending TAA
able treatment plan, weighing the risks of the operation
Recommendations for Endovascular Versus Open Repair of
against the risks of continued surveillance. Descending TAA
Recommendation-Specific Supportive Text COR LOE Recommendations
1. There is an increased incidence of aortic-related 1. In patients without Marfan syndrome, Loeys-
events such as rupture or dissection with aortic Dietz syndrome, or vascular Ehlers-Danlos
syndrome, who have a descending TAA that
diameters >6 cm, justifying intervention when the 1 B-NR
meets criteria for intervention and anatomy
diameter is ≥5.5 cm in size.1,2 suitable for endovascular repair, TEVAR is
2. High-risk features of rupture have been previously recommended over open surgery.1-4
identified, supporting repair at a smaller diameter 2. In patients with a descending TAA that meets
threshold when these criteria are met. Features criteria for repair with TEVAR, who have
1 B-NR smaller or diseased access vessels, consid-
including rapid aortic growth (≥0.5 cm/y),3 symp- erations for alternative vascular access are
tomatic aneurysms,4 underlying connective tissue recommended.5
disorder or HTAD,2 saccular aneurysm morphol- 3. In patients with a descending TAA that meets
ogy,5 female sex,2 and infected aneurysm6 have all criteria for intervention, who have anatomy
unsuitable for endovascular repair, and who
been associated with a higher tendency for rupture. 2a B-NR
are without significant comorbidities and have
3. In patients being considered for open or endovas- a life expectancy of at least 10 years, open
cular repair, high-risk clinical features (Table 18) surgical repair is reasonable.6-9
have been identified that portend poor outcomes
after repair. For open surgical repair, advanced
age,8 preoperative renal insufficiency of stage 3 Synopsis

or greater, chronic obstructive pulmonary disease Although no RCTs comparing TEVAR with open repair
and forced expiratory volume in 1 second ≤50% of descending TAA exist, the pivotal device trials1,3,10
predicted, and previous stroke9 have all been have shown a reduced perioperative morbidity, increased
associated with increased risk of death, periop- clinical utility, and reduced follow-up aneurysm-related
erative morbidity, or both. For endovascular repair mortality compared with open surgical repair. However,
of descending TAA, frailty indicators, pulmonary reintervention after TEVAR is substantial.11 In addition,
disease, as well as procedural complexity are although clinical device trials showed improved periop-
erative and long-term outcomes with TEVAR, Medicare
Table 18. Patient Characteristics Associated With Increased claims data show that the perioperative advantage was
Perioperative Morbidity and Mortality After Open and Endo- lost within the first year after intact aneurysm repair,
vascular Repair of Descending TAA
with a 5-year survival that was significantly worse after
Open Surgical Repair Endovascular Repair TEVAR versus open repair, at 79% versus 89%, respec-
Advanced age 8
Functional dependence tively (P<0.0001).4 Further study should be dedicated to
65–74 y (OR, 1.8; 95% CI, 1.4–2.4; understanding why the benefit from endovascular repair
P <0.001) decays over time. In patients with connective tissue dis-
75 y (OR, 2.6; 95% CI, 2.0–3.5; orders or HTAD, or those with a longer life expectancy,
P <0.001) open surgical repair is reasonable. Open surgical repair
Preoperative renal insufficiency (stage 3 Thoracoabdominal aortic an- of descending TAA reflects a volume-outcomes relation-
or greater CKD) or hemodialysis eurysm extent ship: Although large institutional series have shown good
COPD and FEV1 50% predicted Pulmonary disease outcomes with open repair,6-9 these results are not repli-
Previous stroke 9
Need for iliac access cable at lower volume centers.12 The decision to proceed
Zone 1/2 landing for thoracic with endovascular versus open repair balances the need
stent graft7 for appropriate anatomy and access, as well as a higher
CKD indicates chronic kidney disease; COPD, chronic obstructive pulmo- reintervention rate for TEVAR versus the higher periop-
nary disease; FEV1, forced expiratory volume in 1 second; and TAA, thoracic erative risk associated with more definitive open surgical
aortic aneurysm. repair.

Recommendation-Specific Supportive Text access vessel thrombosis irrespective of the clini-

CLINICAL STATEMENTS
cal setting, type of aortic disease, and device sizing.

1. TEVAR is associated with a reduced periopera-
AND GUIDELINES
3. Open descending thoracic aortic repair can be per-

tive morbidity, reduced hospital length of stay, and
formed with low morbidity and mortality rates in
better freedom from aneurysm-related mortality
high-volume centers.6-8,14 In a multicenter retrospec-
compared to open surgical repair, based on clini-
tive study using the MEDPAR (Medicare Provider
cal device trial data.1,3,10 In the study by Makaroun
Analysis and Review) data,15 the overall mortality
et al in 2008,13 140 patients with fusiform aneu-
rate after open surgical repair of descending TAA
rysms were treated with TEVAR and compared
decreased in high-volume versus low-volume cen-
with 94 open surgical controls. At 5 years, there
ters (11% versus 15%; P<0.01). In addition, data
was a decreased aneurysm-related mortality
using Medicare claims show that the benefit of
(2.8% versus 11.7%, respectively, P=0.008), a
TEVAR is no longer present 1 year after endovas-
reduced major adverse event rate (57.9% ver-
cular therapy, with a significantly worse 5-year sur-
sus 78.7%, respectively, P=0.01), and decreased
vival compared with open repair (79% versus 89%;
major aneurysm-related reintervention (3.6% ver-
P<0.0001).4 In a recent retrospective, single-center
sus 2.1%, respectively) in TEVAR versus open
study in which propensity score matching analysis
repair. In the study by Matsumura et al,10 survival
was used to compare the outcomes of open and
was noninferior for TEVAR (98.1%) versus open
endovascular descending and TAAA repair in 278
surgery (94.3%) at 30 days, but the severe mor-
pairs of patients,16 open repair resulted in better
bidity composite index, a marker for postoperative
10-year survival than endovascular repair (52% ver-
complications, was lower for TEVAR (0.2±0.7 ver-
sus 33%; P<0.0001). Because of the lack of avail-
sus 0.7±1.2, respectively; P<0.01). In the study
able long-term data on aortic-specific mortality rate
by Fairman et al,11 195 TEVAR patients were
in young patients after TEVAR, in patients deemed
compared with 189 open surgical controls, and
to have a life-expectancy of ≥10 years, open surgi-
the 30-day mortality rate was lower (2% versus
cal repair is reasonable.
8%, respectively; P<0.01) and the major adverse
event rate was lower (41% versus 84%, respec-
6.5.3.3. Left Subclavian Artery Management
tively; P<0.01) for TEVAR; at 1 year, aneurysm-
Recommendations for Left Subclavian Artery Management
related mortality rate was lower for TEVAR than

for open repair (3.1% versus 11.6%, respectively; summarized in the Online Data Supplement.
P<0.002). However, in a registry study using COR LOE Recommendations
Medicare claims data,4 although short-term out-
1. In patients with descending TAA who undergo
comes were similarly better with TEVAR com- TEVAR with planned left subclavian artery
pared with open repair, that survival advantage coverage, revascularization of the left subcla-
was no longer present at 1 year and, at 5 years, 1 B-NR vian artery before TEVAR is recommended to
prevent spinal cord injury (SCI)1,2 and poten-
survival was significantly worse for TEVAR ver- tially to reduce stroke risk2 and prevent other
sus open repair at 79% versus 89%, respectively ischemic complications.
(P<0.0001). Overall, the data show that TEVAR 2. In patients with descending TAA who have
is beneficial in the short- to intermediate-term in undergone TEVAR with left subclavian cover-
age and develop SCI that is unresponsive
patients with appropriate anatomy for endovascu- 2b C-LD
to an increase in BP or a cerebrospinal fluid
lar repair, but the advantage is not sustained over drain, left subclavian artery revascularization
time. may be considered.3
2. Because of the relatively large delivery systems
for thoracic endografting, iliac artery graft con-
duits may be required to ensure safe delivery of Synopsis
the endograft into the aorta. In the clinical device Left subclavian artery coverage is required in up to 40%
trials, access of vessels other than the femoral of cases of TEVAR of descending TAAs.4 SCI and stroke
artery was required in 9.4% to 21.1% of patients remain devastating complications associated with TEVAR.
because of small or diseased access vessels.1-3 In Addressing these modifiable risk factors would allow
a multicenter cohort study from the GREAT (Global for better outcomes after this less invasive treatment
Registry for Endovascular Aortic Treatment) regis- strategy. In addition, special considerations include the
try,5 the overall access complication rate was 2.8%, prevention of vertebrobasilar insufficiency (particularly
and women had a higher rate of access complica- among those with a dominant left vertebral artery), pres-
tions than men (4.7% versus 1.8%, respectively; ervation of any preexisting left internal mammary artery
P=0.013), with a higher rate of the need for iliac coronary bypass graft, as well as left upper extremity
and aortic access or surgical conduit, as well as dialysis access or other left upper extremity-based graft.

Currently, pivotal as well as feasibility trials are ongoing the SMA and celiac artery-based collaterals. In
CLINICAL STATEMENTS
for branched endografts intending to preserve flow to patients undergoing TEVAR with celiac artery cov-
AND GUIDELINES
the left subclavian artery. Longer-term follow-up of this erage who have adequate collateralization on CTA,
technology is needed, but initial results are promising.5,6 angiography, or both, a small percentage of patients
go on to develop postoperative visceral ischemia.
Although the risk of visceral ischemia after celiac
Recommendation-Specific Supportive Text artery coverage with TEVAR is relatively low, there
1. Up to 40% of patients undergoing TEVAR for thoracic remains a finite risk (3.2% in largest clinical series)3
aneurysm repair require left subclavian artery cover- for visceral ischemic complications, which can lead
age. Preoperative left subclavian revascularization to death.
has been shown to decrease the rates of stroke2,7,8
and SCI.1,2 Vertebrobasilar insufficiency and left arm 6.5.3.5. Ruptured Descending TAA
ischemia can also occur without left subclavian artery Recommendations for Ruptured Descending TAA
revascularization.9,10 Patients with a patent left inter- Referenced studies that support the recommendations are
nal mammary artery to left anterior descending artery summarized in the Online Data Supplement.
coronary artery bypass graft, or who are otherwise COR LOE Recommendations
reliant on inflow from the left subclavian artery (eg, 1. In patients with ruptured descending TAA who
for dialysis access), should undergo left subclavian are anatomic candidates for endovascular
1 B-NR repair, TEVAR is recommended over open
revascularization to preserve flow.9
repair because of decreased perioperative
2. Patients undergoing TEVAR with left subclavian death and morbidity.1-5
coverage may not be hemodynamically stable 2. In patients with ruptured descending TAA
enough to undergo preemptive revascularization of undergoing TEVAR, intentional coverage of
the left subclavian artery. If such patients go on to 2b B-NR the left subclavian artery, celiac artery, or both
may be considered to increase the landing
develop SCI after TEVAR, there have been case zone for endovascular repair.5-7
reports of SCI reversal with secondary revascular-
ization of the left subclavian artery.3
Synopsis
6.5.3.4. Celiac Artery Management Ruptured TAA carry a high mortality rate. Single-center
Recommendation for Celiac Artery Management data, meta-analyses, and clinical trials have all shown
References that support the recommendation are included in the the lower rates of perioperative death and complica-
Online Data Supplement.
tions associated with endovascular versus open surgical
repair.1-5 However, the survival advantage shown in Medi-
1. In patients with descending TAA undergoing care-based claims data disappears after 1.5 years,4 and
TEVAR in whom celiac artery coverage is
2a B-NR
being considered, it is reasonable to first con- single-institution series1,3 reflect the frequent need for
firm adequate collateralization.1 reintervention over time. Furthermore, a meta-analysis2
showed that aneurysm-related survival was decreased in
the TEVAR group over time, underscoring the importance
Synopsis of continued surveillance in this high-risk population.
Celiac artery coverage is estimated to be necessary
in 15% of patients undergoing TEVAR for descend-
ing TAA repair.2 The safety and use of this practice has Recommendation-Specific Supportive Text
previously been shown with single-institution series cit- 1. For repair of ruptured descending TAA, TEVAR is
ing low incidence of postoperative visceral ischemia. associated with decreased perioperative morbid-
However, despite the preoperative evaluation with CTA, ity and mortality compared with open repair. In 1
angiography, or both to confirm adequate collateraliza- retrospective multi-institution study, TEVAR had a
tion between the celiac and superior mesenteric artery lower composite rate of death, stroke, and perma-
(SMA), a small percentage of patients still die from vis- nent paraplegia compared with open surgery and
ceral ischemia. In addition, late distal migration of the a trend toward lower aneurysm-related mortality
endograft can encroach on the SMA, creating SMA at 4 years.1 Similarly, a meta-analysis showed that
stenosis and compromising flow through the SMA and TEVAR was associated with a lower perioperative
celiac-based collaterals. mortality and myocardial infarction rate compared
with open repair.1 A multicenter, prospective clinical
trial for aortic catastrophes—including aortic rup-
Recommendation-Specific Supportive Text ture—showed that TEVAR was superior with regard
1. Migration of the endograft distally over time can to the composite endpoint of mortality and para-
cause stenosis of the SMA and decrease flow to plegia, compared with open repair.5 Although the

perioperative benefit of endovascular repair of rup- of patients because of small or diseased access
CLINICAL STATEMENTS
tured TAA was again corroborated in a Medicare- vessels.6-8

AND GUIDELINES
claims dataset, the survival advantage with TEVAR 2. Alternative access was required in up to 21.1%
disappeared after 1.5 years.4 of patients undergoing TEVAR in the clinical
2. When ruptured descending TAA present, coverage device trials.8 Women have a higher incidence of
of the left subclavian artery, celiac artery, or both smaller diameter external iliac arteries compared
may be necessary to gain the necessary 2 cm of with men.1,2 Direct aortic or iliac artery exposure,
seal zone for successful endovascular repair. Left iliac conduits, or endovascular techniques may
subclavian artery and celiac artery coverage during be used to facilitate safe delivery of endografts
thoracic aortic rupture has been associated with during TEVAR.1,2 Preoperative case planning will
reasonable technical success and outcomes in sin- enable safe delivery of endografts without vascular
gle-institution series6 and 1 clinical trial5 in patients complications.
with acute rupture or complicated dissection of the 3. Percutaneous access for delivery of TEVAR has
descending thoracic aorta. been performed safely and with a high degree of
success, as shown in single-institution4,5 as well
6.5.3.6. Access Issues for TEVAR in Descending TAA as multi-institution registries.3 Technical success
Recommendations for Access Issues for TEVAR in Descending TAA ranged from 94.4% to 98.9%, and percutaneous
Referenced studies that support the recommendations are access was associated with fewer complications
and a shorter length of stay compared with those
COR LOE Recommendations with surgical cutdown.
1. In patients with descending TAA undergoing
TEVAR, review of preoperative CTA of the
1 B-NR 6.5.4. Thoracoabdominal Aortic Aneurysms
iliofemoral vessels should be performed to
evaluate access.1,2
6.5.4.1. Size Thresholds for Open Surgical Repair of TAAA
2. In patients with descending TAA undergoing
Recommendations for Size Thresholds for Open Surgical Repair of
TEVAR, if iliac access is marginal or inade-
TAAA
1 B-NR quate to prevent access-related complications,
the use of alternative conduits is recom-
mended.1,2
3. In patients with descending TAA undergo-
ing TEVAR who have suitable anatomy, 1. In patients with intact degenerative TAAA,
total percutaneous femoral access is a 1 B-NR repair is recommended when the diameter is
2a B-NR
reasonable alternative to open surgi- ≥6.0 cm.1-3
cal cutdown to avoid access-related
complications. 3-5 2. In patients with intact degenerative TAAA,
repair is reasonable when the diameter is
2a B-NR ≥5.5 cm and the repair is performed by expe-
rienced surgeons in a Multidisciplinary Aortic
Synopsis Team.1-3
Iliac artery access for stent-graft delivery systems is 3. In patients with intact degenerative TAAA
who have features associated with an
marginal in up to 21% of cases in which TEVAR is per-
2a B-NR increased risk of rupture (Table 19),
formed for descending TAA.1 Careful review of the CTA repair is reasonable when the diameter is
of the iliofemoral system is required to ensure that mar- <5.5 cm.4
ginal or inadequate access is noted and properly man-
aged. Marginal access can be successfully circumvented
using surgical bypass, direct aortic or iliac exposure, or
Synopsis
endovascular techniques to treat vessel stenosis. Percu- The data supporting aortic diameter thresholds for either
taneous access was used successfully for endovascular open or endovascular repair of TAAA are similar to that
abdominal aortic repair before it was applied to larger presented for repair of descending TAA (see Section
sheath devices. This technology has also been applied 6.5.3, “Descending Thoracic Aortic Aneurysms”). All
to TEVAR with a similarly high degree of success and are single-institution series with longitudinal follow-up
reduced hospital length of stay.
Table 19. Features Associated With an Increased Risk of
TAAA Rupture
Recommendation-Specific Supportive Text Rapid growth (confirmed increase in diameter of 0.5 cm/y)
1. Thoracic endovascular stent grafts are housed in Symptomatic aneurysm
large delivery systems, thus thorough review of
Significant change in aneurysm appearance
the iliofemoral system is required to avoid access
Saccular aneurysm or presence of penetrating atherosclerotic ulcers
complications. In the clinical device trials, alter-
native access was required in 9.4% to 21.1% TAAA indicates thoracoabdominal aortic aneurysm.

via surveillance imaging and detection of aortic-related 6.5.4.2. Open Versus Endovascular Repair of TAAA
CLINICAL STATEMENTS
events and death. Intervention at diameters of <6.0 cm Recommendations for Open Versus Endovascular Repair of TAAA
AND GUIDELINES
would reduce aortic-related events and death. There Referenced studies that support the recommendations are
are also conditions in which intervention may be justi-
fied at smaller diameters (eg, rapid growth, symptoms, COR LOE Recommendations
penetrating ulcers, mycotic aneurysms, connective tissue Ruptured TAAA

disorders). Concerns for operative death in the setting 1. In patients with ruptured TAAA requiring inter-
1 B-NR
of comorbid conditions is certainly justified. However, in vention, open repair is recommended.1-5
centers with a Multidisciplinary Aortic Team, excellent 2. In patients with ruptured TAAA requiring inter-
outcomes can be obtained despite the presence of such vention, provided that the patient is hemo-
dynamically stable, endovascular repair may
conditions, and fatal aortic events may thus be avoided. 2b C-LD
be reasonable in centers with endovascular
expertise and access to appropriate endovas-
cular stent grafts.6
Recommendation-Specific Supportive Text Intact TAAA
1. Aortic event rates begin to rise significantly, and 3. In patients with Marfan syndrome, Loeys-Dietz
5-year survival begins to fall when TAAA diame- syndrome, or vascular Ehlers-Danlos syn-
1 C-LD drome and intact TAAA requiring intervention,
ters are >6.0 cm. At this diameter, the risk of an open repair is recommended over endovascu-
adverse aortic event ranges from 9.3%1 to 19%,3 lar repair.7-9
which is 2 to 4 times the median operative mortal- 4. In patients with intact degenerative TAAA
ity rate for open TAAA repair. In patients with mul- and suitable anatomy, endovascular repair
tiple comorbidities known to substantially increase with fenestrated stent grafts, branched
2b B-NR stent grafts, or both may be considered in
the risk of open TAAA repair (eg, chronic obstruc- centers with endovascular expertise and
tive pulmonary disease, advanced age, preopera- access to appropriate endovascular stent
tive renal dysfunction, preoperative left ventricular grafts.10-13
dysfunction), it may be appropriate to continue to

observe patients with TAAA diameters >6.0 cm or
to refer them for endovascular repair. Synopsis
2. In centers with a Multidisciplinary Aortic Team, There are no RCTs comparing early or late outcomes
despite the presence of comorbid conditions, for open versus endovascular repair for TAAA. As of
excellent outcomes can be achieved with meticu- November 2022, there are no FDA-approved devices for
lous perioperative preparation and care as well as endovascular TAAA repair. Most of the endovascular pro-
technically sound surgery. On multivariable analy- cedures currently performed are done so with customized
sis, patients undergoing TAAA repair with a left fenestrated or branched endografts on investigational
ventricular ejection fraction <40% were not more device exemption- or industry-sponsored trials. Although
prone to operative death (OR, 0.28; 95% CI, 0.02– the number of endovascular repairs performed has been
4.14; P=0.58) or long-term death (OR, 0.55; 95% steadily increasing, follow-up remains limited, and open
CI, 0.17–1.80; P=0.23) than those with higher repair therefore remains the preferred therapy for patients
ejection fractions.5 Similarly, carefully selected with TAAA who require intervention. The results for open
octogenarians undergoing open TAAA repair had a repair are excellent in centers with a Multidisciplinary Aor-
similar operative mortality rate as those <80 years tic Team. In the largest series published to date, the oper-
of age (5.2% versus 5.7%; P=0.852).6 ative mortality rate in 3 309 patients undergoing open
3. Certain clinical factors associated with an TAAA repair was 7.5%, including >1 000 patients under-
increased risk of TAAA rupture may prompt con- going repair of an extent II aneurysm, with a low risk of
sideration of open or endovascular intervention aortic-related reintervention. Other high-volume centers
at a diameter below the standard surgical thresh- have reported similar outcomes for open repair. In 1 cen-
olds. In patients with intact TAAA who are being ter, the operative mortality rate in 783 patients was 5.6%,
observed with surveillance imaging, confirmed with a low risk of SCI of 2.0% and need for postoperative
rapid aneurysm growth (≥0.5 cm/y) would suggest hemodialysis of 5.2%. Another center, whose operators
the need for intervention regardless of absolute used deep hypothermic circulatory arrest, reported an
diameter.4 Symptoms consistent with an enlarging operative mortality rate of 6.8% with an SCI risk of <3%
TAAA that are not attributable to alternative pathol- and postoperative hemodialysis risk of 2.2%.
ogy portend potential rupture and also suggest the
need for surgery.7 Patients with symptoms second-
ary to either PAU or saccular aneurysms are also at Recommendation-Specific Supportive Text
a higher risk for rupture and should be considered 1. In patients with ruptured TAAA, open repair can
for intervention regardless of absolute diameter.8 be performed with low mortality by surgeons in

centers with a Multidisciplinary Aortic Team. In a no mortality rate, 100% technical success, and 1
CLINICAL STATEMENTS
series of 100 consecutive patients with ruptured reintervention at mean follow-up of 34 months.6
AND GUIDELINES
TAAA, an operative mortality rate of 14% and Endovascular repair may be reasonable in patients
an SCI rate of 5% was achieved.5 Although the who failed previous open repair or are considered
study population was replete with comorbid con- high risk and have stent-grafts placed into synthetic
ditions, the only risk factor remaining significant landing zones, or when used as a bridge to open
after propensity matching was “shock” on arrival repair in patients with hemodynamic instability.
to the hospital. Furthermore, 5-year actuarial sur- 4. Single- and multi-institution series of physician-
vival was 47.5%. Centers experienced in complex sponsored investigational device exempt trials have
endovascular repair may opt to use this technique. shown the promise of fenestrated and branched
In a national registry of 140 ruptured descending endovascular stent grafts. When performed by
aneurysms, the operative mortality rate (10%) was experienced surgeons, technical success may be
good, but there was a disappointingly high rate of achieved in a high percentage of cases (92%–
stroke (14.7%), SCI (9.6%), and need for reinter- 99.6%) with low perioperative mortality rate. At
vention within 30 days (19.7%). At a median fol- 1-year follow-up imaging, branch vessel patency
low-up of 17 months, actuarial 5-year survival rate was also good (96%–98%) and, at 3 years, free-
was 31.9%. These results were similar to those dom from aortic-related death was 91% and over-
reported from a device registry.1,5 Although com- all survival 57%.15
plex endovascular repair of intact TAAA has shown
promise in experienced hands and in select cen- 6.5.4.3. TAAA Spinal Cord Protection
ters, in the setting of TAAA rupture, the endovascu-
Recommendations for TAAA Spinal Cord Protection
lar approach is hampered by patient instability and Referenced studies that support the recommendations are
the need for customized grafts (which may take summarized in the Online Data Supplement.
several weeks to manufacture). In addition, most COR LOE Recommendations
of the reported series of endovascular repair of 1. In patients undergoing open TAAA repair who
ruptured TAAA are small; larger series with longer- are at high risk for SCI, cerebrospinal fluid
term follow-up will be necessary to delineate the 1 A drainage is recommended to reduce the inci-
dence of temporary SCI, permanent SCI, or
role for endovascular repair in the setting of aortic both.1-7
rupture.
2. In patients who experience delayed spinal

2. Endovascular repair requires sequential steps for cord dysfunction after either open or endo-
successful stenting of side branches without the 1 B-NR
vascular TAAA repair, timely measures to
optimize spinal cord perfusion and decrease
ability to achieve rapid control of hemorrhage. intrathecal pressure are recommended
Therefore, the role of off-the-shelf branched repair (Table 20).1-4,8
has been limited in patients with ruptured aneu-
rysms and hemodynamic instability. However, in
higher-risk patients who present with symptomatic Synopsis
or contained ruptured aneurysms, are hemody- SCI is a devastating complication of open and endo-
namically stable, and have suitable anatomy, endovascular thoracoabdominal aneurysm repair, with an
vascular repair with an off-the-shelf or modified incidence rate of 2% to 15%, depending on aneurysm
device may be considered. Kolbel et al14 reported a extent and cause, underlying patient comorbidities,
mortality rate of 15% for symptomatic and 30% for urgency of the procedure, and surgeon and center expe-
ruptured TAAA treated by multibranch endovascu- rience. Previous ACC/AHA guidelines did not address
lar repair. the issue other than to suggest higher-risk populations
3. In patients with known or suspected connec- that might benefit from adjuncts to reduce the incidence
tive tissue disorders, such as Marfan syndrome, of SCI.9 The 2014 European guidelines assigned cere-
Loeys-Dietz syndrome, or vascular Ehlers-Danlos brospinal fluid drainage a I B recommendation to reduce
syndrome, open repair is recommended. Operative the risk of SCI.10 However, data were limited at the time
mortality rate is lower than in the general popula-
tion undergoing open TAAA repair, as is the inci- Table 20. Measures to Optimize Spinal Cord and End-Organ
dence of major complications, such as stroke and Perfusion
SCI. Importantly, freedom from aortic reintervention Cardioversion for tachyarrhythmias
is excellent, as is long-term survival. Conversely, Insertion of cerebrospinal fluid drain
data are lacking on complex endovascular repair of
Increase mean arterial pressure to >100 mm Hg
TAAA for patients with connective tissue disorders.
Transfuse to a hemoglobin >10 g/dL
A small study of 17 patients treated by fenestrated-
branched endovascular aortic repair (FEVAR) had Volume resuscitation

to support this recommendation, and an earlier RCT11 deficits anytime in the first 2 weeks postoperatively.
CLINICAL STATEMENTS
had not shown a benefit for cerebrospinal fluid drainage The reported incidence of delayed SCI is approxi-
AND GUIDELINES
in TAAA repair. A more recent RCT did show a significant mately 5%, nearly twice that of deficits recognized
reduction in SCI for a cohort undergoing repair of exten- immediately after surgery. Delayed deficits usu-
sive TAAA (extent I and extent II) when cerebrospinal ally present in the setting of a hemodynamic insult
fluid drainage was used. Additional nonrandomized data (atrial fibrillation, hypovolemia, hemorrhage, infec-
support this recommendation. tion) and may be responsive to aggressive mea-
Delayed SCI may occur up to 2 weeks after surgery. sures to optimize spinal cord perfusion (Table 20).
This complication has a profound impact on short- and Cerebrospinal fluid drainage immediately reduces
long-term outcomes.10,12 Early recognition and aggres- intrathecal pressure and increases spinal cord
sive management of SCI can lead to a return of lower perfusion pressure (spinal cord perfusion pres-
extremity function. The reinsertion of a cerebrospinal sure equals mean arterial pressure minus spinal
fluid drain is a key component to salvage lower extrem- cord fluid pressure).8,12,14 A significant proportion
ity function. Additional therapies, such as volume loading, (57%) of patients with late deficits experience an
increasing mean arterial pressure, and maximizing oxy- improvement in their neurologic examination, with
gen delivery to the cord through transfusion or supple- 17% having complete resolution of their deficits.14
mental oxygen, are also critical. The operative mortality rate for those with persis-
tent SCI is nearly 3-fold higher than for those who
recover (38% versus 13%, respectively; P<0.001).
Recommendation-Specific Supportive Text In addition, 5-year survival is significantly worse
1. TAAA repair remains a formidable undertak- (from 75% with a return of function to 28% with-
ing regardless of whether open or endovascular out; P<0.001).14
repair is performed. SCI, with either paraparesis or
paraplegia, may be temporary or permanent and 6.5.4.4. TAAA Renal and Visceral Organ Protection
has a profoundly negative impact on short- and
Recommendations for TAAA Renal and Visceral Organ Protection
long-term survival as well as quality of life after Referenced studies that support the recommendations are
repair. Many techniques have been suggested to summarized in the Online Data Supplement.
reduce the incidence of this significant complica- COR LOE Recommendations
tion. Intraoperative management ranges from deep
1. In patients undergoing open repair of TAAA

hypothermic circulatory arrest to left heart bypass involving the renal arteries, cold blood or crys-
1 A
to a “clamp-and-sew” technique, and support exists talloid renal perfusion is recommended to pro-
vide effective protection against renal injury.1-6
for each approach. Similarly, intraoperative and
postoperative spinal cord neuromonitoring is not 2. In patients undergoing open or endovascular
TAAA repair who have end-organ ischemia or
widespread but has support that is institutionally 1 B-NR significant stenoses from atherosclerotic vis-
based. Other interventions have been also advo- ceral or renal artery disease, additional revas-
cated as intrathecal papaverine to enhance spinal cularization procedures are recommended.7
cord protection.13 Cerebrospinal fluid drainage

remains the only technique proven to reduce the
incidence of perioperative SCI. In an RCT exam-
Synopsis
ining the incidence of SCI in patients undergoing Postoperative renal dysfunction after open TAAA repair
high-risk extent I and II TAAA repair, cerebrospi- has a significantly negative impact on short- and long-
nal fluid drainage was associated with a signifi- term mortality as well as quality of life. Efforts to reduce
cant reduction in SCI compared with those having renal injury during open TAAA repair include local organ
surgery without cerebrospinal fluid drainage. Over hypothermia with either cold crystalloid or cold blood-
the past decade, there are few centers performing based perfusate.
open TAAA repair without the aid of cerebrospinal
fluid drainage. Furthermore, patients undergoing
endovascular repair requiring extensive descending Recommendation-Specific Supportive Text
thoracic aorta coverage or in the setting of a pre- 1. Renal dysfunction after TAAA repair is defined as
vious infrarenal aneurysm repair also benefit from a doubling of the creatinine or the need for hemo-
cerebrospinal fluid drainage (nonrandomized).6 dialysis. When this significant complication occurs,
2. In patients undergoing open TAAA repair, delayed short- and long-term survival is compromised, and
paraplegia may account for nearly 60% of all spi- the incidence of postoperative respiratory failure,
nal cord deficits encountered. Despite having an SCI, and cardiac complications increase. To iden-
intact neurologic examination immediately after the tify methods to reduce the incidence of postop-
procedure, patients can experience these delayed erative renal dysfunction, 2 RCTs were performed

comparing cold crystalloid renal preservation to nor- for those with suitable common femoral artery
CLINICAL STATEMENTS
mothermic blood perfusate and, subsequently, cold anatomy.3 In the PiERO (Percutaneous femoral
AND GUIDELINES
blood perfusate. When compared with normother- access in Endovascular Repair versus Open femo-
mic blood delivered into the renal arteries directly ral access) study, investigators evaluated whether
from the left heart bypass circuit, the delivery of cold ultrasound-guided percutaneous access via the
crystalloid perfusate into the renal arteries during common femoral artery decreased the risk of
open TAAA repair resulted in a 3-fold reduction in surgical site infections compared with cutdown.
the incidence of postoperative renal dysfunction.7 Although the incidence of surgical site infections
Subsequently, cold blood perfusate delivered to was too low to produce a difference in outcomes,
the renal arteries through occlusion or perfusion investigators found that, compared with open cut-
catheters was found to provide the same level of down for access, groins accessed and closed per-
renal protection as cold crystalloid perfusate during cutaneously healed faster and patients reported
open TAAA repair.5 The results of this second RCT less pain.1 Although the PEVAR trial did not require
provided surgeons with 2 options for renal protec- ultrasound-guided femoral access, it was routine in
tion when open TAAA repair requires renal artery the PiERO trial. Furthermore, a multicenter obser-
reconstruction. vational study of common femoral artery access
2. In patients with renal or visceral artery stenoses showed a significant decrease in groin hematomas
or ostial obstruction secondary to chronic or acute with routine ultrasound-guided access.4 Lastly, in
dissection flaps, end-organ perfusion may be com- an extensive comparison of different closures using
promised. Improvement in perfusion to the celiac data from 13 087 patients in the Vascular Quality
axis, SMA, and both renal arteries may be achieved Initiative registry, there was a significantly higher
by bypass, endarterectomy, or balloon angioplasty rate of cardiac complications (OR, 1.5; 95% CI,
and stent placement. Patency of target vessel 1.14–2.05) and 30-day mortality rate (OR, 1.56;
revascularization strategies has been documented 95% CI, 1.05–2.32)2 in those undergoing cutdown
in small series of patients having open TAAA repair versus percutaneous access.2 Operative time, esti-
with a “debranching” technique and in those under- mated blood loss, and length of stay were all signifi-
going endovascular TAAA repair. cantly higher in those undergoing groin cutdowns
compared with percutaneous access.
6.5.5. Abdominal Aortic Aneurysms

6.5.5.2. Repair of Ruptured AAA
6.5.5.1. Access During Endovascular Repair of AAA Recommendations for Repair of Ruptured AAA
Recommendation for Access During Endovascular Repair of AAA Referenced studies that support the recommendations are
Referenced studies that support the recommendation are summarized summarized in the Online Data Supplement.
in the Online Data Supplement.
1. In patients presenting with ruptured AAA
1. In patients undergoing endovascular repair who are hemodynamically stable, CT imag-
of AAA who have suitable common femoral 1 B-R ing is recommended to evaluate whether
artery anatomy, ultrasound-guided percutane- the AAA is amenable to endovascular
1 B-R ous access and closure is recommended over repair.1-3
open cutdown to reduce operative time, blood
2. In patients presenting with ruptured AAA
loss, length of stay, time to wound healing, and
who have suitable anatomy, endovas-
pain.1,2
1 B-R cular repair is recommended over open
repair to reduce the risk of morbidity and
mortality.1,4-6
Synopsis 3. In patients undergoing endovascular repair for
Increased availability of percutaneous closure devices 2a B-NR
ruptured AAA, local anesthesia is preferred to
general anesthesia to reduce risk of periop-
and lower profile endovascular stent grafts have made erative mortality.7-9
ultrasound-guided percutaneous access and closure
4. In patients with ruptured AAA, permissive
more feasible. Two RCTs and a large national retrospec- 2a C-LD hypotension can be beneficial to decrease the
tive review showed favorable outcomes from percutane- rate of bleeding.1,3,10-12
ous common femoral artery access and closure such as
reduced operative time, reduced blood loss, and improved
patient-centered outcomes, such as reduced pain. Synopsis
The mortality rate from ruptured abdominal aortic aneu-
rysms (rAAA) is estimated to be 80% to 90%, with most
Recommendation-Specific Supportive Text patients never reaching the hospital.13 For those who
1. The PEVAR trial showed the noninferiority of present to a hospital, the historical mortality rate for open
total percutaneous access and closure for EVAR repair was approximately 50%. With improved team

organization, prompt diagnosis, and endovascular repair 0.36–0.9).16 Contemporary observational studies
CLINICAL STATEMENTS
options, the mortality rate after repair for rAAA has been showed significant survival benefit from an endo-
AND GUIDELINES
reported to be as low as 18.5% after instituting an endovascular approach to rAAA. For example, Wang
vascular repair-first strategy in at least 1 observational et al6 used propensity-matched data from the
series.1 Initial randomized trials for endovascular repair Vascular Quality Initiative registry and showed that
for rAAA (rEVAR) versus open repair generally showed rEVAR resulted in a lower 30-day mortality rate
no early survival benefit. However, shortcomings of these than open repair (21% versus 34%, respectively;
trials raised questions about their applicability.2,14,15 Lon- P<0.001) and that mortality rates after rEVAR
ger-term studies of rEVAR, such as 3-year results from have been steadily decreasing since 2008. Other
the IMPROVE (Immediate Management of the Patient studies have corroborated this general decline in
With Rupture: Open Versus Endovascular Repair) trial, the rEVAR mortality rate and comparatively better
showed late survival benefit from rEVAR over open repair. postoperative outcomes.4,17 Newer endovascular
Many authors have evaluated institutional experience devices have enabled treatments of rAAA that do
with using rEVAR in anatomically suitable candidates not necessarily meet instructions for use criteria.
and aimed to improve the process of care for rAAA by However, caution should be exercised, because
adopting “rupture protocols” that include early imaging, observational studies showed increased risk of
permissive hypotension, endovascular balloon occlusion perioperative death and long-term complica-
under fluoroscopy to reduce excessive bleeding, and a tions when devices are used off-label in a rupture
team-based organization to facilitate immediate transfer scenario.18,19
of patients to the operating room for prompt hemorrhage 3. Patients presenting with rAAA often maintain
control and repair.1,3 adequate BPs, in part because of the body’s cat-
echolamine responses.20 However, once induced
with general anesthesia, the loss of this physiologic
Recommendation-Specific Supportive Text response—coupled with anesthetic agents that can
1. The IMPROVE trial was the first trial to evaluate depress BP—can lead to circulatory collapse.21-23
a new paradigm in evaluating rAAA.2 Specifically, General anesthesia has also been shown to have
patients who were hemodynamically stable were deleterious effects on inflammatory and body
first transported to the radiology suite for CTA temperature regulation.24,25 Subanalysis of the
to assess whether their ruptured aneurysm was IMPROVE trial showed that patients with rAAA who
amenable to endovascular repair or required open underwent EVAR with only local anesthesia had
repair. This is in contrast to a strategy of transport lower risk of mortality compared with those who
to the operating room for open surgery without were treated under general anesthesia (adjusted
preoperative imaging. The trial did not identify any OR, 0.27; 95% CI, 0.1–0.7).7 Although the trial
increased risk of death from a strategy of acquiring was not designed and powered for this specific
preoperative imaging and, because of the different outcome, recent observational studies from large
repair options available today, such assessments registries have corroborated this finding.8,9
can help surgeons choose appropriate therapy 4. Although there are no RCTs of outcomes specific
based on patient aneurysm anatomy and clinical to permissive hypotension in rAAA, data from the
status. In contemporary practice, many patients trauma literature evaluating fluid management in
will have a CT scan, although some of these scans hemorrhagic shock show benefit in using a strat-
will not be ideally timed arterial phase imaging. egy of permissive hypotension.11,12 Many authors
Given that time is of the essence in rAAA repair, managing rAAA have similarly described main-
if a patient’s CT scan provides enough anatomic taining low arterial pressures to decrease rate of
information to identify whether endovascular repair bleeding in patients with rAAA.1,3,10 An SBP that
is feasible, another more dedicated CTA scan may allows a patient to maintain mentation, typically
add unnecessary delays to the patient’s care. between 60 and 90 mm Hg, is suggested.
2. Although 3 clinical trials aimed to evaluate poten-
tial survival benefit for rEVAR over open repair,
6.5.5.3. Threshold for AAA Repair
none showed significant early benefit. However, tri-
Recommendations for the Threshold for AAA Repair
als excluded patients who were hemodynamically Referenced studies that support the recommendations are
unstable, thus excluding patients that may have summarized in the Online Data Supplement.
benefitted most from an endovascular approach. It COR LOE Recommendations
should be noted, however, that the IMPROVE trial
1. In patients with unruptured AAA, repair is rec-
subsequently showed that between 90 days and ommended in those with a maximal aneurysm
1 A
3 years, rEVAR had superior survival rates com- diameter of ≥5.5 cm in men or ≥5.0 cm in
women.1-6
pared with open repair (hazard ratio, 0.57; 95% CI,

Recommendations for the Threshold for AAA Repair (Continued) of rupture, a threshold of ≥5.5 cm is acceptable
CLINICAL STATEMENTS

for men with infrarenal AAA. In the UKSAT study,
AND GUIDELINES
which included more women than the previous

2. In patients with unruptured AAA who have
symptoms that are attributable to the aneu- studies, women were found to have higher rates
1 B-NR
rysm, repair is recommended to reduce the of aneurysm rupture and higher rates of aneu-
risk of rupture.7,8 rysm-related deaths than men.2,3 The mean maxi-
3. In patients with unruptured saccular AAA, mum aneurysm diameter at rupture was 5.0 cm in
2b C-LD intervention to reduce the risk of rupture may
be reasonable.9
women and 6.0 cm in men. More recent data high-
light a different method for quantifying aneurysm
4. In patients with unruptured AAA and aneu-
rysm growth of ≥0.5 cm in 6 months, repair rupture risk by indexing aneurysm size to the BSA
2b C-LD
to reduce the risk of rupture may be reason- (ASI equals aneurysm diameter [cm]/BSA [m2]); in
able.1-5 women, ASI has been shown to be more predictive
of rupture risk than is maximum diameter.12 Further
Synopsis research will help clarify whether ASI is a better
One of the most significant risk factors for continued metric for aneurysm repair thresholds than maxi-
aneurysm growth and rupture is the maximum diameter. mum diameter.12
Thresholds for AAA repair must balance the expected 2. Approximately 6% to 22% of treated aneurysms
risk of rupture against the risk of operative interven- are symptomatic but unruptured. Symptoms that
tion. Historically, the risk of rupture was reported to be are considered high risk for impending rupture
0.5% to 5% for aneurysms <5 cm in maximum diameter, include pain in the back, abdomen, or flank, and
3% to 15% for aneurysms 5 cm to 6.9 cm, and ≥30% sometimes radiating to the groin, which is attrib-
for aneurysms ≥8 cm.10 Multiple trials that are now >2 utable to the AAA. Patients presenting with such
decades old evaluated the use of early repair of AAAs symptoms should be admitted to an ICU for arte-
measuring 4.0 cm to 5.4 cm via open or endovascular rial BP monitoring, tight BP control, medical opti-
means. All found no survival benefit attributable to early mization, and AAA repair, ideally in 24 to 48 hours
repair and but did find an increased risk of subsequent to reduce risk of free rupture. Other symptoms
reintervention. These studies and others have found that that warrant expedited, although not necessarily
rupture does occur at smaller diameters for women; thus, urgent AAA repair, include tenderness to palpation
overlying the AAA in the abdomen, back, or flank,
size thresholds for men and women differ to account

for these observed differences.6,11 Newer data highlight embolism (eg, blue toe syndrome) or compressive
other considerations, such as aortic indexing, which may symptoms (eg, obstructive uropathy). Observational
better predict aneurysm rupture risk. Lastly, although lim- studies show that patients treated for symptomatic
ited data exist for the natural history of saccular AAAs, aneurysms have higher mortality and morbidity
available data suggest that their morphologic features rates than those treated electively.7,8 Although tim-
may make them more likely to become symptomatic, ing of repair of symptomatic aneurysms remains
rupture at smaller diameters, or both than fusiform AAAs. controversial, most studies have reported out-
comes of symptomatic aneurysms repaired during
a patient’s index operation, with some studies find-
Recommendation-Specific Supportive Text ing that performing surgery on a nonemergency
1. Clinical trials conducted in the late 1990s and basis and potentially optimizing patient’s cardiore-
early 2000s, including the UKSAT (UK Small spiratory status during their hospitalization may be
Aneurysm Trial) and ADAM (Aneurysm Detection advantageous.8,13-15
and Management) trial for early open aneurysm 3. Saccular AAAs are rare and, consequently, there
repair and CAESAR (Comparison of surveillance are limited natural history data. In a Dutch regis-
vs. Aortic Endografting for Small Aneurysm Repair) try of patients treated for fusiform and saccular
and PIVOTAL (Positive Impact of endoVascular AAAs, researchers found that saccular aneurysms
Options for Treating Aneurysm earLy) trials for early appeared more common in women and were more
endovascular repair, did not find a survival benefit likely to be symptomatic at smaller sizes than fusi-
for repair of aortic aneurysms measuring 4.0 cm form aneurysms.9 Of 7 659 patients with AAA,
to 5.4 cm.1-5 Although long-term outcomes in the 6.1% had saccular AAA. Of patients with saccular
UKSAT group seemed to show better survival rates aneurysms and acute presentation, 25% had diam-
in patients in the early open surgery group, this was eters <5.5 cm, and 8.4% had diameters <4.5 cm.
thought to be attributable to higher rates of smok- In contrast, only 8.1% and 0.6% of patients with
ing cessation in the early surgery group compared fusiform AAA presenting acutely had diameters
with the surveillance group.2,3 Based on these data, <5.5 cm and <4.5 cm, respectively. In their 2017
balancing the risk of intervention versus the risk guidelines on AAA,16 the Society for Vascular

Surgery recommended elective repair of patients data support either approach. Historic RCTs evaluating
CLINICAL STATEMENTS
presenting with saccular AAA, although size guid- outcomes of EVAR versus open repair showed an ini-
AND GUIDELINES
ance is lacking because of limited natural history tial survival advantage for EVAR that dissipates at differ-
data. Clearly, the decision to intervene must be ent time intervals.1,3-8 Contemporary investigations have
informed by the patient’s individual anatomy. shown a steady decline in mortality rates for EVAR in
4. Pooled analysis from thousands of patients general20 and a much larger perioperative survival ben-
included in AAA surveillance studies from North efit from EVAR versus open repair.9 However, similar to
America, Western Europe, and East Asia showed historic clinical trials, these survival benefits can dissipate
that, although aneurysm growth is highly variable, over time and must be weighed against suboptimal sur-
growth rates range from 1.5 mm/y to 2 mm/y for veillance that can occur in those treated with EVAR, lead-
those with AAA of 3.0 cm to 3.9 cm and from 3.3 ing to higher rates of late rupture and associated death.21
mm/y to 5.7 mm/y in AAA of 4.0 cm to 5.9 cm For repair of juxtarenal aneurysms using FDA-approved
at baseline.17,18 The 4 major trials evaluating effi- fenestrated devices, available data show similar findings
cacy of early open and endovascular treatment of (ie, an initial survival benefit that may wane over time).10,11
AAA for small aneurysms all excluded patients with
aneurysms that grew ≥7 mm in 6 months or >10
mm in 12 months, given concern for increased risk Recommendation-Specific Supportive Text
of rupture. Thus, balancing the risks, aneurysms 1. Pooled data from 7 RCTs evaluating all-cause
with size increases of ≥0.5 cm in 6 months or ≥1 death after EVAR versus open surgery for infrare-
cm in 1 year are considered to be rapidly growing nal AAA repair show that the risk of perioperative
and may warrant consideration of repair. mortality is much lower in those treated with EVAR
(OR, 0.36; 95% CI, 0.2–0.66). This advantage per-
6.5.5.4. Open Versus Endovascular Repair of AAA sists at 6 months, after which survival from both
Recommendations for Open Versus Endovascular Repair of AAA approaches become equivalent. Moreover, after 8
Referenced studies that support the recommendations are years, those treated with EVAR have a higher risk of
aneurysm-related death (hazard ratio, 5.12; 95% CI,
1.6–16.4), secondary intervention (hazard ratio, 2.1;
1. In patients with nonruptured AAA with low 95% CI, 1.7–2.7), aneurysm rupture (OR 5; 95%
to moderate operative risk and who have
CI, 1.1–23.3), and death attributable to rupture (OR
anatomy suitable for either open or EVAR, a

1 A 3.6; 95% CI, 1.9–6.8) compared with open repair.22
shared decision-making process weighing the
risks and benefits of each approach is recom- Observational studies, such as the large propensity-
mended.1-11
matched study evaluating EVAR and open repair in a
2. In patients undergoing elective endovascular Medicare population, found that the survival advan-
repair for nonruptured AAA, adherence to
1 B-NR
manufacturer’s instructions for use is recom- tage for EVAR lasted longer among older patients.9
mended.12-16 For complex repairs, a similar survival advantage
3. In patients with nonruptured AAA and a high is seen for fenestrated repair over complex open
2a B-NR
perioperative risk, EVAR is reasonable to repairs in the first 30 days after surgery. More data
reduce the risk of 30-day morbidity, mortality,
or both.9,10
are necessary to identify longer-term outcomes and
to determine for which groups one approach may
4. For patients with nonruptured AAA, a
moderate to high perioperative risk, and
be more advantageous. Given the current clini-
anatomy suitable for an FDA-approved cal equipoise, engaging the patient in a process of
2a B-NR fenestrated endovascular device, endovas- shared decision-making is recommended, as further
cular repair is reasonable over open repair
to reduce the risk of perioperative compli-
detailed in Section 5, “Shared Decision-Making.”
cations.10,11,17,18 2. Patient-specific anatomical characteristics of the
aorta, such as neck diameter, length, and angulation,
and iliac seal diameter, length, and vessel access,
Synopsis must all be considered in endovascular repair. Some
Options for repair of AAA have substantially grown since observational studies show that treating aneurysms
the first description of open repair in 1952.19 In particular, outside of the manufacturer’s instructions for use
EVAR has made it possible to treat patients who may increases failure rates, resulting in increased risks
have never qualified for open surgery because of signifi- of graft migration, endoleaks, late rupture, and
cant cardiopulmonary comorbidities, renal comorbidities, deaths.12,13 For example, Shanzer et al12 found that
or both. With the abundance of options, clinicians must in a multicenter retrospective study of >10 000
remain informed regarding empirical data that may favor patients undergoing EVAR between 1999 and
one approach over another in a particular patient and 2008, patients with AAA treated with devices off
consider patient preferences for surgical options when instructions for use had significantly higher rates

of sac enlargement. More recently, Herman et al13 However, rates of late reintervention are higher
CLINICAL STATEMENTS
found that any deviation from instructions for use after FEVAR,11,18 as are the rates of persistent renal
AND GUIDELINES
increased risk of graft-related adverse events (haz- impairment11 and 3-year mortality rate (excluding
ard ratio, 1.8; 95% CI, 1.05–3.1). A meta-analysis perioperative deaths) (hazard ratio, 1.7; 95% CI,
of 17 studies found that patients treated with non- 1.1–2.6).17 Thus, similar to infrarenal repair, FEVAR
instructions for use higher overall mortality rates may be most beneficial for the moderate- to high-
(hazard ratio, 1.2; 95% CI, 1.02–1.42; P=0.03).14 risk surgical candidates who are more likely to
Given these findings, in most patients, treating off experience perioperative complications.
instructions for use in elective AAA repair is dis-
6.5.5.5. Treatment of Concomitant Common Iliac
couraged. Those who have been treated off instruc-
Aneurysms
tions for use warrant closer follow-up because of
higher rates of failure from endoleaks, graft migra- Recommendations for the Treatment of Concomitant Common Iliac
Aneurysms
tion, and late rupture. Referenced studies that support the recommendations are
3. EVAR-2 (UK Endovascular Aneurysm Repair 2) summarized in the Online Data Supplement.
was an RCT that evaluated outcomes of EVAR in COR LOE Recommendations
high-risk patients. Patients were enrolled if they 1. For patients with asymptomatic small
were determined to be unfit for open surgery, with AAA and concomitant common iliac artery
fitness assessed using cardiac, respiratory, and renal 1 C-LD aneurysm(s) ≥3.5 cm, elective repair of both
abdominal and iliac aneurysms is recom-
criteria.23 In these patients, the trial initially showed mended.1-4
that EVAR did not improve survival compared with
2. When treating common iliac artery aneu-
the control of no intervention; however, more than rysms or ectasia as part of AAA repair,
a decade later, those treated with EVAR had sig- 1 B-NR
preservation of at least 1 hypogastric
artery is recommended, if anatomically
nificantly lower aneurysm-related mortality (hazard
feasible, to decrease the risk of pelvic
ratio, 0.55; 95% CI, 0.34–0.91).24,25 Contemporary ischemia. 5,6
analyses of outcomes in high-risk patients show
that perioperative death after EVAR has markedly
decreased (eg, 9% in EVAR-2 versus 1.9% in the Synopsis
ACS national registry).26 Furthermore, in evaluating The prevalence of common iliac artery aneurysms in the
a propensity-matched Medicare population, postop-
presence of AAA has been reported to be as high as

erative complications that are more likely to affect 20% to 40% in surveillance studies.1,2 In patients with
high-risk patients, such as myocardial infarction, both aortic and iliac aneurysms, it is common for an iliac
pneumonia, acute renal failure, and need for dialysis, aneurysm to reach a size appropriate for elective repair
were all significantly less likely to occur after infra- before the AAA does. Although no randomized studies
renal EVAR compared with open repair.9 In assess- for iliac aneurysm repair size thresholds exist, in large
ing which patients are “high risk” for elective AAA case series and registry reports, rupture of iliac aneu-
repair, risk calculators derived using data from the rysms at diameters <4 cm is rare.3,7 Thus, a repair thresh-
Vascular Quality Initiative and the Vascular Study old of 3.5 cm seems reasonable to balance procedural
Group of New England can be helpful in informing risks with rupture risk. Furthermore, to achieve adequate
discussions with patients about repair options and AAA repair, repair of iliac artery ectasia or aneurysms
potentially identify patients for which even EVAR often may be required. Consideration of pelvic perfusion
would be of prohibitively high risk.27-29 is of great importance when managing concomitant iliac
4. Recent observational studies aimed to compare disease. In such cases, there is a high risk of ischemic
outcomes between open and endovascular repair complications from exclusion of internal iliac arteries that
for complex aortic aneurysms. Using propensity can lead to buttock claudication, bowel ischemia, and
score matching, investigators found that periopera- erectile dysfunction.5,6 For some patients, adequate treat-
tive mortality rates between patients undergoing ment of diseased iliac arteries cannot be accomplished
open repair or FEVAR were similar in those enrolled without internal iliac artery sacrifice. Thus, individualized
in the Vascular Quality Initiatives registry (4.7% ver- treatment plans with shared decision-making are impor-
sus 3.3%, respectively, P=0.17).17 Evaluating data tant when treating aorto-iliac aneurysm disease.
from the ACS, Varkevisser et al found much higher
odds of 30-day death from open repair compared
with FEVAR (OR, 4.9; 95% CI, 1.4–19).10 The risk Recommendation-Specific Supportive Text
of immediate postoperative complications, such as 1. In a large single-center case series by Huang et al,8
myocardial infarction, acute kidney injury, and the 438 patients with common iliac artery aneurysms
initiation of dialysis, is significantly higher after were observed for an average of 3.7 years. Eighty-
open complex repair compared with FEVAR.11,17,18 six percent of patients had current or previously

treated AAA. Common iliac artery aneurysms grew Recommendations for Surveillance After TAA Repair (Continued)
CLINICAL STATEMENTS
at an average rate of 2.9 mm/y, and no iliac aneu- COR LOE Recommendations
AND GUIDELINES
rysm ≤3.8 cm ruptured. A multinational retrospective
3. In patients treated with open repair of the
review of patients with internal iliac artery aneu- thoracic aorta without residual aortopathy,
rysms found that 41.7% of individuals had a con- 2a B-NR surveillance imaging with a CT or MRI within
comitant AAA. Of 63 patients, 1 patient presented 1 year postoperatively and then every 5 years
thereafter is reasonable.10-14
with a ruptured internal iliac artery aneurysm of ≤3
4. In patients treated with open repair of the
cm, and 4 individuals’ iliac aneurysms ruptured at thoracic aorta who have residual aortopa-
diameters ≤4 cm. Recently published data from the 2a C-EO thy or abnormal findings on surveillance
Dutch Surgical Aneurysm Audit showed that of the imaging, annual surveillance imaging is
reasonable.
857 patients with treated iliac artery aneurysms, the
median iliac artery aneurysm size at elective repair
was 4.3 cm, while ruptured iliac aneurysms had a
median diameter of 6.8 cm at presentation.
Synopsis
2. In a meta-analysis of studies reporting exclusion or The role of surveillance imaging after thoracic aneurysm
preservation of the internal iliac artery, Kouvelos et repair is to identify complications of the repair or monitor
al5 found an increased pooled occurrence of but- for progression of residual aortic pathology. CT is gener-
tock claudication in those undergoing unilateral ally the preferred imaging modality for surveillance imag-
(27%) or bilateral (36%) internal iliac artery exclu- ing after TEVAR7,15; MRI, although generally more limited
sion. In a separate meta-analysis, Bosanquet et al6 by metallic artifact, is a reasonable alternative. Open
found similar rates of buttock claudication, as well repair of the thoracic aorta is durable.2,5,10-14 In patients
as a 10% occurrence of erectile dysfunction in men. undergoing TEVAR, there is a higher incidence of com-
Other ischemic events, such as spinal, bowel, and plications and reintervention compared with patients
gluteal ischemia, were rare, occurring at a rate of undergoing open repair2,4,5,10-12; TEVAR complications
<1%.6 Another consideration in treating aorto-iliac can include endoleak (see Section 2.6, “Classification of
disease is the risk of late intervention from growth Endoleaks”), retrograde type A aortic dissection, stent-
of ectatic or aneurysmal iliac arteries. In a retrospec- graft migration, stent-graft fracture or collapse, and an
tive analysis of prospectively collected data, Gibello increase in aortic size.6,7 Complications of open repair
et al4 found that in patients with AAA undergoing that can be detected by surveillance imaging include
EVAR, after a mean follow-up of 6.2 years, those graft infection and anastomotic pseudoaneurysm.10,16
with common iliac arteries of ≥18 mm in diameter Additionally, after both open repair and TEVAR, patients
had a significantly higher rate of type Ib endoleaks may develop progressive aneurysmal dilation of adjacent
(7.2% versus 3.2%; P=0.01) and late reinterventions or remote aortic segments.
(19% versus 11.8%; P=0.01), leading to higher
odds of composite EVAR failure (OR, 1.8; 95% CI,
1.2–2.7) and need for reintervention (OR, 1.9; 95% Recommendation-Specific Supportive Text
CI, 1.15–3.3). Hassen-Khodja et al10 and Sala et al9 1. Use of TEVAR is associated with reintervention
found that, after open repair of AAA, common iliac rates ranging from 7% to 23%.1,2,4,5 In the Gore TAG
arteries of ≥18 mm in diameter tended to dilate over study,17 there was an 11% incidence of endoleak18
time, warranting consideration of bifurcated grafting at 30 days, 6% at 1 year, and 9% at 2-year follow-
rather than aorto-aortic tube grafting.9,10 up after TEVAR.2,17 A 6-month follow-up study may
be useful in detecting a delayed retrograde type A
6.5.6. Surveillance After Aneurysm Repair aortic dissection.
6.5.6.1. Surveillance After TAA Repair 2. MRI has some advantages over CT, including the
Recommendations for Surveillance After TAA Repair
avoidance of ionizing radiation and iodinated intra-
Referenced studies that support the recommendations are venous contrast administration.7,8 However, MRI is
summarized in the Online Data Supplement. limited by its higher cost, longer acquisition times,
COR LOE Recommendations lower resolution, and limited visualization of metal-
1. In patients treated with TEVAR, surveillance lic stent graft components and adjacent structures.
1 B-NR
imaging with CT is recommended after 1 MRI has a potential growing role, particularly in
month and 12 months and, if stable, annually
patients who are middle aged or younger, in whom
thereafter.1-5
the consequences of lifelong surveillance in terms
2. In patients treated with TEVAR, longitudinal
surveillance with MRI is a reasonable alterna-
of contrast-induced nephropathy and cumulative
2a B-NR tive to CT for reduction of long-term radiation radiation dose should be considered.9
exposure or avoidance of an iodinated con- 3. Open repair for any segment of the thoracic
trast allergy.6-9
aorta has proven to be durable in extended

follow-up.10,11,13,14,19 Treatment failure after open Table 21. Abnormal Findings on Duplex Imaging After EVAR
CLINICAL STATEMENTS
repair of either the proximal or distal thoracic aorta That Should Prompt Additional Imaging
AND GUIDELINES
requiring reintervention ranges from 1% to 7% Aneurysm sac enlargement

in long-term (10-year) follow-up.10-12 In patients Any endoleak
without a genetic syndrome or residual aortopathy
Stent graft fracture
shown on a postoperative imaging, surveillance
Stent graft migration
can be done at longer intervals.
4. The appropriate frequency surveillance imaging Stent graft separation
in the presence of abnormal findings has neither EVAR indicates endovascular abdominal aortic aneurysm repair.
been studied nor validated but, in such cases,
annual surveillance imaging is typical. Patients radiation, and requires the use of iodinated contrast that
requiring reintervention have a higher incidence of is potentially nephrotoxic.12,13 Duplex ultrasound, with or
HTAD.10,16 without contrast enhancement, has been shown to be
specific for the detection of endoleaks after EVAR9,14
6.5.6.2. Surveillance After AAA Repair and complex EVAR15; however, ultrasound is limited in
Recommendations for Surveillance After AAA Repair
its ability to detect stent migration, fracture, or noncon-
Referenced studies that support the recommendations are tiguous aneurysms. MRI has high diagnostic accuracy
summarized in the Online Data Supplement. for endoleaks16 but must be accompanied by a plain
COR LOE Recommendations abdominal radiograph to assess for endograft stent frac-
1. In patients with AAA treated with EVAR, ture, because MRI cannot accurately visualize the metal-
baseline surveillance imaging with CT lic stent struts.
is recommended at 1 month postopera-
tively 1,2; if there is no evidence of endoleak
The role of routine surveillance after open AAA repair
1 B-NR is to prevent late aneurysm rupture and aneurysm-
or sac enlargement, continued surveil-
lance with duplex ultrasound at 12 months related death by detecting para-anastomotic and new
and then annually thereafter is recom-
mended. 1,3,4
aneurysms. Para-anastomotic aneurysms can occur after
open AAA repair as a result of anastomotic disruption,
2. In patients with AAA treated with EVAR who
are undergoing annual surveillance imaging leading to pseudoaneurysm formation or progression of
2a C-LD
duplex ultrasound, additional cross-sectional aneurysmal disease in the adjacent visceral aorta or iliac
imaging with CT or MRI of the abdomen and arteries.17 Patients with a history of AAA are also at risk
pelvis every 5 years postoperatively is reason-
able.5-8 of developing an aortic aneurysm in a noncontiguous
location.18
3. In patients with AAA treated with EVAR and
abnormal findings (Table 21) on any surveil-
2a C-LD
lance duplex ultrasound, additional cross-sec-
tional imaging with CT or MRI is reasonable.9 Recommendation-Specific Supportive Text
4. In patients with AAA treated with complex 1. The incidence of late aortic rupture after EVAR is
EVAR, a modified surveillance imaging plan
2a C-LD that combines cross-sectional imaging and
>5% through 8 years of follow-up.3 Significant risk
duplex ultrasound of target vessels is reason- factors for rupture include endoleak with associ-
able.10,11 ated aneurysm sac enlargement.19,20 Endoleaks
5. In patients with AAA who have undergone may be present for 10% to 17% of EVAR at 30
open repair, surveillance imaging with CT or days postoperatively.1,2 In patients without early
2a C-LD MRI of the abdominopelvic aorta within 1 year
postoperatively and then every 5 years there-
(30-day) endoleak, the incidence of new endoleak
after is reasonable.5,6 at 6 and 12 months postoperatively is simi-
lar.1 Earlier detection of an endoleak at 6 vs. 12
months is not associated with improved long-term
Synopsis outcomes.1,2
The role of routine surveillance after EVAR is to iden- 2. Stent graft fracture and migration is a long-
tify endoleak, sac growth, endograft migration, or endo- term complication after EVAR that occurs in
graft failure. Although the initial surveillance intervals 3% to 4% of patients by 4 years postopera-
after EVAR were at 1 month, 6 months, and 12 months tively.7,8 Duplex ultrasound has been shown to
postoperatively to be consistent with surveillance imag- be specific for the detection of endoleaks after
ing intervals used in FDA-sponsored device trials, more EVAR9,14,15 but is limited in its ability to detect
recent data suggest that the 6-month interval can be stent migration, fracture, or new noncontiguous
eliminated if no concerning findings are observed on the aneurysms.
1-month imaging (Table 21).1,2 3. Duplex ultrasound is 95% accurate for measuring
CT is the gold standard for follow-up imaging after aortic aneurysm sac diameter and 100% specific
EVAR, but it is expensive, exposes the patient to ionizing for the detection of type I and type III endoleaks

(Figure 11) after EVAR but is insufficient for 7. ACUTE AORTIC SYNDROMES
CLINICAL STATEMENTS
detecting type II endoleaks9 or for characteriz-
7.1. Presentation
AND GUIDELINES
ing anatomy related to stent graft migration or
failure. AAS, although uncommon, are associated with life-
4. Duplex ultrasound has been shown to be a useful threatening complications and a mortality rate as high
modality for surveillance of target branch vessels11 as 1% to 2%/h if the AAS is not rapidly identified and
after FEVAR. However, complex EVAR involv- appropriate therapy is not instituted promptly.1 The diag-
ing stenting of ≥1of the renovisceral vessels is nosis of AAS can be challenging, however, because the
at higher risk of type III endoleak than standard presenting symptoms overlap with other more common
EVAR10 and may benefit from routine cross-sec- emergency department complaints.Although the clas-
tional imaging for surveillance of fenestration sites,
sic textbook description of AAS is of acute “tearing” or
branch junctions, and adequacy of flow in the renal
“ripping” pain, patients more commonly report the abrupt
and mesenteric arteries.21
onset of severe “sharp” or “stabbing” pain in the chest
5. Para-anastomotic aneurysms after open AAA
or back (and sometimes abdomen), maximal at the start,
repair tend to occur late, with estimated incidence
that sometimes radiates.2-5 Depending on the extent of
rates of 1%, 6%, and 27% to 35% at 5, 10, and
15 years postoperatively, respectively.5,6 Late aor- aortic involvement, patients may present with various
tic aneurysms in noncontiguous arterial segments additional signs and symptoms (Table 22). Recording a
from the initial aortic repair have been reported in careful history of the presenting symptoms is essential,
45% at a mean of 7 years postoperatively.18 As a as is obtaining a detailed family history of TAAs, genetic
result, the Society for Vascular Surgery and the aortopathies, aortic dissection, or unexplained sudden
European Society of Cardiology have both recom- death.
mended surveillance imaging every 5 years after BP should be measured in both arms and both lower
open AAA repair.22 No data support the use of 1 extremities, to exclude a BP differential resulting from
cross-sectioning imaging modality over another for an AAS. One should auscultate for the murmurs of aortic
the surveillance of para-anastomotic aneurysms stenosis, perhaps indicating an underlying BAV, and AR,
after open AAA repair.18 which commonly accompanies type A aortic dissection.
Table 22. Signs and Symptoms of AAS
Clinical Signs and Symptoms Cause

Asymmetric blood pressure (>20 mm Hg) between limbs Compromise of branch artery flow
Bowel ischemia or gastrointestinal bleed Malperfusion of the celiac or superior mesenteric artery
Dysphagia Compression of the esophagus
Dyspnea Compression of trachea or bronchus, congestive heart failure from aortic regurgitation, or
cardiac tamponade
Hemoptysis Vascular rupture into lung parenchyma
Hoarseness Compression recurrent laryngeal nerve
Horner’s syndrome Compression of sympathetic chain
Myocardial ischemia or myocardial infarction Coronary artery involvement by dissection or compression by aneurysm
New murmur of aortic regurgitation Incomplete aortic valve closure secondary to leaflet tethering by the dilated aorta or cusp
prolapse because of dissection into the aortic root
Oliguria or hematuria (gross) Malperfusion of 1 or both renal arteries
Paraplegia Spinal malperfusion attributable intercostal artery involvement
Lower extremity ischemia Malperfusion of iliac artery
Shock Cardiac tamponade, hemothorax, frank aortic rupture, acute severe aortic regurgitation, severe
myocardial ischemia
Shortness of breath Pericardial effusion, congestive heart failure from acute severe aortic regurgitation, or
hemothorax
Stroke symptoms Carotid or vertebral artery involved
Superior vena cava syndrome Compression of the superior vena cava
Syncope Carotid artery involvement or cardiac tamponade
AAS indicates acute aortic syndrome.

7.2. AAS: Diagnostic Evaluation (Imaging, Table 24. Aortic Dissection Detection Risk Score (ADD-RS)
CLINICAL STATEMENTS
Items5,14
Laboratory Testing)
AND GUIDELINES
High-Risk
Recommendations for AAS: Diagnostic Evaluation (Imaging, Laboratory
High-Risk High-Risk Pain Examination
Testing)
Conditions Features Features
Marfan syndrome Chest, back, or Pulse deficit or
1. In patients with a suspected AAS, CT is rec- or other connective abdominal pain systolic blood
ommended for initial diagnostic imaging, given tissue disease described as: pressure differential
1 C-LD its wide availability, accuracy, and speed, as Family history of aortic Abrupt onset Focal neurologic
well as the extent of anatomic detail it pro- disease Severe in intensity deficit (with pain)
vides.1-5 Known aortic valve Ripping or tearing Murmur of aortic
disease in quality regurgitation (new,
2. In patients with a suspected AAS, TEE and Recent aortic with pain)
2a C-LD MRI are reasonable alternatives for initial manipulation Hypotension or shock
diagnostic imaging.1-6 Known thoracic aortic state
aneurysm
For each risk category, 1 point is assigned if 1 risk factors are present.
Synopsis Consequently, the total ADD-RS will range from 0 to 3. An ADD-RS of 0 points
is low risk; 1 point is moderate risk; and 2 to 3 points is high risk. Adapted with
A plain chest x-ray is neither sufficiently sensitive nor permission from Hiratzka et al.5 Copyright 2010, American Heart Association,
specific for AAS to be used to be diagnostic, but certain Inc., and American College of Cardiology Foundation.
radiographic findings (Table 23) may raise suspicion of
aortic dissection or suggest an alternate diagnosis for
the patient’s symptoms, in particular when there is previ- Table 25. Aorta Simplified Score (AORTAs)11 Pretest
ous radiography that shows the changes to be new in the Probability Assessment Score
interval.1,2,7 Fortunately, CT, TEE, and MRI are all highly Clinical Item Points
accurate for the diagnosis of AAS.3 Aortography is rarely Hypotension/shock 2
used given its invasive nature and significantly lower
Aneurysm 1
sensitivity than the other imaging modalities.8 Acute
Pulse deficit 1
aortic dissection risk scoring systems (eg, aortic dissec-
tion detection risk score [AAD-RS] or aorta simplified Neurologic deficit 1
score [AORTAs]) can aid in the diagnostic evaluation of Severe pain 1

patients presenting with AAS (Table 24 and Table 25)5,9-12 Sudden-onset pain 1
but have not been uniformly adopted.4 No biomarkers The patient is given the number of points corresponding to each clinical item
are considered diagnostic, although in patients with a that is positive in the patient’s presentation. The points are summed, and a total
low previous probability of AAS a nonelevated D-dimer score of 0 to 1 point is low-probability of aortic dissection, where a total of
2 points is high probability. Reprinted with permission from Morello et al.11
(<500 ng/mL) makes the diagnosis unlikely. Conse-
quently, integrating a low aortic dissection risk score and
a low D-dimer may be a useful strategy to exclude the
in the emergency department and is quick to
diagnosis of AAS.13
perform. Not only does it diagnose the underly-
ing AAS, it also shows the full extent of the dis-
Recommendation-Specific Supportive Text section and, in some cases, the entry tear site.
CT can detect the presence and mechanism of
1. Although the sensitivity and specify of CT, MRI,
aortic branch vessel involvement as well as vessel
and TEE are all high,3 CT has become the pre-
patency, signs of malperfusion, pericardial effu-
ferred modality for evaluating most patients with
sion and hemopericardium, periaortic or medi-
suspected AAS. CT is widely available at all hours
astinal hematoma, and pleural effusion. Use of
electrocardiographic-synchronized CT techniques
Table 23. Plain Chest X-Ray Suggestive of Aortic Dissection2
should be considered when there is a need to
Signs of Aortic Dissection on Chest X-Ray Findings accurately depict mediastinal structures (eg, prox-
Mediastinal widening imal aorta, coronary ostia). When IMH is present,
Disruption of the normally distinct contour of the aortic knob the extent and thickness of the hematoma can
be documented and, when PAUs are present, the
”Calcium sign,” which appears as a separation of the intimal calcification
from the aortic wall of >5 mm presence of and size of pseudoaneurysms can be
Double density appearance within the aorta
easily defined.
2. In general, the choice of the initial imaging modal-
Tracheal deviation to the right
ity should be based on the patient’s history and
Deviation of the nasogastric tube to the right
clinical presentation, the specific clinical questions
Reprinted with permission from Strayer et al.2 to be answered, and the institutional availability,

experience, and expertise with each of the diag- Synopsis
CLINICAL STATEMENTS
nostic imaging techniques.6 In certain clinical cir-
Patients presenting with AAS need to be treated promptly to
AND GUIDELINES
cumstances, for example, patients with a history
prevent acute and chronic complications. In all patients with
of an iodinated contrast reaction or patients who
AAS, immediate medical therapy is indicated while consider-
are too unstable to travel to the radiology suite, CT
ing urgent surgical (in patients with type A aortic dissection),
may not be preferred. Echocardiography (TEE/
endovascular intervention (in patients with type B aortic dis-
TTE) is an alternative. TTE is noninvasive, can be
section), or both; medical therapy includes aggressive heart
performed at the bedside, and may be helpful in
rate and BP management as well as pain control. Studies
eliciting the diagnosis of AAS and quickly identify-
have shown that, beyond surgical and endovascular treat-
ing complications of AAS, such as AR or pericardial
ment, medical therapy has an important role in decreasing
effusion and tamponade. TEE is preferred to TTE,
long-term aorta-related adverse events.1,4,9-11 Beta blockers
however, because of its higher sensitivity and bet-
and intravenous vasodilators are the medications most com-
ter anatomic resolution; TEE can be performed at
monly studied for the initial treatment of patients with AAS,
the bedside in the emergency department or, alter-
with the goal of decreasing aortic wall stress.2,8 A recent
natively, once the patient is in the operating room.
large study showed that angiotensin-converting enzyme
MRI is most commonly the third-choice modality,
inhibitors (ACEIs) and ARBs are beneficial in the long-term
given that it is not readily available, requires skilled
management of hypertension in patients with aortic dissec-
interpretation, and has longer acquisition times, as
tion.5 Statins are used routinely in patients after aortic dis-
well as the fact it is challenging to provide clini-
section, although the evidence is not very robust.12
cal care to potentially unstable patients while in
an MRI scanner. Consequently, MRI is most often
used as a follow-up imaging modality in patients in Recommendation-Specific Supportive Text
which there is diagnostic uncertainty. Nevertheless,
MRI may be the study of choice in the acute set- 1. There are no randomized studies that have evalu-
ting for a stable patient with a contraindication to ated different medical treatments in the treatment
iodinated contrast. of AAS, although extensive clinical experience has
established the current standard of anti-impulse
therapy. This is usually accomplished with a com-
7.3. Medical Management of AAS bination of intravenous beta blockers (eg, esmo-
lol, metoprolol, and labetalol) and vasodilators (eg,

7.3.1. Acute Medical Management of AAS nicardipine, clevidipine, and sodium nitroprusside)
Recommendations for Acute Medical Management of AAS with the goal of reducing both heart rate and BP to
decrease aortic wall stress.2-5,7,8,11
2. Small, single-center studies have highlighted the
importance of reducing heart rate to 60 to 80 bpm
1. In patients presenting to the hospital with and SBP to <120 mm Hg. Experts believe that the
AAS, prompt treatment with anti-impulse
therapy with invasive monitoring of BP with an lowest BP that does not compromise end-organ
1 B-NR
arterial line in an ICU setting is recommended function should be targeted.3,11
as initial treatment to decrease aortic wall 3. Intravenous beta blockers have been the mainstay
stress.1-5
of acute medical treatment, and studies report-
2. Patients with AAS should be treated to an
SBP <120 mm Hg or to lowest BP that main-
ing benefits over the long term and emphasizing
1 C-LD the importance of continuing this therapy at the
tains adequate end-organ perfusion, as well
as to a target heart rate of 60 to 80 bpm.3,6 time of hospital discharge to improve clinical out-
3. In patients with AAS, initial management comes.1-3,5,7,9 Caution should be used in patients
1 B-NR should include intravenous beta blockers, with contraindications to beta blockers (eg, acute
except in patients with contraindications.2,5,7
AR, heart block, or bradycardia). In patients who are
In those with contraindications or intolerance
to beta blockers, initial management with
intolerant to beta blockers, intravenous non-dihy-
2a B-NR an intravenous non-dihydropyridine calcium dropyridine calcium channel blockers (ie, verapamil
channel blocker is reasonable for heart rate or diltiazem) are typically used for initial treatment.2
control.1,2,5
4. Intravenous vasodilators are useful adjunctive
4. In patients with AAS, initial management therapy for intravenous beta blockers but should
should include intravenous vasodilators if the
1 C-LD
BP is not well controlled after initiation of
be avoided as initial treatment (before starting beta
intravenous beta-blocker therapy.8 blockers or calcium channel blockers), given the
5. Patients with AAS should be treated with pain potential for compensatory tachycardia.8,9
1 C-EO control, as needed, to help with hemodynamic 5. Pain related to AAS can trigger a rise in heart rate
management. and BP, so treating the pain symptoms can help to

control the patient’s BP and heart rate. Intravenous shown to improve long-term outcomes in patients
CLINICAL STATEMENTS
opiates are particularly efficacious in this situation. with AAS treated with both surgical and endovascu-
AND GUIDELINES
Intravenous nonsteroidal anti-inflammatory drugs, lar treatments.1-4 Although calcium channel block-
such as ketorolac, may not be suitable because of ers showed some benefit in patients with type B
the risk of inducing hypertension as well as adverse aortic dissection, further studies in mouse models
renal effects. of Marfan syndrome as well as case control studies
in Marfan syndrome and other inherited aortopa-
7.3.2. Subsequent Medical Management of AAS thy patients in the GenTAC (Genetically Triggered
Recommendation for Subsequent Medical Management of AAS Thoracic Aortic Aneurysms and Cardiovascular
Referenced studies that support the recommendation are summarized Conditions) registry showed deleterious effects of
in the Online Data Supplement.
long-term calcium channel blocker use and, con-
COR LOE Recommendation sequently, it may be best to avoid these agents in
1. In patients with AAS, it is recommended to patients with Marfan syndrome unless necessary to
treat with long-term beta blockers (unless
contraindicated) to control heart rate and BP
achieve BP control.8
1 B-NR to reduce late aortic-related adverse events.1-7
Additional antihypertensive agents (particu-
larly ARBs and ACEIs) should be added, as
necessary, to adequately control BP. 7.4. Surgical and Endovascular Management of
Acute Aortic Dissection
Synopsis The primary goals of open surgical or endovascu-
lar stent-graft repair for acute aortic dissection are to
Patients with AAS with surgical or endovascular treat-
prevent (or treat) aortic rupture, prevent retrograde
ment need continued and long-term medical manage-
extension of the dissection into the aortic root, prevent
ment. Controlling hypertension has consistently been
antegrade propagation of the dissection into distal yet
shown to decrease aorta-related adverse events. Recent
undissected segments, and alleviate malperfusion syn-
studies have shown long-term benefit with specific BP
dromes. Acute aortic dissection management strate-
agents such as beta blockers, ACEIs, and ARBs.
gies are therefore “complication specific,” guided by the
patient’s signs and symptoms, the presence or absence
Recommendation Supporting Text of complications, and the specific features and con-
1. Long-term oral antihypertensive regimens that straints of the patient’s aortic and branch vessel anat-
included beta blockers, ACEIs, and ARBs have omy (Figure 21).
Figure 21. Acute Aortic Dissection: Malperfusion Treatment Options.

AoD indicates aortic dissection; and TEVAR, thoracic endovascular aortic repair.

7.4.1. Acute Type A Aortic Dissection Recommendation-Specific Supportive Text
CLINICAL STATEMENTS
7.4.1.1. Initial Surgical Considerations in Acute Type A 1. The potential sequelae of acute type A aortic dis-
AND GUIDELINES
Aortic Dissection section, including myocardial infarction, acute AR,
Recommendations for Initial Surgical Considerations in Acute Type A cardiac tamponade, aortic rupture, and end-organ
Aortic Dissection malperfusion, are associated with high rates of
Referenced studies that support the recommendations are morbidity and mortality. Given the acuity, unpre-
dictability, and finality of such events, immediate
evaluation for surgical intervention is warranted to
1. In patients presenting with suspected or con- reverse any ongoing physiologic compromise and
firmed acute type A aortic dissection, emer-
gency surgical consultation and evaluation
mitigate the risk of fatal events. The mortality rate
1 B-NR
and immediate surgical intervention is recom- of unoperated acute type A aortic dissection is
mended because of the high risk of associated 1%/h,15 and the time intervals between symptom
life-threatening complications.1,2
onset, diagnosis, and surgery have a significant
2. In patients presenting with acute type A
effect, with the highest mortality rate occurring
aortic dissection, who are stable enough
2a B-NR for transfer, transfer from a low- to a high- in those undergoing surgery 8 to 12 hours after
volume aortic center is reasonable to improve diagnosis.16 Patients presenting with clinical indi-
survival.3,4
cators of severe physiologic compromise (shock,
3. In patients presenting with nonhemorrhagic neurologic deficits, malperfusion, myocardial isch-
stroke complicating acute type A aortic dis-
2a B-NR section, surgical intervention is reasonable
emia) mandate the most immediate consideration
over medical therapy to reduce mortality and for repair as the only potential option for survival.
improve neurologic outcomes.5,6 2. Patients with acute type A aortic dissection who
present with hemodynamic stability have an unpre-
dictable course because of the inability to predict
Synopsis eventual rupture. Although some studies have sug-
Acute type A aortic dissection is a life-threatening condi- gested that night-time surgery is associated with a
tion because of potential sequelae, including rupture that higher mortality rate,17,18 other studies have shown
causes cardiac tamponade, acute severe AR that causes no diurnal difference in outcomes,19,20 and all stud-
heart failure or shock, compromised coronary artery ostia ies have shown no difference with weekend sur-
causing myocardial ischemia, or malperfusion causing gery. Surgeon and center experience and resource
end-organ ischemia or infarction, all of which can all be availability should be considered to ensure optimal
fatal. Suspected or diagnosed acute type A aortic dis- outcomes. Despite an inherent delay in the start
section warrants urgent surgical evaluation, because time of surgery, transfer from low- to high-volume
the mortality rate of medical management alone is 2 to hospitals (one that performs ≥7 aortic root, ascend-
3 times that of surgical intervention.1 Data from IRAD ing aorta, or transverse arch aortic dissection
showed that from 1995 to 2013, the surgical mortal- repairs per year),3 as part of regionalization of care,
ity rate decreased from 25% to 18%, while the medi- can result in significantly improved outcomes.3
cal mortality rate remained unchanged at 57%. Surgical 3. In patients with cerebral malperfusion, survival is
intervention mitigates the immediate risk of aortic rup- superior with surgery; in patients with acute type A
ture/tamponade, corrects AR and myocardial ischemia, aortic dissection and an acute stroke, the mortal-
and reestablishes flow to malperfused vessels. ity rates of surgical versus medical management
Nevertheless, the benefits of surgery must be weighed are 25% to 27% versus 76%, respectively.5,21 Even
against the risks of the surgery itself (ie, a demanding, more strikingly, Estrera et al showed that patients
complex operation in patients who often are physiologi- with acute type A aortic dissection who had pre-
cally compromised). Universally recognized risk factors sented with stroke had an operative mortality rate
that increase the surgical mortality rate include shock of only 7% and showed no worsening of neuro-
and tamponade, neurologic or visceral malperfusion, and logic status postoperatively.6 Although their study
preoperative myocardial ischemia.7-9 Although age is a and others,6,22 have emphasized the timeliness of
risk factor, elderly patients still benefit from surgery, with the aortic repair in stroke patients, with a cutoff of
superior immediate and midterm outcomes compared ∼5 to 10 hours (after which neurologic outcomes
with medical therapy.10,11 Short- and midterm outcomes declined), Fischbein et al23 found no association
can be equivalent to younger populations,12,13 with circu- between postoperative neurologic improvement
latory collapse being the primary predictor of long-term and time from onset of neurologic symptoms to
survival.14 In patients with significant contraindications to surgery. IRAD data revealed that cerebrovascu-
surgery, including frailty, clinical judgment may determine lar accident and coma resolved in 84% and 79%
that the risk-benefit ratio favors medical management. of patients, respectively, despite mean times to

surgery of 12.3 and 13.8 hours, respectively.24 It Recommendation-Specific Supportive Text

CLINICAL STATEMENTS
should be noted, however, that in 1 recent report of

1. In the presence of malperfusion, operative mortal-
AND GUIDELINES
11 patients with acute type A aortic dissection and

ity rate correlates with the number of malperfused
complete occlusion of an internal carotid artery, all
organs. Central aortic repair as the primary strat-
died from cerebral edema and herniation, regard-
egy to restore perfusion has reasonable results
less of management25; consequently, this particular
when renal malperfusion, extremity malperfusion,
subset of patients may not benefit from surgical
uncomplicated mesenteric malperfusion, or all of
intervention.
them is present.9 This strategy rapidly mitigates the
risk of aortic rupture and corrects any associated
7.4.1.2. Management of Malperfusion coronary malperfusion, AR, and the sequelae of
Recommendations for Management of Malperfusion tamponade. After central aortic repair, any residual
Referenced studies that support the recommendations are malperfusion should be assessed with secondary
interventions, as needed.
COR LOE Recommendations 2. Mesenteric malperfusion is one of the worst
1. In patients with acute type A aortic dissection complications of acute type A aortic dissection,
presenting with renal, mesenteric, or lower
with an associated mortality rate of 63.2%.1
1 B-NR extremity malperfusion, it is recommended to
proceed to immediate operative repair of the Consequently, such patients are often man-
ascending aorta.1,2 aged with medical therapy alone; yet, in IRAD,
2. In patients with acute type A aortic dissection the nearly one-third of patients with mesenteric
presenting with clinically significant mesen- ischemia who were treated without intervention
teric (celiac, SMA) malperfusion, either imme-
diate operative repair of the ascending aorta
had an in-hospital mortality rate of 95%.10 For
2a C-LD
or immediate mesenteric revascularization via patients with acute type A aortic dissection who
endovascular or open surgical intervention by present with clinical evidence of mesenteric isch-
those with this expertise before ascending
aortic repair is reasonable.3-6
emia, some centers3,4 have advocated early direct
reperfusion strategies (whether via endovascular
or open abdominal surgery11), before central aortic
Synopsis repair; other centers continue to advocate for the
traditional strategy of central aortic repair first.1,2
Imaging evidence of malperfusion is present in as
Currently, data are limited to help define the best

many as 25% of patients with acute type A aortic
strategy. In IRAD, a surgical and hybrid strategy
dissection but should be distinguished from clini-
appears to have superior outcome to medical or
cal evidence of end-organ ischemia, which is often
endovascular therapy alone. An institution series
referred to as malperfusion syndrome (Table 26).
of endovascular therapy first showed a low aortic
Malperfusion syndrome is associated with a mortal-
repair operative mortality rate of 2.1%; however,
ity rate of 30.5%, compared with a mortality rate of
only 58% of the cohort underwent open repair,
only 6.2% in those without malperfusion syndrome.2
with 24% dying from organ failure and 13% from
Mortality rate correlates with the number of branch
aortic rupture. Moreover, an endovascular therapy
artery vessels involved1 as well as the number of mal-
first approach requires expertise in fenestration,
perfused organs.7 The combination of pulse deficits
to treat dynamic obstruction, and branch stenting,
(a marker of malperfusion) and hypotension should
to treat static malperfusion.5
prompt timely interventions to reestablish vital organ
perfusion, because early reperfusion predicts sur-
vival.8 The traditional approach to reestablish branch Table 26. Clinical Evidence of Malperfusion (“Malperfusion
vessel perfusion has been via central aortic repair Syndrome”)
(ie, at the proximal aortic tear site). However, cardiac End Organ Clinical Findings
and visceral malperfusion portend extremely poor
Cardiac Electrocardiographic changes of ischemia or infarction,
outcomes given the high mortality rate associated troponin elevation, myocardial dysfunction
with irreversible organ damage. More recent series
Cerebral Stroke and neurologic deficits, coma and altered mental
showed potential to improve outcomes by establish- status
ing end-organ perfusion using endovascular means, Spinal Paraplegia
before open central aortic repair (with the timing of
Mesenteric Abdominal pain, bowel ischemia, lactic acidosis, elevation
subsequent open repair decided on a case-by-case of liver function test results
basis).5,8 These procedures may be performed in a Renal Acute kidney injury, oliguria
hybrid operating room if the requisite resources and
Extremity Loss of pulses in 1 extremity, sensory or motor dysfunction
personnel are available.

7.4.1.3. Surgical Repair Strategies in Acute Type A Aortic A nonresected primary tear is a risk factor for reop-
CLINICAL STATEMENTS
Dissection eration.32 A more extensive replacement that involves
AND GUIDELINES
Recommendations for Surgical Repair Strategies in Acute Type A the aortic root, arch, or both adds operative complexity,
Aortic Dissection ischemic time, and potentially circulatory arrest time but
may reduce the risk of future aortic dilation, aortic insuf-
ficiency, or repeat dissection. An individualized approach
to aortic root management is based on pathology and
Aortic Repair Strategies general condition. Younger patients are more likely to
1. In patients with acute type A aortic dissection have proximal extension or root involvement and may
and a partially dissected aortic root but no
1 B-NR significant aortic valve leaflet pathology, aortic
have greater potential for late complications, given their
valve resuspension is recommended over longer life expectancy. VSRR has been described with
valve replacement.1-5 excellent outcomes, but long-term reoperative risk is a
2. In patients with acute type A aortic dissection concern.33
who have extensive destruction of the aortic Similarly, untreated aortic arch or descending thoracic
root, a root aneurysm, or a known genetic aor-
1 B-NR
tic disorder, aortic root replacement is recom- aortic tissue may be at risk of aneurysmal enlargement
mended with a mechanical or biological valved and the need for reintervention, particularly with acute
conduit.6-9 type A aortic dissection that extends into the descending
In selected patients who are stable, valve- thoracic aorta. An open distal anastomosis allows direct
sparing root repair may be reasonable, when
2b C-LD arch inspection for intimal tears and resection of the
performed by experienced surgeons in a Mul-
tidisciplinary Aortic Team.10,11 lesser curve of the arch (ie, hemiarch technique) with-
3. In patients with acute type A aortic dissec-
out increased operative death.12,13,34 In-hospital death is
tion undergoing aortic repair, an open distal lower with hemiarch repair than with total arch replace-
1 B-NR anastomosis is recommended to improve ment. Antegrade stenting of the proximal descending
survival and increase false-lumen thrombosis
rates.12-15
thoracic aorta may promote false-lumen thrombosis and
positive remodeling,35-37 but long-term aortic-related data
tion without an intimal tear in the arch or a are scarce.
1 B-NR significant arch aneurysm, hemiarch repair Involvement of the aortic arch by the aortic dissection
is recommended over more extensive arch can influence both interventional strategies and clinical
replacement.16-18
outcomes. Various interventional approaches, such as

tion and a dissection flap extending through
extended open arch replacement (with or without a fro-
the arch into the descending thoracic aorta, zen elephant trunk),44 hybrid techniques, or endovascular
2b C-LD
an extended aortic repair with antegrade stenting have been described.38-40 Aortic arch exclusion
stenting of the proximal descending thoracic
aorta may be considered to treat malperfu-
with emerging endovascular stents graft devices is a
sion and reduce late distal aortic complica- field in evolution.
tions.19,20
Perfusion and Cannulation Strategies
6. In patients with acute type A aortic dissection
undergoing surgical repair, axillary cannula- 1. Most single-center retrospective studies and
2a B-NR tion, when feasible, is reasonable over femoral
cannulation to reduce the risk of stroke or an IRAD study found no difference in peri-
retrograde malperfusion.21,22 operative mortality or survival when compar-
7. In patients with acute type A aortic dis- ing root replacement with a more limited root
section undergoing surgical repair who repair or supracommissural replacement.2,5,7,41,42
2a B-NR require circulatory arrest, cerebral perfusion
However, a standardized and structured algo-
is reasonable to improve neurologic out-
comes.23-25 rithmic approach showed a mortality rate of only
8. In patients with acute type A aortic dis-
8.1% with aortic valve resuspension as the pre-
section undergoing surgical repair, direct ferred approach, whenever feasible, compared
2a B-NR
aortic26,27 or innominate artery28 cannula- with 23.1% with root replacement.43 Studies on
tion with imaging guidance is reasonable
as an alternative to femoral or axillary
freedom from reoperation are mixed,1,7,41,44-46 but
cannulation.29-31 2 meta-analyses have shown excellent long-
term durability of aortic valve resuspension, with
reoperation rates 1.4% to 2.1% per patient-year
Synopsis and low thromboembolism and bleeding rates
To reduce the risk of late aortic complications, surgical (1.4%/patient-year).3,4
resection should include the tear site, any aneurysmal 2. An aneurysmal root at the time of acute type A
aorta, and the proximal-most extent of the dissection. aortic dissection repair is at long-term risk of

progressive root dilation, secondary aortic insuffi- In a series that included 19 patients with DeBakey
CLINICAL STATEMENTS
ciency, and the need for reoperation. Specifically, type I acute type A aortic dissection and clinical
AND GUIDELINES
an aortic root diameter of >4.5 cm has been shown malperfusion, antegrade stenting was associated
to be a risk factor for late reintervention.6 A valved with resolution of malperfusion in 16 patients
conduit is one option for root replacement but, if (84.2%).19 In patients requiring total arch replace-
the aortic valve leaflet quality is good, the aortic ment, a frozen elephant trunk has higher adverse
insufficiency is primarily attributable to sinus dila- events in acute type A aortic dissection than in
tion, and the surgeon is experienced in VSRR, a elective repairs. The stent length should be <15
VSRR may be reasonable for younger patients. cm and, to avoid SCI, coverage should not extend
3. In the development of the IRAD risk score, right to T8.53
hemiarch replacement was an independent predic- 6. An STS database study50 and 2 meta-analy-
tor for a favorable surgical outcome.15 NORCAAD ses21,22 have found an increased risk of stroke
(Nordic Consortium for Acute Type A Aortic and short-term mortality with femoral compared
Dissection) found that the open-distal technique with axillary cannulation. However, femoral can-
was associated with better short- and midterm sur- nulation is more expedient and is considered the
vival than the clamp-on technique, although it was primary arterial site in patients with hemodynamic
also associated with greater rates of cerebrovas- instability mandating immediate cannulation, or
cular complications.12 Lawton et al14 found superior with anatomic features precluding axillary can-
survival when all 3 components—no cross-clamp nulation. If initial femoral cannulation is required
use, deep hypothermic circulatory arrest, and only for these reasons, it is recommended to centrally
antegrade perfusion after aortic perfusion—were canulate after the distal anastomosis has been
used, compared with the absence of any of these completed, to maximize reestablishment of true
components. Open distal anastomosis is also asso- lumen flow.
ciated with higher rates of complete false-lumen 7. Some form of cerebral perfusion, whether ante-
thrombosis.13 grade or retrograde, has been shown to improve
4. Single-institution study findings that total arch neurologic outcomes, when compared with deep
replacement (TAR) is safe and promotes aor- hypothermic circulatory arrest alone.23 Antegrade
tic remodeling47,48 have not been resulted in cerebral perfusion is associated with both lower
larger studies. GERAADA (German Registry for long-term mortality rates and neurologic dys-
Acute Aortic Dissection Type A) found a trend function rates. Unilateral and bilateral antegrade
toward lower mortality rates with hemiarch ver- cerebral perfusion appear to have similar out-
sus TAR (18.7% versus 25.7%; P=0.07); higher comes, except in cases of prolonged circula-
rates of excessive bleeding and rethoracotomy tory arrest (>30–50 minutes), in which case
in the total arch group; and, in patients without bilateral antegrade cerebral perfusion may be
preoperative neurologic deficits, lower mortal- advantageous.24,54-56
ity rates for hemiarch than TAR (14.1% versus 8. Several series for surgery for acute type A aortic
24%, respectively; P=0.02).49 A n STS database dissection have reported direct aortic cannulation
study of 12 years of acute type A aortic dissec- using a TEE-guided Seldinger technique. When
tion repairs showed significantly lower operative performed correctly, this technique has the benefit
death with hemiarch than with TAR (16% ver- of rapid establishment of cardiopulmonary bypass
sus 27%; P<0.001).50 Two meta-analyses have with true lumen flow. However, when the patient
found significantly lower mortality rates with is stable, its safety relative to axillary cannulation
partial compared with TAR.16,18 Across 3 meta- is controversial,57 because stroke rates with this
analyses, the long-term freedom from aortic technique are as high as 20% in some series.29
reoperation does not appear to be necessarily Rosinski et al found that patients undergoing
superior with TAR.16-18 direct aortic cannulation were more hemodynami-
5. Comparative data on use of antegrade stenting cally unstable and had more extended repairs;
of the descending thoracic aorta in the setting of however, even in multivariable logistic regression,
surgical acute type A aortic dissection repair are it was associated with a higher risk of stroke (OR,
limited. In several noncomparative meta-analyses, 2.3; 95% CI, 1.05–5.1) Further, cerebral perfu-
the mortality rate was ∼8% to 12%, the stroke sion by some means other than axillary perfusion
rate was 5% to 7%, and the SCI rate was 2% to will be required for longer circulatory arrest cases.
3.5%.36,51,52 False-lumen thrombosis rates appear Innominate artery cannulation is another option
favorable,35 but the reintervention rate was not that provides access for antegrade cerebral perfu-
zero, and the long-term benefit for aortic reopera- sion and appears to be safe in acute type A aortic
tion or aortic-related mortality remained undefined. dissection.58-60

7.4.2. Management of Acute Type B Aortic Table 27. Consensus Features of Complicated Acute Type B
CLINICAL STATEMENTS
Dissection Aortic Dissection
AND GUIDELINES
Recommendations for the Management of Acute Type B Aortic Feature Comment
Dissection
Aortic rupture1 This can be either free or contained (in-
cluding hemothorax, increasing periaortic
hematoma, or both; or mediastinal hema-
COR LOE Recommendations toma) and should be addressed promptly.
1. In all patients with uncomplicated acute Branch artery occlusion and Complete or partial occlusion of a major
type B aortic dissection, medical therapy is malperfusion2 branch, with or without clinical evidence of
1 B-NR
recommended as the initial management ischemia; this includes visceral, renal, and
strategy.1-3 peripheral arterial branches.
2. In patients with acute type B aortic dissection Extension of dissection3 Extension of the dissection flap either
1 C-LD and rupture or other complications (Table 27), distally or proximally (ie, retrograde type A
intervention is recommended.4-6 dissection)
In patients with rupture, in the presence of Aortic enlargement Progressive enlargement of the true, false,
suitable anatomy, endovascular stent grafting, or both lumens while in the acute phase
1 C-EO
rather than open surgical repair, is recom- may require prompt intervention.
mended.
Intractable pain15
In patients with other complications, in the
Uncontrolled hypertension15
presence of suitable anatomy, the use of
2a C-LD
endovascular approaches, rather than open
surgical repair, is reasonable.4-6,7
3. In patients with uncomplicated acute type B Table 28. High-Risk Features in Uncomplicated Acute
aortic dissection who have high-risk anatomic Type B Aortic Dissection9
2b B-R
features (Table 28), endovascular manage-
ment may be considered.8,9 High-Risk Imaging Findings
Maximal aortic diameter >40 mm
False-lumen diameter >20–22 mm
Synopsis
Entry tear >10 mm
Although acute complicated type B aortic dissection his- Entry tear on lesser curvature
torically has been treated with open repair, endovascular
Increase in total aortic diameter of >5 mm between serial imaging studies
therapy has largely supplanted open repair given lower
Bloody pleural effusion

morbidity and mortality rates. Additionally, optimal medi-
cal management was associated with a 30-day mortality Imaging-only evidence of malperfusion
rate of 10% and midterm mortality rate of approximately High-Risk Clinical Findings
30%. The introduction of endovascular techniques has Refractory hypertension despite >3 different classes of antihypertensive
resulted in significantly lower morbidity and mortality medications at maximal recommended or tolerated doses
rates when compared with optimal medical management, Refractory pain persisting >12 h despite maximal recommended or toler-
reported in small randomized trials including ADSORB ated doses
(Acute Dissection: Stent graft OR Best medical therapy)8 Need for readmission
and INSTEAD (Investigation of Stent Grafts in Patients
with Type B Aortic Dissection)10; to date, there has not
intervention, risk factors for early death include
been a large RCT comparing open versus endovascu-
shock, evidence of malperfusion, and age1-3
lar repair for either complicated or uncomplicated type B
and can be grouped together with uncontrollable
aortic dissection.
hypertension, pain, and continued growth or exten-
sion of the dissection as a complicated type B aor-
Recommendation-Specific Supportive Text tic dissection.
1. Those patients with type B aortic dissection gen- 2. Patients presenting with complicated acute type B
erally have better survival than those with type A aortic dissection (Table 27), or developing such fea-
aortic dissection. In the acute uncomplicated set- tures after initial presentation, have an increased risk
ting, medical management is the first mode of of morbidity and death, and urgent or emergency
therapy for type B aortic dissection. A review of the intervention may be required. For rupture, rapid
IRAD database showed overall in-hospital mortal- coverage of the affected region of the descending
ity rate of 13%, with those requiring open repair aorta may be lifesaving and does not preclude sub-
having higher mortality rates compared with those sequent further endovascular or open repair. This
managed with optimal medical management and is an important consideration in those patients with
percutaneous intervention (32.1% versus 9.6% genetically triggered aortic diseases (eg, Marfan
versus 6.5%, respectively; P<0.0001).1 Without syndrome, Loeys-Dietz syndrome). Cambria et al11

reviewed the outcomes for AAS managed with Recommendations for Management of IMH (Continued)
CLINICAL STATEMENTS
TEVAR compared with historic controls and found COR LOE Recommendations
AND GUIDELINES
a 1-year survival rate of 79% for acute type B aor-

2. In patients with uncomplicated acute type A
tic dissection treated endovascularly. A subsequent 1 B-NR IMH, prompt open surgical repair is recom-
single-arm study of patients treated with TEVAR mended.1,4-6
found a 30-day mortality rate of 8% and 1-year sur- In selected patients with uncomplicated acute
vival rate of 88%.4 The VIRTUE Registry investiga- type A IMH who are at increased operative
2b C-LD risk and do not have high-risk imaging features
tors5 found a benefit to early intervention but with (Table 30), an initial or expectant approach of
reintervention rates of 20% to 39%. The RESTORE medical management may be considered.6-12
Patient Registry had similar results.6 Fenestration 3. In patients with uncomplicated acute type B
may be required if TEVAR alone does not correct 1 B-NR IMH, medical therapy as the initial manage-
the malperfusion, and visceral or renal artery stent- ment strategy is recommended.1-3,13
ing may also be required. When intervention is an 4. In patients with type B IMH who require repair of
emergency, TEVAR has a significantly lower mor- the distal aortic arch or descending thoracic aorta
2a C-LD (zones 2-5) and have favorable anatomy, endo-
bidity and in-hospital mortality rates compared with vascular repair is reasonable when performed by
open repair, with the greatest advantage among surgeons with endovascular expertise.2,14
older patients.12 5. In patients with type B IMH who require repair
3. With medical management of uncomplicated type B of the distal aortic arch or descending tho-
2a C-LD racic aorta (zones 2-5) and have unfavorable
aortic dissection still having a 30-day mortality rate anatomy for endovascular repair, open surgical
of 10% and a decreased long-term survival, interest repair is reasonable.2,3
remains in determining if early endovascular inter- 6. In patients with uncomplicated type B IMH
vention might reduce the risk of downstream com- 2b C-LD and high-risk imaging features (Table 30),
plication or negative aortic remodeling, particularly intervention may be reasonable.13-16
in patients with high-risk features. In the ADSORB

trial,8 which compared optimal medical manage- Synopsis
ment vs. optimal medical management plus TEVAR,
there were no early deaths in either group and, Aortic IMH is a distinct pathologic entity from aortic
at 1-year follow-up, there was just 1 death in the dissection and PAU. It is characterized by hemorrhage
TEVAR group. TEVAR was superior to optimal medi- within the media of the aortic wall and may occur with or
cal management alone with significant differences without intimal disruption. Radiographically, IMH appears
in incomplete or no false-lumen thrombosis, aortic as a high-attenuation crescentic or circumferential thick-
dilation, and rupture, but the primary clinical benefits ening of the aorta on noncontrast imaging, with absence
are unknown. In the INSTEAD-XL (Investigation of of blood flow through a false lumen on contrast imaging.
Stent-grafts in Aortic Dissection) trial,13 in patients IMH occurs more commonly in the descending thoracic
with uncomplicated type B aortic dissection, prophy- aorta (60%) than in the ascending aorta (30%) or aortic
lactic TEVAR plus optimal medical was associated arch (10%).1 Classification is the same as is used for
with improved 5-year aorta-specific survival and acute aortic dissection. Symptoms at presentation are
delayed disease progression. As the long-term mor- similar to aortic dissection, but patients tend to be older
tality rate for type B aortic dissection that is managed and more often have hypertension and atherosclero-
medically and is strongly related to aortic events, the sis.1,2 Malperfusion can occur but less frequently than in
findings from the ADSORB and INSTEAD-XL trials aortic dissection.1,2 IMH can progress to aortic enlarge-
appear promising, but larger trials with longer-term ment, aortic dissection, or aortic rupture; alternatively,
data are still needed. What remains unknown is the the hematoma can sometimes be resorbed.3 Of patients
optimal timing for TEVAR.14 Features associated presenting with AAS, the proportion who have IMH var-
with an increased need for future intervention are ies based by region, with reports of 6% to 23% in North
summarized in Table 28.9 America and Europe1,6 versus 26% to 44% in Asia.4,5,12
Table 29. Features of Complicated IMH
7.5. Management of IMH Feature
Recommendations for Management of IMH Malperfusion

Referenced studies that support the recommendations are Periaortic hematoma
Pericardial effusion with cardiac tamponade
Persistent, refractory, or recurrent pain
1. In patients with complicated (Table 29) acute
1 B-NR type A or type B aortic IMH, urgent repair is Rupture
recommended.1-3
IMH indicates intramural hematoma.

Table 30. High-Risk Imaging Features of IMH
CLINICAL STATEMENTS
For Type A IMH For Type B IMH
AND GUIDELINES
Maximum aortic diameter >45–50 mm 18,20
Maximum aortic diameter >47–50 mm15,20
Hematoma thickness 10 mm4 Hematoma thickness 13 mm15
Focal intimal disruption with ulcer-like projection involving ascending aorta Focal intimal disruption with ulcer-like projection involving the descending
or arch18,21 thoracic aorta if it develops in acute phase15,16
Pericardial effusion on admission18 Increasing or recurrent pleural effusion19,22
For Both Type A and Type B IMH
Progression to aortic dissection19

Increasing aortic diameter21,22
Increasing hematoma thickness21,22
IMH indicates intramural hematoma.
The management strategy for IMH balances patient management of type A IMH is mostly from Japan,
comorbidities, the differing lethality of type A and type B Korea, and China, all reporting outcomes better
IMH, mortality and death associated with open or endo- than those reported in North America and Europe;
vascular repair in the different segments of the aorta, the differences might be related to genetic or envi-
and the risk of aortic-related complications with medical ronmental factors that affect IMH natural history,
management. Prospective randomized comparative stud- so the Asian results may not be generalizable to
ies are lacking, and most series and registries are limited other ethnic or geographic patient populations.
by small sample sizes. For recommendations regarding The approach varies from initial medical manage-
management of IMH in association with PAU, see Sec- ment with planned “timely” (ranging from within a
tion 7.6, “Management of Penetrating Atherosclerotic few days to before discharge) surgery to expect-
Ulcer (PAU).” ant medical management with surgical intervention
only for complications or disease progression.7-12
One meta-analysis showed acceptable pooled pro-
portion of an all-cause in-hospital mortality rate of

1. IMH, especially type A IMH, can be a lethal condi- 7% and 30-day mortality rate of 15%8 with ini-
tion, complicated by rupture at presentation in 18% tial medical management. Another meta-analysis,
and, in that setting, associated with 100% mortality comparing upfront surgery to initial medical man-
rate without surgical intervention.3 The features of agement with “timely” surgery, showed no signifi-
complications of IMH are summarized in Table 29. cant difference in short-term survival (although an
2. A nonoperative strategy for type A IMH is associ- overall operative approach to type A IMH did show
ated with a mortality rate as high as 40%, according a survival benefit over medical therapy alone).9 For
to the findings of the IRAD.1 Progression to aortic patients at increased operative risk (eg, advanced
dissection, rupture, or other aorta-related adverse age, poor baseline renal function, coronary artery
events occurs in 14% to 37% of patients, with disease), medical management may therefore be
most events occurring within the acute or subacute an option. There are several high-risk imaging fea-
phase.12,17,18 In-hospital mortality (1%–27%)1,4,6 and tures (Table 30) that predict poor outcome (death,
mid- and long-term survival4-6 after operative repair need for surgical intervention, or both) with this
for type A IMH are reasonable and comparable to strategy. Shared decision-making with the patient
or better than survival rates reported for type A aor- should include discussion regarding need for an
tic dissection. There are varied approaches to tim- extended hospital stay of 2 to 3 weeks, including
ing of surgery, with low mortality rate achieved with ≥3 days in the ICU on bedrest, with perhaps ≥5
strategies of repair within 24 hours5 and slightly CTAs during the hospitalization for close monitor-
delayed repair (between 24 and 72 hours), when ing because of the dynamic disease process and
feasible.6 The slight delay may confer an advantage moderate to high risk of progression to aortic dis-
by allowing the hematoma to form and the tissue section and rupture.
quality of the aorta to improve. In addition, the extra 3. Type B IMH may have a more benign prognosis
time can allow for further diagnostic evaluation, than type A IMH, resulting in relatively low in-
optimization of comorbidities, or clearance of novel hospital mortality rate (4%–6%) with medical
oral anticoagulant medications, which may improve management and 9% mortality rate at 1-year fol-
outcomes. Delay is only reasonable in stable low-up.1,2 A strategy of medical management for
patients. The experience with successful medical type B IMH with surgical intervention for severe

recurrent symptoms or radiographic worsening on from the lumen of the aorta in the setting of IMH
CLINICAL STATEMENTS
follow-up was associated with acceptable long- with no associated atherosclerotic plaque. FID
AND GUIDELINES
term survival.3 Intervention, whether surgical or is more specifically defined by its communicat-
endovascular, has associated mortality and mor- ing orifice measuring >3 mm, while tiny intimal
bidity. Although significantly less dissection and disruption has a communicating orifice ≤3 mm.15
rupture may be observed during follow-up with FID occurs in 32% of type B IMH and signifi-
TEVAR, compared with optimal medical therapy, cantly predicts cardiovascular- or aorta-related
this may17 or may not13,14 translate into improved death and aorta-related events,15,16,18 especially
aortic-related outcomes. when it develops in the acute, rather than chronic,
4. Literature supporting endovascular treatment of phase.15 Tiny intimal disruptions are lower risk and
IMH is limited mainly to experience with TEVAR considered a benign finding.16 As 40% of patients
for IMH in the setting of PAU, or TEVAR for mixed can develop FID that was not present on the ini-
type B AAS including IMH; the perioperative mor- tial study,15 early surveillance imaging can help
tality rate for treatment of acute IMH ranges from identify patients at risk for complications. Table
0% to 29%. Of note, in PAU with IMH or IMH with 30 summarizes these and other high-risk imaging
ulcer-like projection, endovascular treatment can features of IMH.
be guided by the focal lesion. IMH with multiple
ulcer-like projections may require more extensive
7.6. Management of PAU
treatment length. Favorable anatomy for TEVAR
would include ideally normal aorta at both proximal 7.6.1. PAU With IMH, Rupture, or Both
and distal landing zones or, at least at the proxi-
Recommendations for PAU With IMH, Rupture, or Both
mal landing zone, as outward tension of the stent Referenced studies that support the recommendations are
graft transferred to abnormal aortic wall can lead summarized in the Online Data Supplement.
to stent-induced new entry tear and subsequent COR LOE Recommendations
aneurysmal degeneration or aortic dissection. In 1. In patients with PAU of the aorta with rupture,
general, stent graft oversizing usually does not 1 B-NR
urgent repair is recommended.1-3
exceed 10%, and balloon aortoplasty at the land- 2. In patients with PAU of the ascending aorta
ing zones is avoided. The experience with TEVAR 1 B-NR with associated IMH, urgent repair is recom-
for retrograde type A IMH associated with a distal mended.1-3
intimal defect (ie, distal arch or descending tho- 3. In patients with PAU of the aortic arch or
2a C-LD descending thoracic aorta with associated
racic aorta) is limited to small case reports and IMH, urgent repair is reasonable.1-3
series. With the higher incidence of atherosclerotic
4. In patients with PAU of the abdominal aorta
disease in patients with IMH compared with aortic 2b C-LD with associated IMH, urgent repair may be
dissection, adequate vessel diameter for endovas- considered.4
cular access should be determined as well.
5. In the IRAD experience for type B IMH, open sur-
gical repair was performed in 5%, endovascular Synopsis
repair in 7%, and a hybrid approach in 1%, with no A PAU is an atherosclerotic lesion of the aorta with
difference in results.1 Good outcomes have been ulceration that penetrates the internal elastic lamina and
reported for open repair,3,19 despite the more inva- allows hematoma formation within the media of the aor-
sive approach. Open surgical repair may be pref- tic wall.5 PAUs may progress to AAS with IMH formation,
erable when IMH extends to the proximal landing aortic dissection, or rupture.1,2,6 PAU with IMH is asso-
zone of anticipated endovascular coverage, the ciated with a high risk of short-term disease progres-
aortic diameter at the proximal or distal extent of sion,1 particularly when localized to the ascending aorta
planned coverage is too large to accommodate (ie, Stanford type A).1,2 Data on outcomes for PAU with
existing stent graft sizes, the hematoma or aneu- descending thoracic and abdominal aorta (ie, Stanford
rysm extends into the aortic arch and circulatory type B) IMH are limited to small retrospective reviews
arrest would facilitate resection of diseased aorta, but suggest significant early disease progression among
or endovascular access for stent deployment is patients treated with medical management.1,2 PAUs tend
anticipated to be inadequate. to affect elderly patients with severe atherosclerotic dis-
6. High-risk imaging features may be present on ease and other comorbidities that put them at high sur-
admission or may develop in the acute, subacute, gical risk even with endovascular interventions, so the
or chronic phases. Ulcer-like projections and risk of repair must be weighed against the risk of severe
focal intimal disruption (FID) are both terms that morbidity and patient life expectancy when making deci-
describe a focal outpouching of contrast arising sions about appropriate management.

Recommendation-Specific Supportive Text Table 31. High-Risk Imaging Features of PAUs
CLINICAL STATEMENTS
1. PAU with rupture that is not treated with interven- Feature
AND GUIDELINES
tion is associated with a high mortality rate (of 5 of Maximum PAU diameter 13–20 mm1
17 patients who presented with PAU with rupture Maximum PAU depth 10 mm1
who did not undergo repair, none survived).3 In con- Significant growth of PAU diameter or depth
trast, in 1 small series, most patients with PAU with PAU associated with a saccular aneurysm5
rupture treated by open or endovascular therapy
PAU with an increasing pleural effusion1
survived to hospital discharge.1
2. PAU of the ascending aorta is uncommon; how- PAU indicates penetrating atherosclerotic ulcer.
ever, when it occurs, and in concert with IMH, the
incidence rate of rupture is 33% to 75%,2,3 and IMH, or both and are more likely to progress or result
progression to aortic dissection is associated with in rupture than asymptomatic PAUs.5 For patients who
a high mortality rate.1 present with a symptomatic PAU but whose symptoms
3. PAUs with type B IMH that are managed conser- resolve with goal-directed therapy or patients who are
vatively are associated with a high risk of disease poor operative candidates at increased risk for morbidity
progression to true aortic dissection or rupture.1,2 In and death from repair, medical management has been
a small retrospective analysis of patients present- pursued, with early and frequent surveillance imaging to
ing with PAUs and type B IMH, 3 of 17 patients assess for disease progression.4
(17.6%) who were managed conservatively died Asymptomatic isolated PAUs are increasingly diag-
from progression of disease to aortic rupture at a nosed incidentally because of the increasing use of CTA.
mean of 9.3 days.1 In contrast, there was 1 death Several series that reported mid or long-term outcomes
among 14 patients (7.1%) who underwent open of retrospective institutional data suggest that isolated
(n=8) or endovascular (n=6) aortic repair for PAU PAUs have radiographic progression in up to 30% of
with type B IMH.1 These data support early inter- patients.1-3,6,7 High-quality data evaluating thresholds for
vention of PAU in the setting of type B IMH in surgical repair are limited, but retrospective data have
patients who are reasonable surgical candidates. shown that PAUs with a diameter of ≥13 mm to 20 mm
4. The natural history of PAU of the abdominal aorta
with associated IMH is not well described, but
low procedure-related and 30-day mortality rates

have been described in several small series and
case reports of both the endovascular and surgical
treatment of abdominal aorta PAUs.7 In a literature
review of 298 published cases of PAU affecting the
abdominal aorta, most authors (62.0%) reported
endovascular stent graft repair as the treatment of
choice, followed by open surgical repair (35.4%)
and conservative management (2.6%).7
7.6.2. Isolated PAU

Recommendations for Isolated PAU

1. In patients with isolated PAU who are symp-
tomatic and have persistent pain that is clini-
1 B-NR
cally correlated with the radiologic findings,
repair is recommended.1-3
2. In patients with isolated PAU who are asymp-
tomatic but have high-risk imaging features
2b C-LD
(Table 31), elective repair may be consid-
ered.1,2,4
Figure 22. Dimensions of Penetrating Atherosclerotic Ulcers.
(A) Maximal aortic diameter at ulcer site diameter (from ulcer across
Synopsis to opposite aortic wall). (B) Depth of intramural blood pool. (C) Length
of intimal defect at ulcer site. (D) Width of intramural blood pool.
Isolated PAUs are those without associated IMH, aortic
Adapted from Gifford et al,11 Copyright 2016, with permission from
dissection, or saccular aneurysm. Symptomatic isolated Elsevier, Inc., and from Cho et al‚9 Copyright 2004 with permission
PAUs may herald a developing peri-ulcer hematoma, from the Society for Vascular Surgery.

or depth of ≥10 mm (Figure 22) are closely associated data are limited about the best treatment approach for
CLINICAL STATEMENTS
with disease progression.1 Significant growth rates are a PAU but, in general, the approach depends on the
AND GUIDELINES
not well defined and depend on the size of the patient, location of the PAU, the patient’s aortic and branch ves-
his or her aortic anatomy, and the presence of high-risk sel anatomy, associated pathology, and patient comor-
features associated with PAU (Table 31). bidities (because these patients tend to be older and
have significant atherosclerosis).4 Procedure-related
Recommendation-Specific Supportive Text and in-hospital death are lower for patients treated with
an endovascular approach, although available data are
1. Symptomatic PAUs are associated with a high risk based on small studies with a high risk of treatment
of early disease progression.2,3,5,7,8 In a small series bias.4 Midterm outcomes after endovascular repair of
of 25 patients presenting with symptomatic PAU PAU have shown a 4% to 8% risk of endoleak4,7 and
managed medically, 30% had disease progression a 5% risk of new PAU formation.7 One-year mortality
on surveillance imaging at a mean of 18 months rates for patients treated with endovascular versus open
follow-up, including expansion of the PAU and repair are similar.7
new IMH in 20% and conversion to aortic dissec- Results of open surgical repair in patients with
tion in 10%.3 All patients in the series went on to ascending aortic PAU are limited to small case series.8-11
require operative repair. In contemporary series, Despite this, open repair remains the gold standard for
most patients with symptomatic PAU without rup- treating AAS that involve the ascending aorta and proxi-
ture have been treated with open or endovascular mal arch, with acceptable morbidity and mortality rates
repair with acceptable results (see Section 7.6.3, compared with medical therapy.12,13
“PAU: Open Surgical Repair Versus Endovascular
Repair”).3,7-9
2. Asymptomatic isolated PAU with large diameter or Recommendation-Specific Supportive Text
depth, significant growth on surveillance imaging, 1. Results of open surgical repair of the ascend-
or other high-risk features (Table 31), are associ- ing aorta and proximal arch can be reasonably
ated with disease progression.1,7 In contrast, inci- applied to PAU. Cases have been reported in
dental aortic PAUs that are asymptomatic and which ascending aortic stenting has been per-
without high-risk features have a low risk of pro- formed with surgeon-modified stent-grafts or
gression (3.6% and 6.5% at 5 and 10 years after off-label use of commercially available devices,
diagnosis, respectively).10 Maximum depth and but currently there is no FDA-approved device
diameter of the PAU can be used to determine for endovascular repair of the ascending aorta or
lesions that would be considered high risk and may proximal arch.
be considered for intervention (Figure 22).4 2. The risk of procedure-related and in-hospital
death is lower for endovascular compared with
7.6.3. PAU Open Surgical Repair Versus open repair of PAU in the descending thoracic and
Endovascular Repair abdominal aorta, although longer-term data are
Recommendations for PAU Open Surgical Repair Versus Endovascular similar for both operative approaches.4
Repair

1. In patients who require repair of a PAU
7.7. Traumatic Aortic Injury
in the ascending aorta or proximal aortic
1 C-LD
arch (zones 0-1), open surgical repair is 7.7.1. Initial Management of Blunt Traumatic
recommended. Thoracic Aortic Injury (BTTAI)
2. In patients who require repair of a PAU in
the distal aortic arch (zones 2-3), descend- 7.7.1.1. Initial Management of BTTAI in the Emergency
2a C-LD
ing thoracic aorta, or abdominal aorta, either Department
open surgical repair1-3 or endovascular repair
is reasonable, based on anatomy and medical Recommendations for Initial Management of BTTAI in the Emergency
comorbidities.4-6 Department

1. In patients with BTTAI, management and
Synopsis treatment at a trauma center with the facili-
1 C-EO
Operative repair of PAUs includes both open and endo- ties and expertise to treat aortic pathology is
recommended.
vascular treatment. Historically, most PAUs were treated
2. In patients with BTTAI, anti-impulse therapy
with open aortic replacement, although more contem- to reduce the risk of injury extension and
porary series have reported good technical success and 1 C-LD rupture should be implemented, except in
short- and midterm outcomes after endovascular repair patients with hypotension or hypovolemic
shock.1,2
in the descending and infrarenal aorta.4-7 Comparative

Synopsis 2. Although no randomized trials exist, historical lit-
CLINICAL STATEMENTS
erature shows that aortic rupture occurs in ∼12%
BTTAI, although rare, is the second-most common cause
AND GUIDELINES
of patients with BTTAI who were awaiting repair
of death in trauma patients; it results from high decelera-
without medical management; small studies using
tion forces and is often associated with concomitant inju-
protocols of beta blockers as first-line therapy
ries. In the ACS National Trauma Databank, the diagnosis
have reported rates of 0% rupture while awaiting
of BTTAI increased 196.8% from 2003 to 2013, likely
repair.1,2 In the acute trauma setting, hypovolemia
attributable to more sensitive imaging.3 The mortality rate
may result in permissive hypotension, obviating
of patients with BTTAI who were treated in the emer-
the need for administering anti-impulse medica-
gency department was ∼19%.4,5 Initial management of
tions (typically intravenous beta blockers with or
polytrauma at trauma centers follows Advanced Trauma
without supplemental intravenous vasodilators [eg,
Life Support protocols. However, for patients with BTTAI,
nicardipine, clevidipine, sodium nitroprusside]) to
special attention to BP and heart rate is warranted
decrease aortic wall stress. Conversely, permis-
because of their effects on injury extension and rupture.
sive hypotension may not be tolerated with other
In stable patients, the 2011 Society for Vascular
concomitant injuries, in which adequate end-organ
Surgery clinical practice guidelines6 suggested urgent
perfusion requires higher BPs.
(<24 h) repair barring other serious concomitant non-
aortic injuries or immediately after treatment of other 7.7.1.2. Approach to the Initial Management of BTTAI
injuries. Optimal timing of intervention, however, remains
Recommendations for Approach to the Initial Management of BTTAI
unclear. In a recent study from the National Trauma Data
Bank, early (<24 h) repair had increased odds of death
(adjusted OR, 2.39; 95% CI, 1.01–5.67; P=0.047).7 A 1. In patients with grade 1 BTTAI (Figure 23),
1 C-LD nonoperative management and follow-up
multicenter study showed worse adjusted mortality rate imaging are recommended.1,2
with early repair overall (adjusted OR, 7.78; 95% CI,
2. In patients with grade 3 to 4 BTTAI (Figure
1.69–35.70; adjusted P=0.008) although, in subgroup 1 C-LD 23) and nonprohibitive comorbidities or inju-
analysis, mortality rate differences only trended toward ries, aortic intervention is recommended.1,3
favoring delayed repair (P>0.05).8 3. In patients with grade 2 BTTAI (Figure 23) and
2a C-LD with high-risk imaging features (Table 32), aortic
intervention is reasonable.3,4
4. In patients with grade 2 BTTAI (Figure 23)

1. Patients with BTTAI are at elevated risk of aortic- and without high-risk imaging features
related and overall mortality. Because of the acuity 2b C-LD (Table 32), nonoperative management and
follow-up surveillance imaging may be
of injury and severity of concomitant polytrauma, reasonable.3,4
expertise in aortic imaging and treatment at the
treating facility is paramount to improve outcomes.
Further, most patients will benefit from care at a Synopsis
level 1 trauma center with multidisciplinary expertise The most common site of BTTAI is the aortic isthmus,
in treating concomitant injuries, although the risk of because of its site as transition from the unfixed aor-
delayed treatment because of transport time must be tic arch to the fixed descending thoracic aorta and the
weighed against the benefits of immediate treatment. relatively lesser tensile strength of this region. Other
Figure 23. Classification System for BTTAIs.

Aortic injuries are classified according to severity, based on the findings of diagnostic imaging. Grade 1, intimal tear. intimal flap, or both. Grade 2,
intramural hematoma. Grade 3, aortic wall disruption with pseudoaneurysm. Grade 4, aortic wall disruption with free rupture. BTTAI indicates blunt
traumatic thoracic aortic injury. Adapted from Azizzadeh et al,2 Copyright 2009, with permission from Elsevier, Inc. and the Society for Vascular Surgery.

Table 32. High-Risk Imaging Features of BTTAI 4. Findings of secondary signs of injury and multiple
CLINICAL STATEMENTS
Posterior mediastinal hematoma >10 mm8 secondary signs are more common in patients with
AND GUIDELINES
higher-grade of aortic injury and may prompt stron-

Lesion to normal aortic diameter ratio >1.48
ger consideration for operative intervention.6 The
Mediastinal hematoma causing mass effect6
presence of aortic arch hematoma of >15 mm in
Pseudocoarctation of the aorta6 thickness was predictive of death.7
Large left hemothorax6
Ascending aortic, aortic arch, or great vessel involvement9 7.7.1.3. Endovascular Versus Open Surgical Repair
Aortic arch hematoma7 Recommendation for Endovascular Versus Open Surgical Repair
BTTAI indicates blunt traumatic thoracic aortic injury. in the Online Data Supplement.

segments that may be involved include the proximal 1. In patients with BTTAI who meet indica-
ascending aorta (8%–27%), aortic arch (8%–18%), and 1 B-NR
tions for repair and with appropriate anat-
omy, TEVAR is recommended over open
distal descending thoracic aorta (11%–21%). The most repair. 1-3
widely used grading scale is that proposed by Estrera
et al and endorsed by the SVS clinical practice guide-
line (Figure 23).1,2 In Estrera’s original paper, all patients Synopsis
with grade 1 injuries were managed medically and had a Endovascular therapy for BTTAI has become the pre-
0% mortality rate.2 Current SVS guidelines recommend dominant approach. From 2007 to 2015, rates of open
expectant management of grade 1 injuries and repair of repair decreased from 7.5% to 1.9%, while rates of
all other grades.1 Trauma studies have found that 32% TEVAR increased from 12.1% to 25.7%.2
of BTTAIs are managed nonoperatively,3 with an associ- No randomized trials for open versus endovascular man-
ated mortality rate of 25%.5 Overall mortality rate was agement have been conducted.4 Rather, trauma registry
significantly higher in nonoperatively managed patients data and meta-analyses have shown that, in patients with
(35.0% versus 11.2%; P<0.001), while aortic-related suitable anatomy, TEVAR offers superior 30-day mortality
mortality rate was similar (9.8% versus 5.0%; P=0.119).3 rates and lower rates of SCI and acute kidney injury. Con-
comitant injuries may prompt concern over procedural use
Recommendation-Specific Supportive Text: of heparin, and the use of periprocedural heparin should be
Management balanced against the overall bleeding risk for each patient.
In a small study of TEVAR in patients with predominantly
1. The decision for nonoperative versus opera- grade 3 BTTAI, there were no differences in bleeding, throm-
tive management of BTTAI includes complex and boembolism, or mortality rates between use of full heparin,
dynamic factors such as the patient’s stability, low-dose heparin, and no heparin, although patients who
concomitant injuries, and potential imaging char- received full heparin underwent repair at a time interval 3
acteristics that may predict aortic stability. Grade 1 times longer than did those who received no heparin.5
BTTAIs are likely to resolve and are associated with
extremely low aortic-related death. Medical man-
agement and follow-up imaging to ensure resolu- Recommendation-Specific Supportive Text
tion is appropriate.1,2 1. Compared with open repair, endovascular treatment
2. Grade 3 and 4 BTTAIs are at high risk of pro- of BTTAI is associated with improved procedural and
gression and rupture and should be treated in an 30-day mortality rates, as well as postoperative com-
urgent manner. In grade 3 injuries, nonoperative plications, including SCI and acute kidney injury.1-3,6
management was an independent predictor of all- In a meta-analysis of 17 retrospective studies,
cause death (OR, 29.65; 95% CI, 5.62–15.649; TEVAR was associated with lower procedural and
P<0.0001), and imaging characteristics did not 30-day mortality rates (OR, 0.31 and 0.44, respec-
predict aortic-related death.4 tively) and postoperative paraplegia (OR, 0.32).1
3. Although injury grade was an independent predic- Murad et al showed similar reductions in mortality
tor of aortic-related death, outcomes of grade 1 (relative risk, 0.61) and SCI (relative risk, 0.34) in
and 2 injuries were similar between nonoperative 139 studies encompassing 7768 patients.3 Studies
management and TEVAR, including in-hospital using the National Trauma Data Bank, a multicenter
and aortic-related death (P>0.05).3 A high-volume registry of trauma centers, also have identified sig-
center reported no differences in mortality rates or nificantly improved mortality rates, shorter ICU and
aortic-related mortality rates between nonopera- shorter hospital stay, and lower rates of acute kidney
tive and operative management of grade 1 and 2 injury and acute respiratory distress syndrome2,6 for
injuries.4 TEVAR compared with open repair.

7.7.2. Initial Management of Blunt Traumatic Table 33. Descriptions of Blunt Aortic Injury Grades
CLINICAL STATEMENTS
Abdominal Aortic Injury (BAAI) Injury
AND GUIDELINES
Recommendations for Initial Management of BAAI Grade Descriptions
Referenced studies that support the recommendations are 1 Minor intimal tear, intimal defect, or thrombus (10 mm)
2 Large intimal flap, intimal defect, or thrombus (10 mm in
COR LOE Recommendations length or width)
1. In patients with grade 1 to 2 BAAI 3 Pseudoaneurysm
(Table 33) without malperfusion, anti-
impulse therapy, if clinically tolerated, and 4 Aortic rupture
1 C-LD
repeat imaging within 24 to 48 hours of the
initial scan is recommended to reduce risk of In their descriptions of management of BAAIs, Shalhub et al1,2 use an aortic
injury progression.1 injury grading system described by Starnes et al13. Instead of using IMH to
define grade 2 injuries, as did Azizzadeh et al,14 Starnes et al13 define grade
2. In patients with grade 4 BAAI (Table 33), 2 injuries based on a higher degree of intimal injury, defect, thrombus, or all of
1 C-LD repair should be performed to address life- them to match radiographic findings that they deemed to be less ambiguous.
threatening aortic injury.2-4 BAAI indicates blunt traumatic abdominal aortic injury; and IMH, intramural
hematoma.
3. In patients with grade 2 BAAI (Table 33) and
2a C-LD associated malperfusion, it is reasonable to
consider repair.1
status, hospital resources, and practitioner experience.
4. In patients with BAAI, treatment with either Additionally, Shalhub et al1 propose using aortic injury
endovascular or open repair is reasonable
2a C-LD and depends on degree of injury, aortic
zone categorization when considering options for repair,
anatomy, and the patient’s overall clinical which differ from traumatic abdominal zones of injury
status.1-4 (Figure 24). Specifically, in their multicenter experience,
5. In patients with grade 3 BAAI (Table 33), some zone 2 and 3 injuries could be managed endovas-
it may be reasonable to consider repair to
2b C-LD cularly while no zone 2 injuries were managed this way.
reduce risk of progression to life-threatening
injury.5 Lastly, data on the use of REBOA for hemorrhage below
6. In patients with BAAI, the usefulness
the diaphragm, not performed in the operating theater,
of routine application of resuscitative and without fluoroscopic guidance are mixed, with few
3: Harm B-NR
endovascular balloon occlusion of the data showing survival benefit and some trauma registry
aorta (REBOA) for hemorrhage control
is unclear and, in some cases, may cause
data showing harm.
harm. 6-8

Synopsis 1. Because BAAI is very rare, in an effort to provide
BAAI represents a rare traumatic entity, occurring in <1% clinical evidence for the management of BAAI,
of patients with blunt trauma. Patients with BAAI often Shalhub et al1 aggregated data from 12 trauma
have concomitant injuries such as rib fractures, abdomi- centers. In the authors’ experience in treating 113
nal visceral injury, and cardiac complications that will patients with BAAI, most of those with grade 1
affect treatment decisions. Similar to BTTAI, abdominal and 2 injuries were successfully managed non-
aortic injuries are graded based on aortic contour defects, operatively with anti-impulse therapy and repeat
and this grading can be used to provide a framework CTA imaging. Most of these injuries did not show
for treatment and determination of risk of major morbid- progression and did not require in-hospital inter-
ity and death from injuries (Table 33). Because BAAI is vention. However, some patients will develop
rare and symptoms are wide ranging, patients should angiographic progression of lesions or develop
be managed on an individual basis. In general, patients symptoms from vessel occlusion, aneurysmal
with grade 1 aortic injuries can likely be managed with degeneration, or pseudoaneurysm formation. Such
antihypertensive therapy, beta blockade, and antiplatelet progression should prompt consideration of treat-
therapy, if not contraindicated, with repeat scan at 24 to ment to prevent further progression to symptom-
48 hours. Grade 2 injuries can similarly be managed non- atic or life-threatening disease.
operatively but may progress to include end-organ ves- 2. Patients with grade 4 injuries are more likely to
sel thrombosis or rupture. Grade 3 injuries may benefit present with hypotension and aortic transection
from endovascular treatments if anatomically amenable. as well as visceral vessel avulsion.2-4 In a single-
Grade 4 injuries are more likely to present with refractory center experience, all 8 patients with grade 4
hypotension, warranting rapid control of hemorrhage, injuries experienced cardiac arrest in the emer-
which may be done in the emergency department (eg, gency department or operating room. Although all
antero-lateral thoracotomy with aortic cross-clamping) or 8 patients survived to reach the operating room
operating room. Whether open or endovascular means and 7 survived the repair, all died within days of
are used for BAAI repair will depend on patient’s clinical injury. In multicenter experience, the mortality rate

CLINICAL STATEMENTS
AND GUIDELINES
Figure 24. Abdominal Aortic Zones of Injury for Surgical Approaches and Abdominal Zones of Injury Based on Trauma
Classification.
(A) The abdominal aortic zones of injury described by Shalhub et al.1 (B) The abdominal zones of injury traditionally described in trauma. The
abdominal aortic zones of injury may help in prognostication and deciding whether an endovascular or open repair is feasible. Shalhub et al1
found that the mortality rate was highest in zone 2 (see panel A) grade 4 aortic injuries (Table 33). Moreover, no zone 2 aortic injuries identified
in a multicenter experience were managed by endovascular means. Panel A, adapted from Shalhub et al.1 Copyright 2014, with permission from
Wolters Kluwer Health, Inc.
for grade 4 injuries was 83%. Most deaths from grade 4 injuries were treated with open surgery.1,2
BAAI were within the first 24 hours of presenta- Currently, no FDA-approved devices are available
tion and attributable to cardiac arrest from hemor- specifically for treating trauma in the abdominal
rhagic shock. aorta; consequently, clinical judgment and experi-
3. Patients may present with grade 2 injuries without ence are paramount in choosing an endovascular
evidence of malperfusion and thus be managed solution.
nonoperatively. However, for patients who pres- 5. Pseudoaneurysm repair is often performed to pre-
ent with or progress to organ or limb malperfu- vent progression to uncontrolled aortic rupture,
sion, endovascular or open repair may be needed although data on characteristics associated with
to reduce morbidity and mortality rates. In their progression are scarce. In their multicenter study
multicenter experience, Shalhub et al1 found that of BAAI, Shalhub et al1 found that only 30% of
of the 38 patients who present with grade 2 inju- pseudoaneurysms were managed nonoperatively,
ries, 45% were initially managed nonoperatively, and failure of nonoperative management occurred
34% were treated with open repair, and 21% were in 3 of these patients.
treated with endovascular repair. Of those initially 6. REBOA has reemerged over the past 10 years
managed conservatively, 3 eventually progressed as a form of rapid hemorrhage control in trauma.
to having ischemic symptoms warranting consid- Many health care centers have shown the feasi-
eration of repair, with 1 patient who refused repair bility of trauma surgeon or emergency physician
who died of sepsis from limb ischemia, another who placement of endovascular balloons for hemor-
died intraoperatively, and a third who successfully rhage control.6,9,10 with a few studies showing sig-
underwent hybrid endovascular and open repair. nificant improvement in SBP after placement11 and
4. Both endovascular and open approaches have survival benefit compared with those who were not
been described for BAAI,1-4 and analyses of large treated with REBOA12 or those treated with open
trauma databases reveal no significant differences methods of hemorrhage control.8 However, pro-
in mortality rates between the two. Anatomical pensity-matched studies using large trauma data-
considerations, patient clinical status and comorbid bases showed increased mortality rate and risk of
injuries, and practitioner experience will influence complications, such as acute kidney injury, amputa-
the choice of approach. Shalhub et al1 found that tion, or both, with use of REBOA.6-8 There are clini-
aortic zone 2 and 3 injuries appeared to be more cal scenarios in which REBOA is contraindicated.
amenable to endovascular approaches, while most According to the current US Army Joint Trauma

System clinical practice guidelines, REBOA is con- 2. Among patients with blunt traumatic injury who
CLINICAL STATEMENTS
traindicated in those with pericardial tamponade are managed nonoperatively, injury progression
AND GUIDELINES
and major thoracic hemorrhage. Relative contrain- occurred in 7.6% of patients, and injury healing
dications to REBOA use include cardiac arrest or or improvement was observed in 34% of patients
shock caused by penetrating chest trauma. after a range of 1 day to 118 months of follow-
up.5 Injury progression, intervention, or both occur
7.7.3. Long-Term Management and Surveillance
in 0.68% of patients with grade 1 to 2 BTAI.5 Long-
After Blunt Traumatic Aortic Injury (BTAI)
term data for outcomes of blunt aortic injuries
Recommendations for Long-Term Management and Surveillance After managed nonoperatively are lacking.
BTAI

1. In patients with BTAI who have undergone 7.8. Long-Term Management and Surveillance
aortic repair, surveillance imaging at intervals
appropriate for the repair approach and loca- Imaging After AAS
2a C-LD
tion (see Section 7.8, “Long-Term Manage-
ment and Surveillance Imaging Following 7.8.1. Long-Term Surveillance Imaging After Aortic
AAS”) is reasonable.1-4
Dissection and IMH
2. In patients with BTAI who have not undergone
repair, surveillance imaging with a CT at 1 Recommendations for Long-Term Surveillance Imaging After Aortic
month, 6 months, and 12 months after the Dissection and IMH
2b C-LD Referenced studies that support the recommendations are
diagnosis and, if stable, at appropriate inter-
vals thereafter (depending on the type and summarized in the Online Data Supplement.
extent of the injury), may be reasonable.5 COR LOE Recommendations
1. In patients who have had an acute aortic dis-
section and IMH treated with either open or
Synopsis 1 B-NR
endovascular aortic repair and have residual
aortic disease, surveillance imaging with a
In-hospital and midterm outcomes after open and endo- CT (or MRI) is recommended after 1 month,
6 months, and 12 months and then, if stable,
vascular repair for BTAI are good for patients who sur- annually thereafter.1-6
vive to hospital discharge.2-4 However, long-term data are
2. In patients who have had an acute aortic dis-
limited that report outcomes after open or endovascu- section and IMH that was managed with medi-
lar surgical repair for blunt aortic injury. The SVS clini-
cal therapy alone, surveillance imaging with a

1 B-NR
cal practice guidelines for traumatic thoracic aortic injury CT (or MRI) is recommended after 1 month,
6 months, and 12 months and then, if stable,
suggest that follow-up after TEVAR could be decreased annually thereafter.7
to every 2 to 5 years in the absence of abnormalities on
follow-up imaging (ie, stent graft migration, endoleak) or
could follow-up standard postoperative imaging surveil- Synopsis
lance paradigms.6 No published guidelines are available
Survival after an acute aortic dissection and IMH does
for postoperative surveillance after open or endovascular
not guarantee freedom from subsequent aortic events
abdominal aortic repair for blunt aortic injury.
because of residual aortic dissection and risk of aneu-
Long-term data about outcomes of blunt aortic inju-
rysm formation. Ten-year survival after repair of acute
ries managed nonoperatively are limited. A recent sys-
type A aortic dissection is approximately 60% to 65%.1,8
temic review of nonoperative management of blunt
Risk of reoperation is increased for the aortic valve, the
thoracic aortic injuries showed low aortic-related event
aortic root, and the distal aorta,1,8,9 with an aortic root
rates but injury progression in 7.6% of patients on sur-
reoperation rate of approximately 15% at 15 years.9,10
veillance imaging (follow-up, 1 day to 118 months).5 In
The growth rate of the distal aorta is ∼1 mm/y, and the
published series of blunt aortic injury, patients with dis-
risk of distal aortic reoperation ranges from 10% to 16%
ease progression on repeat imaging all undergo repair.4,5
at 10 years.1,8,9 Although the use of TEVAR provides pro-
tection from early aortic-related death11 in acute type B
Recommendation-Specific Supportive Text aortic dissection, reintervention rates after TEVAR for
can range from 27% to 39% at midterm follow-up.11,12
1. In-hospital data suggest that endograft malposi- Surveillance imaging after thoracic aneurysm repair is
tion (3%) and endoleak (2%) may occur in some critical to monitor for progression of residual aortic dis-
patients immediately after endovascular repair,1 but ease and the potential need for reintervention.
midterm data after open or endovascular repair of
BTAIs suggest a low incidence of endoleak, stent
migration, or reintervention after a mean of 52 to Recommendation-Specific Supportive Text
60 months.2-4 Long-term data for outcomes of 1. Although patients with uncomplicated type B aor-
open or endovascular repair of BTAI are lacking. tic dissection who are managed medically have a

favorable early prognosis, delayed aortic expansion extent 3A dissection cases. Reintervention rates
CLINICAL STATEMENTS
occurs in 20% to 50% of patients over 4 years,7 so after TEVAR range from 15% to 26% at 5 years and
AND GUIDELINES
regular surveillance imaging is essential to detect are dependent on the extent of aortic dissection.4
midterm and late aortic growth.7
7.8.3. Long-Term Management and Surveillance for
2. After surgical replacement of the ascending tho-
PAUs
racic aorta in acute type A aortic dissection, patients
remain at risk for progressive enlargement of unre- Recommendations for Long-Term Management and Surveillance for
PAUs
paired segments of residual dissected aorta, as well
as potential growth of nondissected native aortic
segments because of underlying medial degenera- 1. In patients with a PAU who have under-
gone aortic repair, surveillance imaging
tion. Consequently, repeat intervention on the aortic at intervals appropriate for the repair
2a C-LD
root, arch, or thoracoabdominal aorta may become approach and location (see Section 6.5.6,
necessary. For acute type B aortic dissection,1,2 “Surveillance After Aneurysm Repair”) is
reasonable. 1-3
TEVAR may leave a patent false lumen, which can
2. In patients with a PAU that is being managed
lead to aneurysm growth, and can be complicated
medically, surveillance imaging with a CT is
by early endoleaks in up to 35% of patients and reasonable at 1 month after the diagnosis
late endoleaks in 13% of patients.5 Careful follow- 2a C-LD and, if stable, every 6 months for 2 years,
and then at appropriate intervals thereafter
up is needed to monitor for progression of disease
(depending on patient age and PAU charac-
in both dissected and nondissected aorta. In addi- teristics).1,4
tion to using cross-sectional imaging for most of
the aorta, TTE can be helpful in monitoring aortic
root anatomy and aortic valve function over time. Synopsis
For patients who undergo repair of a PAU, clinical failure
7.8.2. Long-Term Management After Acute Aortic (defined as endoleak, disease progression, graft occlu-
Dissection and IMH sion, repeat aortic intervention, or procedure or aortic-
Recommendation for Long-Term Management After Acute Aortic related death) by 1 year after endovascular and open
Dissection and IMH repair occur in 8.6% and 8.7%, respectively.1 No long-
in the Online Data Supplement. term data exist for outcomes after repair of PAU, but
aortic-related complication rates after intervention are

likely similar to those for TAA.
1. In patients with a previous acute aortic
dissection and IMH, whether initially
For patients with PAUs who are managed nonopera-
treated medically or with intervention, tively, the risk of disease progression is significant.1,5,6
1 B-NR who have chronic residual TAD and an Disease progression occurs more frequently in patients
aneurysm with a total aortic diameter of
≥5.5 cm, elective thoracic aortic repair is
presenting with symptomatic versus asymptomatic
r ecommended. 1-4 PAUs7 but is >15% for both.7,8 Among patients with a
PAU who have progression of disease on surveillance
imaging, 73% will show continued worsening on subse-
Synopsis quent imaging, and 46% will have progression to frank
Despite the outcomes reported for surgical repair of dissection after a mean of 12 months.
acute aortic dissection and IMH, a risk of ongoing growth
is possible in the residually dissected as well as nondis-
sected thoracic aortic segments. When surveillance imag-
ing detects progression of residual aortic disease after 1. After open or endovascular repair of a PAU, there
successful treatment of acute aortic dissection and IMH, is a 9% risk of clinical failure by 12 months postop-
there may be a potential need for aortic reintervention. eratively.1 Freedom from cumulative complications
and interventions is 86% at 12 months, 79% at
24 months, and 71% at 36 months postopera-
Recommendation-Specific Supportive Text tively.2 After 18 months of follow-up, new PAUs
1. Reoperation after acute type A aortic dissection are observed in approximately 5% of patients who
repair is associated with low rates of complication. have undergone repair of a different PAU.3
The primary indications for reoperation are aneu- 2. In a series of 109 patients with acute PAUs, 28%
rysms of the thoracic aorta, aortic anastomotic suffered from an aortic-related adverse event by
pseudoaneurysms, progressive AR, or graft infec- 30 days of follow-up.4 Based on a systematic
tion.1 Operative mortality rate of elective repair is review of 184 patients with either thoracic or aortic
<10%.1-4 After TEAVR, false-lumen thrombosis can PAU, 30% had radiographic evidence of disease
occur in 62% of extent 3B dissection and 91% of progression on midterm follow-up.1 For patients

with abdominal PAU, 23% have disease progres- Synopsis
CLINICAL STATEMENTS
sion on CTA by 8 months of follow-up, although the
Pregnancy leads to hemodynamic and hormonal changes
AND GUIDELINES
risk is higher in symptomatic (43%) versus asymp-
and is a risk factor for aortic dissection in women with
tomatic (17%) patients.7
aortopathy.21 Aortic dissection may occur throughout
pregnancy or several weeks postpartum, with most in the
third trimester or up to 12 weeks’ postpartum.21 Women
8. PREGNANCY IN PATIENTS WITH with aortopathy, including Marfan syndrome,6,7,13,14,18,19,22
AORTOPATHY Loeys-Dietz syndrome,2,22,23 vascular Ehlers-Danlos syn-
8.1. Counseling and Management of Aortic drome,3,24 nsHTAD,25,26 Turner syndrome,12,27 and BAV
with aneurysm21,28 are at risk of pregnancy-related aortic
Disease in Pregnancy and Postpartum dissection. Type A aortic dissection in pregnancy associ-
Recommendations for Counseling and Management of Aortic Disease ates with aortic dilation, but type B aortic dissection may
in Pregnancy and Postpartum
occur without aortic dilation.6,13,22
Before pregnancy, women with or at risk for aortopa-
Prepregnancy thy undergo TTE (and MRI or CT, as appropriate) and
1. In patients with genetic aortopathies attributable are counseled about risks of aortic dissection informed
to syndromic (Marfan syndrome, Loeys-Dietz by specific circumstances. Aortic surveillance imaging
syndrome, vascular Ehlers-Danlos syndrome)
1 C-LD and nsHTAD and who are contemplating preg- throughout pregnancy and several weeks postpartum is
nancy, genetic counseling before pregnancy to performed to monitor aortic size.9
discuss the heritable nature of their condition is In women at low risk, vaginal delivery is performed with
recommended.1-4
efforts to lessen hemodynamic stress and shorten the
2. In patients with syndromic and nsHTAD, Turner
syndrome, BAV with aortic dilation, and other
second stage of labor.9,14 Women at increased risk of aor-
aortopathy conditions, aortic imaging (with tic complications typically undergo cesarean d elivery.9,14
1 C-LD
TTE, MRI or CT, or both as appropriate) before In women with aortopathy, prepregnancy genetic
pregnancy is recommended to determine aortic
diameters.1-3,5-13
counseling, aortic imaging, discussion about aortic dis-
section risk, and shared decision-making are necessary.9
3. In patients with syndromic and nsHTAD,
Turner syndrome, BAV with aortic dilation, and
other aortopathy conditions, who are contem- Recommendation-Specific Supportive Text
1 C-LD
plating pregnancy, counseling about the risks
of aortic dissection related to pregnancy is 1. HTAD encompasses conditions in which aortic
recommended.2-5,10,12,14
disease has an underlying genetic trigger or famil-
During Pregnancy ial occurrence.29,30 Marfan syndrome, Loeys-Dietz
4. In patients with aortic aneurysms, or at increased syndrome, vascular Ehlers-Danlos syndrome, and
risk of aortic dissection, or both, it is recom- nsHTAD are autosomal dominant conditions with
mended that pregnancy be managed by a
multidisciplinary team including a maternal fetal an inheritance risk of 50%.9,30 BAV may also be
2a C-EO
medicine specialist and cardiologist, and, if familial. Prepregnancy counseling by a genetic
logistically feasible, that delivery be planned in counselor, medical geneticist, or both or aortopathy
a hospital where the capability for emergency
aortic repair is available. specialist is recommended to discuss the heritabil-
5. In patients with aortopathies who are preg-
ity of these conditions and to identify at risk rela-
1 C-LD nant, guideline-directed treatment of hyper- tives and to discuss pregnancy concerns.9,30
tension is recommended.61517 2. Women with aortopathic conditions are at risk for
6. In patients with syndromic and nsHTAD, aortic dilation and aortic dissection related to preg-
1 C-EO
beta-blocker therapy during pregnancy and nancy.14,22,67 Evaluation of the aortic root, ascending
postpartum is recommended, unless contrain-
dicated.
aorta, or both by echocardiogram before preg-
nancy in women with aortopathy is important for
7. In pregnant patients with an aortopathic
condition or a dilated aortic root or ascend- prepregnancy counseling and management during
ing aorta, surveillance TTE to monitor aortic pregnancy.1-3,5-7,9,11,13 In conditions that associate
diameters and aortic valve function is recom- with aortic disease distal to the ascending aorta,
1 C-LD mended each trimester and again several
weeks postpartum, although imaging may be prepregnancy MRI or CT is performed to evaluate
more frequent depending on aortic diameter, for aortic disease.2,5,9,14
aortic growth rate, and underlying condi- 3. The risk of type A aortic dissection in pregnancy
tion.7-9,17,18
relates to the aortopathy condition and aortic diam-
8. In pregnant patients with aortic disease who
require surveillance imaging of the aortic arch,
eter, but type B aortic dissection may occur with-
1 C-LD descending, abdominal aorta, or all 3, MRI without significant aortic dilation.6,22 Most dissections
out gadolinium is recommended over CT to avoid related to pregnancy occur in the third trimester
radiation exposure to the fetus.19,20
and in the first 12 weeks’ postpartum.21 Awareness

of the signs and symptoms of acute aortic dissec- absence of controlled trials, beta blockers are used
CLINICAL STATEMENTS
tion among stakeholders may improve diagnosis in other aortopathic conditions, and continuation of
AND GUIDELINES
and outcomes. In Marfan syndrome, type A aortic such therapy during pregnancy is recommended
dissection risk is very low when aortic diameters are unless contraindicated.2,5,9 Shard decision-making
<4.0 cm and are much higher at diameters >4.5 is required, understanding that fetal growth and
cm. In series of women with TGFBR1 or TGFBR2 weight may be impaired when beta blockers are
pathogenic variants, aortic dissection was reported used in pregnancy.15 However, in ROPAC (Registry
in 0% to 19% of pregnancies.21 Rapid aortic growth Of Pregnancy And Cardiac disease), there was
in pregnancy is reported in Loeys-Dietz syndrome.2 no significant difference in birth weight in women
Limited data are available on pregnancy and treated with a beta blocker compared with
SMAD3, TGFB2, or TGFB3 pathogenic variants.31,32 untreated women (2960 g [2358–3390 g] versus
Maternal mortality rates in vascular Ehlers-Danlos 3270 g [2750–3570 g]); P =0.25).14 Because aor-
syndrome have ranged from 4% to 25%.3 Among tic dissection may occur postpartum, beta-blocker
283 women with vascular Ehlers-Danlos syndrome therapy is continued for at least several weeks
with 616 delivered pregnancies, 30 women died, after delivery and indefinitely for those with indica-
with a pregnancy-related death rate of 4.9%.3 tions for long-term use.
Pregnancy has typically been avoided in women 7. Pregnancy-associated increases in maternal blood
with vascular Ehlers-Danlos syndrome.9 The deci- volume, heart rate, stroke volume and cardiac out-
sion to proceed with pregnancy in vascular Ehlers- put, and neurohormonal activation begin in the
Danlos syndrome is complex and, for some women first trimester and peak in the third trimester and
with specific genetic variants, null mutations, and peripartum period.9 In women with aortopathic
normal vascular imaging, the risk may be lower, and conditions, the aorta may dilate during preg-
shared decision-making is required.3 Aortic dissec- nancy,7 and significant dilation is a risk factor for
tion at small aortic diameters has been reported in ROPAC.8,14 Aortic imaging frequency during preg-
some patients with nsHTAD.25,26,33 Aortic dissection nancy is variable and is performed every trimester
related to BAV is rare and, when reported, associ- but may be performed more frequently depending
ates with aneurysmal dilation.14,21,28 on individual factors, including the specific aorto-
4. For women with aortopathic conditions, multidisci- pathic condition, aortic diameter, and rate of aortic
plinary evaluation before and throughout pregnancy growth.3,5,9,10,12-14,25 Evaluation of the aorta several
can evaluate and manage BP, aortic diameter, and weeks postpartum to determine aortic diameter is
monitor pregnancy for complications. Delivery in a performed to evaluate for aortic dilation.9
setting in which cardiothoracic surgery is available 8. In patients with aortopathy that involves the aortic
for urgent management of aortic dissection allows arch, descending or abdominal aorta or branches,
rapid treatment for this complication.25 Educating or all of them, cross-sectional imaging identifies
women with aortopathic conditions and their phy- aortic anatomy and diameters. MRI without gado-
sicians about the signs and symptoms of acute linium contrast is low-risk during pregnancy and is
aortic dissection may allow earlier diagnosis and preferred over CT for elective imaging to avoid the
improve outcomes.12,21,25 risks of ionizing radiation exposure to the devel-
5. Hypertension is a risk factor for aortic dissection in oping fetus.9,19,20 A TEE can be performed during
pregnancy.6 For appropriate patients with or with- pregnancy, if required, to evaluate the descending
out hypertension, beta blockers are used through- aorta.
out pregnancy and postpartum, recognizing that
fetal growth may be impaired.13,15 Labetalol is sug-
8.2. Delivery in Pregnant Patients With
gested as the beta blocker of choice for use in
pregnant women with hypertension.35 Other agents Aortopathy
may be required as suggested by international Recommendations for Delivery in Pregnant Patients With Aortopathy
guidelines.16,34 ARBs and ACEIs are contraindi- COR LOE Recommendations

cated during pregnancy because of teratogenicity. 1. In pregnant patients with a history of chronic
Calcium channel blockers are generally avoided, 1 C-EO aortic dissection, cesarean delivery is recom-
mended.
when possible, in Marfan syndrome based on lim-
ited information and concerns raised from mouse 2. In pregnant patients with an aortopathy and
an aortic diameter of <4.0 cm, vaginal delivery
models.34,35 1 C-EO
(when otherwise appropriate) is recom-
6. Beta-blocker therapy has been shown to lessen mended.
aortic growth rates in Marfan syndrome and is rec- 3. In pregnant patients with a diameter of the
ommended to lessen hemodynamic aortic stress in 2a C-EO aortic root, ascending aorta, or both, of ≥4.5
cm, cesarean delivery is reasonable.
Marfan syndrome and related conditions.4,5,13 In the

Recommendations for Delivery in Pregnant Patients With Aortopathy dissection has been reported to be low risk in small
CLINICAL STATEMENTS
(Continued) series of women with aortic diameters between
AND GUIDELINES
COR LOE Recommendations 4.0 cm and 4.5 cm,2,7,8 but aortic dissection related
4. In pregnant patients with a diameter of the to pregnancy at this diameter may occur.1 Type B
aortic root, ascending aorta, or both, of 4.0 cm aortic dissection related to pregnancy may occur
2b C-EO to 4.5 cm, vaginal delivery with regional anes- without significant aortic dilation and after previous
thesia, expedited second stage, and assisted
delivery may be reasonable. aortic root replacement.1,11
5. In pregnant patients with syndromic and
3. In the absence of controlled trials, cesarean deliv-
nsHTAD, and a diameter of the aortic root, ery is often performed in women with Marfan syn-
2b C-EO
ascending aorta, or both, of 4.0 cm to 4.5 cm, drome and a significantly dilated aorta to allow for
cesarean delivery may be considered.
a planned delivery.2,9
4. There are no randomized trials of delivery methods
in women with aortopathy. When the aorta is not
Synopsis significantly dilated, vaginal delivery using methods
The risk of type A aortic dissection related to pregnancy to lessen hemodynamic stress, including regional
in Marfan syndrome is related to aortic root diameter, anesthesia and an expedited second stage and
with a low risk (∼1%) of aortic dissection at an aortic assisted delivery, is often performed.2,8,9 Coexistent
diameter <4.0 cm and much greater risk at aortic diam- lumbosacral dural ectasia, spine disease, or both in
eters >4.5 cm.1-3 Progressive aortic dilation and hyper- women with aortopathic conditions may complicate
tension also determine dissection risk.4-6 Complex and epidural anesthesia.13,14
shared decision-making is required when the aorta is 5. Cesarean delivery is often performed in women
between 4.0 cm and 4.5 cm in diameter, recognizing that with Marfan syndrome and aortic dilation of >4.0
although some series report low risk,2,7,8 aortic dissection cm.2,8 Among 27 women with Marfan syndrome
related to pregnancy at this diameter may occur.1 Modi- and aortic dilation, 21 of 27 women had a vaginal
fied World Health Organization classification on cardio- delivery. The cesarean delivery rate was 23.8% and
vascular risk places women with Marfan syndrome and 16.7% in women with diameter <4.0 cm and 4.0
moderate aortic dilation of 4.0 cm to 4.5 cm in modified cm to 4.5 cm, respectively.8
World Health Organization class III and those with aor-
tic diameter >4.5 cm in class IV.9 Because of increased
8.3. Surgery Before Pregnancy in Women With

risk of aortic dissection, pregnancy is avoided when the
aortic root diameter is >4.5 cm.1-3,9 Type B aortic dissec- Aortic Disease
tion is responsible for 20% to 40% of pregnancy-related Recommendations for Surgery Before Pregnancy in Women With Aortic
Disease
dissections in Marfan syndrome, often occurring without
aortic dilation1,6,8,10 and may occur after previous aortic COR LOE Recommendations
root replacement in Marfan syndrome and Loeys-Dietz 1. In patients with Marfan syndrome and an
1 C-LD aortic root diameter of >4.5 cm, aortic surgery
syndrome.11,12 Aortic dissection frequently occurs post- before pregnancy is recommended.1-4
partum, with heightened risk up to 12 weeks after deliv-
If the aortic root diameter is 4.0 cm to 4.5
ery.1 Patients at risk and their care teams should remain cm, aortic surgery before pregnancy may be
alert to signs and symptoms suggesting acute dissection. considered, especially if there are risk factors
2b C-LD
for aortic dissection (ie, rapid aortic growth of
≥0.3 cm/y or a family history of aortic dissec-
Recommendation-Specific Supportive Text tion).1,2,5-8
2. In patients with Loeys-Dietz syndrome
1. Very limited information exists about pregnancy- attributable to pathogenic variants in
related aortic risks in patients with chronic aortic 2a C-EO TGFB2 or TGFB3 and an aortic diameter of
≥4.5 cm, surgery before pregnancy is rea-
dissection. Because of concerns for aneurysmal
sonable.
enlargement, recurrent dissection and aortic rup-
If the Loeys-Dietz syndrome is attributable
ture, pregnancy is considered to be high risk in to pathogenic variants in TGFBR1, TGFBR2,
women with chronic aortic dissection. To allow opti- 2b C-EO or SMAD3, and the aortic diameter is ≥4.0
mal timing of delivery, elective cesarean delivery is cm, surgery before pregnancy may be con-
sidered.
usually performed in women with chronic aortic
dissection. 3. In patients with nsHTAD and an aortic diam-
1 C-EO eter of ≥4.5 cm, surgery before pregnancy is
2. Type A and type B aortic dissection related to preg- recommended.
nancy may occur in Marfan syndrome.1,2,6 Women If the aortic diameter is 4.0 cm to 4.4 cm,
with aortic root dilation >4.0 cm and, especially surgery before pregnancy may be considered,
2b C-EO
>4.5 cm, are at increased risk of type A aortic dis- depending on the molecular diagnosis, family
history, and aortic growth rate.
section during pregnancy and postpartum.1,3 Aortic

Recommendations for Surgery Before Pregnancy in Women With Aortic

Table 34. Prophylactic Aortic Surgery Before Pregnancy in
CLINICAL STATEMENTS
Disease (Continued) Women With Aortopathic Conditions

AND GUIDELINES
COR LOE Recommendations Surgical Threshold Before

Pregnancy* by Aortic
4. In patients with Turner syndrome and ASI of
Diameter (cm) or Aortic Size
1 C-LD ≥2.5 cm/m2, surgery before pregnancy is rec-
Condition Index (cm/m2)
ommended.9-11
Marfan syndrome >4.5 cm
5. In patients with a BAV (in the absence of
Turner syndrome or an HTAD) and an aortic Marfan syndrome with risk factors 4.0–4.5 cm
1 C-EO
diameter of ≥5.0 cm, surgery before preg- (rapid aortic growth of 0.3 cm/y;
nancy is recommended. family history of aortic dissection)
6. In patients with sporadic aortic root aneu- Loeys-Dietz syndrome (attributable 4.0 cm
rysms, ascending aortic aneurysms, or both to pathogenic variants in TGFBR1,
1 C-EO
and a diameter of ≥5.0 cm, surgery before TGFBR2, or SMAD3)
pregnancy is recommended.
Loeys-Dietz syndrome (attributable 4.5 cm
to pathogenic variants in TGFB2 or
TGFB3)
Synopsis Nonsyndromic heritable thoracic 4.5 cm†
The decision to proceed with operative intervention for aortic disease
an aortic root, ascending aortic aneurysm, or both in a Turner syndrome 2.5 cm/m2
woman contemplating pregnancy is complex and depends Bicuspid aortic valve 5.0 cm‡
on many factors. Considerations that inform this decision
*Shared decision-making is required to determine the surgical threshold be-
include the specific disorder, genetic variant, rate of aortic fore elective aortic root, ascending aortic surgery, or both and is informed by
growth, family history, and phenotype and include shared the condition, specific pathogenic variant, age, body size, aortic growth rate,
decision-making (Table 34). Specialists involved in this phenotype, and family history of aortic dissection, and surgery at smaller aortic
diameters may be considered depending on individual circumstances.
decision may include aortopathic specialists, cardiologists, †Aortic dissection related to pregnancy has occurred at small aortic diameters
medical geneticists, maternal fetal medicine specialists, in women with ACTA2 and MYLK pathogenic variants. Prophylactic aortic sur-
and aortic surgeons at experienced centers. The risks of gery before pregnancy at smaller aortic diameters may be reasonable in these
conditions and other nonsyndromic heritable thoracic aortic disease and may be
aortic surgery should be considered and although prophy- informed by the molecular diagnosis, family history, and aortic growth rate.
lactic aneurysm surgery will prevent proximal aortic dis- ‡Prophylactic aortic surgery may be considered at smaller aortic diameters
section, a risk remains of pregnancy-related dissection depending on body size, aortic growth rate, and family history.
Colors correspond to Class of Recommendations in Table 2.

distal to the aortic graft in HTAD, and this risk may be
higher in women with Loeys-Dietz syndrome attributable
to pathogenic variants in TGFBR1 and TGFBR2.12,13
pathogenic variant and the family history, rate of
aortic growth, extra-aortic phenotypic features,
Recommendation-Specific Supportive Text and involves shared decision-making. Patients with
1. Women with Marfan syndrome and aortic root TGFB2- and TGFB3-related Loeys-Dietz syndrome
dilation >40 mm and especially >4.5 cm are at may have a lower aortic dissection risk than those
increased risk of type A aortic dissection during with variants in TGFBR1, TGFBR2, or SMAD3.16,17
pregnancy and postpartum.1,2,6,7,12 Pregnancy in Women with aortic root diameters of >4.0 cm
small series of women with Marfan syndrome and are likely at increased risk for pregnancy-related
aortic diameters between 4.0 cm and 4.5 cm was aortic dissection based on data from women with
reported to be relatively safe in carefully monitored Marfan syndrome and the more severe aortopathy
women,1-4 although acute type A aortic dissection in Loeys-Dietz syndrome attributable to TGFBR1,
may occur.5 The presence of additional risk factors TGFBR2, and SMAD3 variants.5,18-20 There were no
for aortic dissection, including family history of aor- pregnancy-related aortic dissection reported in a
tic dissection and rapid aortic growth (≥0.3 cm/y), series of women with SMAD3 variants, but only 2
and patient preference may inform the shared women had aortic diameters known before preg-
decision for aortic surgery before pregnancy when nancy (and both were normal).20
the aortic diameter is <4.5 cm.8,14,15 3. Because phenotypic features are absent in
2. Information is lacking about aortic diameters patients with nsHTAD because of pathogenic vari-
and aortic dissection risk related to pregnancy in ants in multiple genes (eg, ACTA2, MYH11, MYLK,
Loeys-Dietz syndrome, because most women who PRKG1, and others), the first manifestation of dis-
were pregnant were unaware of their diagnosis ease may be acute aortic dissection, including that
before pregnancy. The size threshold for elec- related to pregnancy.21 In a series of patients with
tive surgery to replace the dilated aortic root and ACTA2 pathogenic variants, 20% of aortic dissec-
ascending aorta in Loeys-Dietz syndrome depends tion were related to pregnancy.21 Aortic dissection
on multiple factors and is informed by the specific at small aortic diameters has been reported related

to pregnancy in patients with ACTA2- and MYLK- and other factors involving a shared decision
CLINICAL STATEMENTS
related HTAD.21,22 Ruptured type B dissection has depending on individual circumstances.
AND GUIDELINES
been reported.23 Individualized assessment of preg-
nancy risks based on the specific genetic condition 8.4. Pregnancy in Patients With Aortopathy:
and other individual factors may inform pregnancy
management, recognizing that limited information Aortic Dissection and Aortic Surgery in
is available to guide decision-making.21,22,24 Pregnancy
4. Among those with Turner syndrome, an ASI Recommendations for Pregnancy in Patients With Aortopathy: Aortic
Dissection and Aortic Surgery in Pregnancy
>2.0 cm/m2 is considered dilated, and the risk
of aortic dissection in Turner syndrome is great- COR LOE Recommendations
est when the ASI is ≥2.5 cm/m2.9,10 When aortic 1. In patients experiencing an acute type A aortic
dissection occurs in Turner syndrome, almost 90% dissection during the first or second trimester
1 C-LD
of pregnancy, urgent aortic surgery with fetal
of cases have an identifiable risk factor, such as monitoring is recommended.1-3
underlying aortic dilation, aortic coarctation, BAV, 2. In patients experiencing an acute type A
or hypertension.11 aortic dissection during the third trimester of
5. Despite the relative frequency of BAV in the popu- 1 C-LD pregnancy, urgent cesarean delivery imme-
diately followed by aortic surgery is recom-
lation, aortic dissection related to pregnancy in mended.1-4
patients with a BAV (and without Turner syndrome
3. In patients experiencing an acute type B
or HTAD) is rarely reported.5,25 In 88 women with aortic dissection during pregnancy, medical
BAV and without aortic dilation, there were no 1 C-EO therapy is recommended, unless endovascular
cases of aortic dissection in 186 deliveries.26 In a or surgical therapy is required to manage
acute complications.5
series of 49 patients with BAV with moderate aortic
dilation (median aortic diameter 42 mm) reporting 4. In patients with progressive aortic dilation
during pregnancy, prophylactic aortic surgery
pregnancy outcomes, there were no cases of aor- 2b C-EO
may be considered, depending on individual
tic dissection.3 When type A aortic dissection did circumstances.1,2,4
complicate pregnancy in isolated BAV, significant
aortic dilation was noted.5,25 There is no evidence-
based information regarding pregnancy outcomes Synopsis
in women with BAV and aortic diameters >4.5 cm
During pregnancy, if marked aortic dilation is present

to inform aortic risk. In these cases, pregnancy or rapid aortic expansion occurs, risks of maternal aor-
management and shared decisions about aortic tic dissection or rupture must be considered. If early in
surgery may be informed by other risk factors for pregnancy, high maternal risk of morbidity or death may
dissection, including rapid aortic growth, body size, warrant pregnancy termination.1,4 Prophylactic aortic sur-
and family history. Aortic dissection risk increases gery during pregnancy requires complex decision-making
in patients with BAV when the aortic diameter and should be individualized based on maternal and fetal
exceeds 5 cm.27 Because of risk of aortic dissec- risks.1,2,4 Cardiac surgery in the first trimester has risks of
tion, pregnancy in patients with a BAV and an aor- fetal developmental defects, while surgery in the third tri-
tic diameter of >5.0 cm is classified to be modified mester carries risks to fetal circulation and maternal hemo-
World Health Organization class IV, carrying high dynamics.1 Semi-elective surgery during pregnancy may
risk of maternal morbidity and mortality.28 have its lowest collective risk to fetal organogenesis and
6. Aortic root and or ascending aortic dilation >4.0 cm maternal hemodynamics during the second trimester.1,3,4 If
in a woman of child-bearing age is uncommon, and type A aortic dissection occurs during pregnancy, urgent
its presence should trigger an evaluation for under- obstetric and cardiac surgical consultation is necessary,
lying genetic aortopathy.29 Even when there is clear because management strategies depend on the viability
evidence of an autosomal dominant transmission of the fetus and condition of the mother. If type A aortic
of TAA in a family, currently available molecular dissection occurs in the first 26 weeks, emergency car-
genetic technology only identifies a pathogenic diac surgery is performed, recognizing risk of fetal loss.1,2,4
variant in a known gene leading to TAA in about When duration of pregnancy associates with higher like-
20% to 25% of families.29 In sporadic TAA disease, lihood of independent fetal survival (especially after 28
genetic variants are found in even fewer cases. In weeks), cesarean delivery followed by aortic repair pro-
young patients at low surgical risk with aortic root vides the best chances for fetal and maternal survival.1,2,4
or ascending aortic aneurysms of 5.0 cm, surgical
intervention is performed. Surgery before preg-
nancy at smaller aortic diameters is sometimes Recommendation-Specific Supportive Text
performed and is informed by aortic growth rate, 1. If type A aortic dissection occurs during the first 2
hypertension, surgical expertise, patient wishes, trimesters, emergency aortic surgery is performed

first with fetal monitoring and modifications to Recommendations for Inflammatory Aortitis: Diagnosis and Treatment
CLINICAL STATEMENTS
anesthesia and cardiopulmonary bypass, recogniz- of Takayasu Arteritis and GCA (Continued)
AND GUIDELINES
ing the high risk of fetal loss.1-4 If acute type A aor- COR LOE Recommendations
tic dissection occurs between 24 and 28 weeks, 5. In patients with active GCA or Takayasu
the care team must balance maternal and fetal arteritis, treatment efficacy should be
risks when deciding between cesarean delivery fol- periodically assessed by monitoring inflam-
1 C-LD matory serum markers (C-reactive protein
lowed by aortic surgery or aortic surgery with fetal and erythrocyte sedimentation rate), imag-
surveillance.1,4 ing with CT, MRI, or FDG-PET, and clinical
2. If type A aortic dissection occurs in the third tri- symptoms.1,7,15,17-20
mester, given the increased likelihood of inde- 6. In patients with GCA or Takayasu arteritis
who are in remission, elective endovascular
pendent fetal survival, emergency cesarean 2a C-LD or open surgical intervention is reasonable
delivery followed by maternal aortic surgery is rec- to treat aortic and branch vessel complica-
ommended.1,2,4 In a series of 20 patients with type tions.7,21
A aortic dissection during pregnancy, 19 under- 7. In patients with GCA or Takayasu arteritis
went surgical repair and, of those at >28 weeks 2a C-EO
and aortic involvement who are in remission,
annual surveillance imaging with CT, MRI, or
gestation, delivery first followed by aortic surgery FDG-PET is reasonable.1,7,17-19
achieved good fetal outcomes.2
3. Although uncomplicated type B aortic dissection in
pregnancy is treated medically, 20% will go on to Synopsis
develop complications that require intervention5; in
LVV comprises Takayasu arteritis and GCA, which are the
such cases, endovascular repair is preferred over
most common causes of aortitis.22,23 Other known causes
open surgery, whenever feasible.5
of aortitis include immunoglobulin G4–related disease,
4. Prophylactic aortic surgery during pregnancy
antineutrophil cytoplasmic antibody-related vasculitis,
requires complex decision-making, and manage-
sarcoidosis, Behçet’s disease, relapsing polychondritis,
ment is individualized based on maternal and fetal
and granulomatosis with polyangiitis; many cases of aor-
risks and benefits.1,2,4
titis remain idiopathic. Whereas Takayasu arteritis and
GCA tend to affect the thoracic aorta, immunoglobulin
G4–related disease most commonly affects the abdomi-
9. OTHER AORTIC CONDITIONS
nal aorta. Diagnostic criteria are summarized in Table 35.

9.1. Inflammatory Aortitis: Diagnosis and Prompt diagnosis and initiation of treatment is of utmost
Treatment of Takayasu Arteritis and Giant Cell importance, because potential complications include
Arteritis (GCA) aortic aneurysms, aortic dissection, IMH, PAU, and rup-
ture, as well as progressive atherosclerosis and throm-
Recommendations for Inflammatory Aortitis: Diagnosis and Treatment
of Takayasu Arteritis and GCA botic complications.24 18F-FDG-PET with CT is useful
Referenced studies that support the recommendations are for both the diagnosis of suspected LVV and to evaluate
summarized in the Online Data Supplement. anti-inflammatory treatment response, especially before
COR LOE Recommendations planned revascularization.4,5 Initial treatment options for
Diagnosis Takayasu arteritis and GCA include high-dose glucocor-
1. In patients with large vessel vasculitis ticoid therapy (prednisone at 40–60 mg/d, or equivalent)
(LVV), prompt evaluation of the entire or, for select cases, intravenous pulse steroids along with
aorta and branch vessels with MRI or adjunctive therapy, including (but not limited to) tocili-
1 C-LD
CT, with or without 18F-FDG positron
emission tomography (FDG-PET), is zumab and methotrexate (Figures 25 and 26). Revas-
r ecommended. 1-6 cularization may be warranted in select cases of stable
Treatment disease, as well as in AAS.
2. In patients with active GCA or Takayasu arteri-
1 B-NR tis, initial medical therapy should include high-
dose glucocorticoids.7-12 Recommendation-Specific Supportive Text
3. In patients with GCA who have evidence of 1. In suspected GCA or Takayasu arteritis, early imag-
active aortitis, tocilizumab is recommended ing can confirm the diagnosis when the results com-
1 B-R
as adjunctive therapy to glucocorticoids, with
methotrexate as an alternative.7,13,14 plement clinical findings.1 Imaging the aorta should
be performed as soon as possible so that initiation
4. In all patients with Takayasu arteritis, non-
biological disease-modifying anti-rheumatic of treatment is not delayed, given the risk of compli-
1 C-LD
drugs (DMARD) should be given in combina- cations from untreated LVV. Sensitivity of diagnostic
tion with glucocorticoids.7,15,16
imaging in the initial diagnosis of LVV decreases

Table 35. Diagnostic Criteria for Inflammatory Aortitis
CLINICAL STATEMENTS
Names Criteria Used in Diagnosis When Is Diagnosis Established?
AND GUIDELINES
Takayasu arteritis Age of onset <40 y 3 criteria are present (sensitivity 90.5%; specificity 97.8%)
Intermittent claudication
Diminished brachial artery pulse
Subclavian artery or aortic bruit
Systolic BP variation of >10 mm Hg between arms
Aortographic evidence of aorta or aortic branch stenosis
Giant cell arteritis Age >50 y 3 criteria are present (sensitivity >90%; specificity >90%)
Recent-onset localized headache
Temporal artery tenderness or pulse attenuation
Elevated erythrocyte sedimentation rate >50 mm/h
Arterial biopsy shows necrotizing vasculitis
Reprinted from Hiratzka et al. 2019.27
with duration of glucocorticoid treatment.2 FDG- of prophylactic antithrombotic therapy in all patients
PET has a reported specificity for GCA-related aor- with GCA25; instead, an individualized approach
titis as high as 100% and CTA about 85%.5 In CT, to antithrombotic therapy is recommended in the
wall thickening from inflammation may be mistaken acute and chronic phases of GCA and Takayasu
for atherosclerosis; however, given CT’s usefulness arteritis, based on imaging and clinical findings of
in assessing occlusive lesions, intimal injury, ulcer- aortic and branch vessel complications.26
ative plaques, and aneurysmal disease, it is often 3. In an RCT of 251 patients with GCA, a 26-week
combined with FDG-PET in LVV.1 Evidence is limited prednisone taper combined with tocilizumab, an
for the role of MRI in GCA, but MRI is widely used interleukin-6 receptor inhibitor, was superior to
in Takayasu arteritis given the patients’ younger age either a 26-week or 52-week prednisone taper
at diagnosis and need for lifelong surveillance imag- plus placebo in reducing the primary outcome of
ing.6 If proximal aortic involvement is confirmed by glucocorticoid-free disease remission at 1 year.10
CT or MRI, then echocardiography may be helpful to Tocilizumab gained approval for use in 2017
assess aortic valve function. as adjunctive therapy for select patients with
2. Active vasculitis is diagnosed by clinical symp- GCA, with methotrexate remaining an alternative
toms of GCA or Takayasu arteritis with evidence option.7,13,14 The EULAR 2018 updated guidelines
of inflammation by serum biomarkers, imaging, or recommended limiting the use of adjunctive ther-
both. High-dose glucocorticoid therapy (prednisone apy to those with refractory or relapsing disease,
at 40–60 mg/d or equivalent) is standard induction those at risk of adverse effects of glucocorticoid
therapy for GCA and Takayasu arteritis and leads treatment, or those at risk of cardiovascular com-
to remission and control of active disease in most plications (aortitis and major branch vessel involve-
patients7-12 (Figures 25 and 26). Evidence support- ment) from GCA7 (Figure 25).
ing the efficacy of induction therapy with high-dose 4. High-quality randomized clinical trial evidence sup-
intravenous methylprednisolone in GCA comes porting the use of adjunctive therapy in Takayasu
only from small clinical trials, and thus the 2018 arteritis is limited. However, consensus expert
recommendations from the European Alliance of opinion is to initiate DMARDs in combination
Associations for Rheumatology (EULAR; formerly with glucocorticoids in all patients with Takayasu
the European League Against Rheumatism) limits arteritis, given high relapse rates of up to 70%.7
its use to patients with severe GCA at risk for blind- Nonbiological DMARDs (eg, methotrexate,
ness in the acute setting, and administration should hydroxychloroquine, azathioprine, sulfamethoxa-
not delay oral glucocorticoid treatment.7-9 Once zole, and leflunomide) are considered first line
the acute phase is controlled, glucocorticoid taper according to the EULAR 2018 updated guide-
should be initiated to reach a target prednisone lines on Takayasu arteritis treatment, with biologi-
dose of 15 to 20 mg/d within 2 to 3 months, and cal DMARDs (eg, tocilizumab or tumor necrosis
≤5 mg/d for GCA and ≤10 mg/d for Takayasu arte- factor-inhibitors) as second-line agents in select
ritis after 1 year.7 Older guidelines have supported patients who relapse on initial combination ther-
the use of antiplatelet or anticoagulants in LVV. apy7,15,16 (Figure 26). Optimal treatment duration in
Evidence from a meta-analysis does not support use Takayasu arteritis is less well understood, because

CLINICAL STATEMENTS
AND GUIDELINES
Figure 25. The 2018 European Alliance of Associations for Rheumatology (EULAR; formerly European League Against
Rheumatism) Recommended Algorithms for the Pharmacological Treatment of Giant Cell Arteritis.
GC indicates glucocorticoids; GCA, giant cell arteritis; and TNF, tumor necrosis factor. Modified from Hellmich et al.7 Copyright 2020, with
permission from BMJ Publishing Group Limited.
defining remission in Takayasu arteritis is challeng- therapies and a consensus definition of treatment
ing. Outcomes measures may include any of these: success.
remission based on clinical criteria, normalization of 5. The EULAR 2018 updated guidelines placed the
inflammatory biomarkers, stabilization on serial CT greatest emphasis on both the improvement of
or MRI, improvement on PET-CT imaging, quality clinical symptoms and the stability of inflammatory
of life, and presence of clinical disease relapse.15 biomarkers in defining the remission phase of LVV.
A clear need remains for both adequately pow- Consequently, data are limited regarding the role
ered randomized clinical trials of Takayasu arteritis of surveillance imaging in those with no signs or

CLINICAL STATEMENTS
AND GUIDELINES
Figure 26. The 2018 European Alliance of Associations for Rheumatology (EULAR; formerly European League Against
Rheumatism) Recommended Algorithms for the Pharmacological Treatment of Takayasu Arteritis.
csDMARD indicates conventional synthetic disease modifying antirheumatic drug; GC, glucocorticoids; and TNF, tumor necrosis factor. Modified
from Hellmich et al.7 Copyright 2020, with permission from BMJ Publishing Group Limited.
symptoms of active disease. Currently, tomographic activity at 3 months after treatment initiation but no
imaging is complementary to clinical symptoms and further change at 6 months, with most patients in
laboratory surveillance, and its use should be indi- clinical remission still showing positive PET find-
vidualized, focused mostly on the evaluation of new ings.20 What remains unknown are the potential
symptoms or signs of aortic, major branch artery anatomic consequences of having a positive FDG-
stenoses or aneurysms, or both.1,7,15,17-19 One pro- PET scan despite clinical remission.
spective cohort study using FDG-PET in disease 6. In patients with LVV who are in remission and have
surveillance of GCA showed reduced inflammatory aortic or branch artery complications that do not

warrant urgent intervention, the role of elective endo- Synopsis

CLINICAL STATEMENTS
vascular or open surgical repair approach should be

The term “infectious aortitis” describes an infection of
AND GUIDELINES
determined by a multidisciplinary team including, but

the aorta and has supplanted the older term “mycotic
not limited to, vascular surgery, vascular medicine,
aneurysm,” which was used broadly but actually implies a
cardiology, and radiology specialists. The risk of such
fungal cause. Aortic infections arise from either contigu-
elective intervention is lowest when patients are in
ous spread from adjacent structures or septic emboli and
the remission phase of the LVV7; therefore, before
hematogenous spread of microorganisms to the aortic
intervention, imaging with 18F-FDG-PET CT is often
wall via a vulnerable plaque or preexisting aneurysm.4
helpful to assess treatment response and quantify
Staphylococcus aureus, Pneumococcus, Escherichia coli,
the degree of ongoing active inflammation.1,4,5,18
and Salmonella are the pathogens identified in most
7. The EULAR consensus definitions for relapse and
reports.1-6 Syphilitic aortitis, which typically appears 10
remission have been incorporated into the 2018
to 25 years after systemic Treponema pallidum infection,
updated recommendations for management of
is now rare. Fungal aortitis (from Candida or Aspergil-
LVV.7 A major relapse of GCA and Takayasu arteritis
lus) and tuberculous aortitis are uncommon and typically
includes recurrence of clinical features of ischemia
arise in immunocompromised hosts.
(ie, visual loss, jaw claudication, limb claudication,
Medical therapy is challenging because the caus-
stroke) or evidence of active aortic inflammation
ative organism is not always identified, but a prolonged
resulting in branch vessel stenosis, aortic aneurysm,
course of antibiotics is often warranted.4 The mortal-
or dissection. Remission of LVV is characterized by
ity rate of infectious aortitis is high, because complica-
lack of new clinical symptoms, a normalization of
tions include sepsis, aneurysm formation (saccular or
inflammatory biomarkers, and no evidence of pro-
pseudoaneurysm), erosion and subsequent fistula, dis-
gressive aortic and branch artery dilation or nar-
section, or rupture. CT and MRI can size the aneurysm,
rowing by surveillance imaging. However, signals
detect complications, and aid in interventional plan-
of vessel inflammation may persist even in the
ning. TEE is especially useful for imaging involvement
absence of clinical disease.1,6,19 For those in remis-
of the aortic root and associated complications.5 Open
sion, annual surveillance imaging with CT or MRI is
surgical repair is the standard treatment for infectious
useful to detect disease progression in the aortic
aortitis; however, in select patients with rupture, fistula,
and branch arteries, even in the absence of inflam-
hemodynamic instability, or both, a hybrid or bridging
mation. More frequent surveillance imaging may be
approach with endovascular therapy (Table 36) may be

necessary when evidence of active disease pro-
used.2-8
gression is apparent on annual imaging or if new
symptoms suggestive of arterial stenosis arise.
9.2. Infectious Aortitis 1. A diagnosis of infectious aortitis or mycotic aneu-
rysm and its complications warrants prolonged
9.2.1. Diagnosis and Management of Infection of antimicrobial therapy regardless of intervention,
the Native Aorta with 2016 scientific statement from the AHA
Recommendations for Diagnosis and Management of Infection of the
suggesting a duration of 6 weeks to 6 months,
Native Aorta with consideration of lifelong suppressive therapy
in some cases.5 Given the high risk of rupture or
contained rupture in infectious aortitis, open sur-
1. In patients with infectious aortitis and associ-
ated aneurysms or dissection of the thoracic gical repair is often warranted, although the data
1 C-EO
or abdominal aorta, open surgical repair is supporting open surgical repair are limited, with
recommended. most evidence derived from single-institution
2b C-LD
In select patients, treatment with endovascular case series and small cohort studies.6-8 Open
repair may be considered.1-3
surgical repair includes in situ reconstruction or
2. In patients with infectious aortitis compli- aortic resection with extra-anatomic bypass (ie,
cated by rupture, either open or endovas-
2a C-EO cular repair is reasonable, based on the axillobifemoral bypass or femorofemoral cross-
patient’s status at presentation and institu- over bypass graft placement)3; surgical debride-
tional expertise. ment of all infected tissue is essential to minimize
3. In patients with infectious aortitis, intravenous the risk of persistent infection. The use of endo-
antimicrobial therapy of at least 6 weeks’
vascular repair has been increasing in select
duration may be considered, with lifelong sup-
2b C-EO
pressive therapy in select cases not amenable patients with infectious aortitis.6-8 Limited data are
to interventional repair or who have recurrent available for comparison of open surgical versus
infection.
endovascular repair; some small studies showed

Table 36. Management of Aortic Mycotic Aneurysm: Comparison of Resection and Extra-Anatomic Reconstruction, In Situ
CLINICAL STATEMENTS
Reconstruction, or Endovascular Device Repair
AND GUIDELINES
Procedure Potential Indications* Advantages Disadvantages
Extra-anatomic Infrarenal location with gross puru- Avoids placement of foreign body in Not technically feasible for thoracic, suprarenal,
reconstruction lence, psoas or retroperitoneal ab- infected area or visceral location or for emergency use
scess, vertebral osteomyelitis, inade- Long operating time
quate response to antibiotic therapy,
selected aortoenteric fistulae Long-term patency rates low Stump blowout
Limb ischemia, amputation
Reinfection rate higher than for in situ recon-
struction
Ischemic colitis
In situ reconstruction Thoracic, suprarenal, infrarenal, or More versatile than extra-anatomic: Theoretical risk of infection because of interposi-
visceral location fewer long-term complications, higher tion of foreign material in infected site
Selected aortoenteric fistulae patency rates, lower recurrent infection
rate, shorter operating time
Polyester grafts† available for emer-
gency surgery
Endovascular device Bridge procedure‡: hemodynamic Emergency stabilization Persistent infections and device infections
repair instability, uncontrolled bleeding, Low early morbidity, mortality Less Higher long-term morbidity, mortality with device
rupture or impending rupture, se- invasive retention
lected patients with aortocentric
fistulae, patients who are not fit for No cross-clamping of aorta: spinal cord Requires device explanation, reconstruction
open surgery injury, reperfusion injury
*Potential indication; must be individualized for each patient.

†Polyester grafts, rifampin-soaked or silver-coated; less experience reported with cryopreserved arterial allografts or venous autografts.
‡Bridge procedure, used to stabilize patients until device explanation and arterial reconstruction.
Adapted from Wilson et al5 with permission of the American Heart Association, Inc. Copyright 2016 American Heart Association, Inc.
similar long-term survival between the 2 methods intervention is based on multiple factors (Table
in treatment of infectious abdominal aortitis,1-4 36), including the location and extension of the
although the evidence may have selection bias. aneurysm(s), the presence of fistulae, and the
In a nationwide Swedish retrospective population- patient’s clinical status. In select patients with
based cohort study of 132 patients, of whom 50 aneurysm rupture and hemodynamic instabil-
(38%) presented with rupture, using propen- ity and/or uncontrolled bleeding, endovascular
sity score analyses, 5-year survival was similar repair may be used.6
with open repair versus EVAR, at 60% versus 3. Because peripheral blood cultures and surgical
58%, respectively.3 Moreover, the use of EVAR specimen cultures may be negative in a large
was associated with improved short-term sur- proportion of patients with infectious aortitis,5 the
vival and was not associated with an increase in choice of antimicrobial agents may be empiric,
infection-related complications or a need for late and infectious disease experts are usually
reoperation.3 Use of endovascular repair in the involved in directing therapy. Treatment with anti-
management of infectious thoracic aneurysms, microbial therapy alone (ie, without intervention)
abdominal aneurysms, or both warrants ongoing is associated with high mortality rate and may not
study, and at present may be most appropriate prevent aneurysm expansion or rupture6,9,10 and
as a bridge procedure in cases of instability or is thus reserved for patients who are not can-
impending rupture, or in patients who may not be didates for open or endovascular repair or for
fit for open surgical intervention5 (Table 36). those in whom a palliative approach is appropri-
2. The prognosis is often poor for infectious aor- ate. No clinical trial data are available to define
titis, especially if rupture has occurred.6 From the optimal duration of antimicrobial therapy,
a large single-institution study over 18 years whether as solo therapy or as adjunctive therapy
of 2520 patients who underwent surgery for to aortic intervention, but expert opinion suggests
infectious aortic aneurysms, 24% of aneurysms a duration of at least 6 weeks, and possibly lon-
had already ruptured at presentation, and 61% ger.5,11 Because the response of uncomplicated
had penetrated into periaortic tissues.6 Open (without rupture or fistulae) infectious aortitis to
surgical treatment options include resection antimicrobial therapy may influence the choice
of infected aorta with extra-anatomic recon- of interventional approach, it is also reasonable
struction (for abdominal aneurysm), or in situ to have patients undergo surveillance imaging
reconstruction (for thoracic aneurysms and at intervals deemed appropriate by a multidisci-
some aortoenteric fistulae).2-5,7,8 The choice of plinary care team.

9.2.2. Diagnosis and Management of Prosthetic with endovascular versus open repair, the EVAR-1 (UK
CLINICAL STATEMENTS
Aortic Graft Infection Endovascular Aneurysm Repair 1) RCT and a large

AND GUIDELINES
Recommendations for Diagnosis and Management of Prosthetic Aortic Medicare analysis found equivalent rates of graft infec-
Graft Infection tion.23-25 Common sources of infection include: contami-
fmkReferenced studies that support the recommendations are
nation at the time of implantation; graft enteric erosion or
fistula to adjacent bowel, esophagus, or airway; or, rarely,
hematogenous spread from remote infection. Suspicion
Diagnosis is usually raised by symptoms, laboratory test abnormali-
1. In patients with a prosthetic aortic graft, ties, or axial imaging findings. In the presence of an aor-
who have signs and symptoms or culture
tic graft infection, no surgical option is clearly superior.
evidence of unexplained infection or have
2a B-NR unexplained gastrointestinal bleeding, Basic tenets are to remove all infected tissue, includ-
cross-sectional imaging is reasonable ing the graft and surrounding tissue, reconstruction of
to evaluate for an underlying aortic graft
distal flow either as an extra-anatomic or in situ bypass,
infection.1-6
and coverage of the contaminated field with omen-
Treatment
tum, muscle flaps, or pleura. Previously, extra-anatomic
2. In patients with an infected prosthetic aor- bypass followed 24 to 48 hours later by graft explant and
tic graft who are hemodynamically stable
and have appropriate anatomy, it is reason- oversewing of the aortic stump was considered the gold
2a B-NR
able to perform open surgery with either standard for abdominal aortic infection but is usually not
in situ reconstruction or extra-anatomic appropriate for the thoracic aorta. Aortic allografts, deep
bypass. 7-13
vein, and silver-impregnated or rifampin-soaked pros-
3. In patients with an infected prosthetic
aortic graft who are hemodynamically
thetic grafts placed in situ have all shown good results
2a B-NR unstable, it is reasonable to perform as well, often with lower complication rates. A 6-week
open surgery with either explant or in situ course of intravenous antibiotics is typically used, some-
r econstruction. 7
times followed by long-term oral suppressive therapy.
4. In patients with an infected prosthetic aortic
graft, endovascular therapy is reasonable,
either as bridge therapy in those with hemo-
2a C-LD
dynamic instability or as long-term therapy in Recommendation-Specific Supportive Text
those who are unsuitable candidates for open
1. Early graft infection (≤3 mo) is often associated
surgery.13-15
with fever and back pain, whereas late graft infec-
Late Management
tions (>3 mo) may have an insidious onset with
5. In patients who have undergone treatment symptoms of fatigue and malaise, or may have
of an acute prosthetic aortic graft infection,
targeted intravenous antimicrobial therapy fever, an elevated white blood cell count, eryth-
1 C-LD
of at least 6 weeks’ duration, with prolonged rocyte sedimentation rate, C-reactive protein, or
suppressive oral therapy in select cases, advanced signs of sepsis with hemodynamic insta-
plus a consultation and follow-up with an
infectious disease specialist, is recommen bility or frank hemorrhage from rupture or fistulae
ded.7,11,12,16,17 to adjacent bowel, esophagus, or airway. Because
6. In patients with an infected prosthetic aor- these signs and symptoms are nonspecific for site
tic graft and either an extensive perigraft of infection, the initial workup should include basic
abscess or an infection caused by methicillin-
resistant S. aureus, Pseudomonas aeruginosa,
blood work, blood cultures, and axial imaging, pref-
2b C-LD or a multidrug-resistant microorganism, or erably with CTA. In those patients with bleeding,
who have undergone in situ reconstruction, endoscopy may be used to rule out other causes
lifelong suppressive oral antimicrobial therapy
may be considered after the initial course of
and potentially temporize bleeding. Findings of
therapy.14,15,18,19 graft infection on CT include peri-graft air, abscess,
inflammatory changes, pseudoaneurysms, or frank
hemorrhage. CTA has a sensitivity of 94% and
Synopsis specificity of 85% to 100% with advanced graft
Recommendations in this section apply to prosthetic infection, but the sensitivity is only 64% for those
aortic grafts. This includes tube grafts placed via open with low-grade infection.1,2 The sensitivity and
surgery as well as endovascular stent grafts. Although specificity for low-grade infection may be increased
these grafts are typically made with Dacron or polytet- from 77% to 93% and 70% to 89%, respectively,
rafluoroethylene, these recommendations also apply to with the use of PET-CT.3-5 MRI, tagged white blood
allografts (eg, cryopreserved aorta) and autografts (eg, cell scans, or both may also be useful, depending
femoral vein). on local expertise and availability.6
Aortic graft infection is uncommon (0.3%–3%).20-22 2. Extra-anatomic bypass with subsequent graft
Extension to the groin increases the risk of subsequent explant, aortic stump oversewing, and omental cov-
infection. Although some studies suggest a lower risk erage has a reasonably low rate of reinfection but a

relatively high rate of amputation and occlusion and 9.3. Atherosclerotic Disease
CLINICAL STATEMENTS
is susceptible to stump blow-out.7,8 In situ venous Recommendations for Atherosclerotic Disease
AND GUIDELINES
reconstruction has the lowest rate of reinfection
but is associated with long operative times, size
1. In patients with aortic atherosclerotic
mismatch, and lower extremity venous morbidity.7,9
disease and concomitant coronary artery
Cryopreserved allografts have a low rate of rein- disease, PAD or both, it is recommended to
1 C-LD
fection (similar to vein) but are susceptible to early prescribe antiplatelet therapy, anticoagu-
lant therapy or both, guided by the clinical
and late degeneration, may have limited lengths
setting.1-3
and diameters, and have limited availability for
2. In patients with aortic atherosclerotic
emergencies.7,10,11 Rifampin- or silver-impregnated disease and risk factors for confirmed
prosthetic grafts are more readily available and 2a C-LD coronary artery disease, it is reasonable
faster to implant than vein or extra-anatomic repair to prescribe a moderate- or high-intensity
statin.4-6
but are more susceptible to reinfection.7,26 None of
these graft options is clearly superior to the others 3. In patients with aortic atheromas of a thick-
2b C-LD ness ≥4 mm, statin therapy may be reason-
and, as such, in the stable patient without extensive able.1,7-9
infection with resistant organisms, the use of any
of these is acceptable.27 For those with extensive
peri-graft abscess, or infection with methicillin- Synopsis
resistant S. aureus, Pseudomonas, or multidrug Atherosclerosis is a chronic immunoinflammatory, fibro-
resistant organisms, extra-anatomic reconstruction proliferative disease of the aorta and its branches that
(when feasible) or in situ reconstruction with femo- is propagated by lipids.10 This disease process has mul-
ral vein or allograft may offer improved freedom tiple risk factors and begins early in life so that the aorta
from reinfection.7,13,26 may develop extensive disease over many decades.11
3. Hemodynamically unstable patients require emer- The diagnosis of aortic atherosclerosis may occur inci-
gency proximal control with a clamp or balloon, and dentally, during the evaluation of symptomatic vascular
rapid in-line reconstruction, which is best performed events, or both. The size and location of aortic plaques
with either an allograft (if immediately available) or have been associated with embolic complications.4,7,8,12-16
a silver- or rifampin-impregnated prosthetic graft.7 The presence of aortic atheromas has been significantly
4. Endovascular intervention allows relatively rapid
associated with all-cause death.9 The management of

control of hemorrhage and may improve survival aortic atherosclerosis includes, in general, control of risk
in patients with an aorto-enteric fistula, when used factors, lifestyle modification, and appropriate pharmaco-
as a bridge to definitive therapy.13-15 For patients logical therapies. Although lifestyle changes may be the
who are not candidates for surgical graft excision, most important treatment strategy, compliance may be
endovascular therapy may be considered for defin- challenging.17,18
itive therapy, in which case lifelong antibiotic sup-
pression should be considered.
5. Consultation with an infectious disease specialist Recommendation-Specific Supporting Text
is recommended for all patients with aortic graft 1. Patients with aortic atherosclerosis often have con-
infection. A 6-week course of intravenous antimi- comitant cardiovascular diseases such as coronary
crobial therapy has been recommended in multiple artery disease, atrial fibrillation, and PAD. These
reports from high-volume centers and in scientific concomitant conditions frequently determine the
statements.7,11,16,17,26,28 For Pseudomonas or mul- selection of guideline-based antiplatelet agents,
tidrug resistant organisms, multiple antimicrobial anticoagulant agents, or both.1-3
agents may be needed. A subsequent course of 2. The indications for statin therapy in patients with
oral antimicrobial therapy for 3 to 6 months may a history of coronary artery disease, myocardial
be considered depending on the specific organism, infarction, and stroke are well established.5,6 The
the extent of infection, and the type of repair. data for statin therapy specific to aortic atheroscle-
6. Lifelong suppressive oral antimicrobial therapy rosis alone are very limited. Therefore, this recom-
has been suggested for selected patients, such as mendation has been made for those patients at
those with extensive infection, aggressive organ- risk for or with confirmed coronary artery disease
isms, in situ prosthetic replacement, or endovas- because the available data best support statin
cular coverage without resection.14,15,18,19 Axial therapy in this cohort.
imaging is typically continued long term to iden- 3. Atherosclerotic disease of the aortic arch is a
tify evidence of reinfection, such as inflammatory potential source of emboli to the brain.1,2,9 A pro-
changes, fluid or air collections, or pseudoaneu- spective study (N=500) showed that the OR for
rysm formation. stroke among patients with aortic atheromatous

plaques (atheromas) of ≥4 mm versus controls Treatment in acute presentations is typically surgical,

CLINICAL STATEMENTS
was 9.1 (95% CI, 3.3–25.2; P<0.001).7 Moreover, including open embolectomy in the setting of embolus or
AND GUIDELINES
in a clinical trial of 519 patients with severe tho- aorto-iliac and femoral reconstruction for atherosclerotic
racic aortic plaques, multivariate analysis showed occlusion.1 Chronic aortic occlusion can occasionally be
that statin therapy was protective against strokes asymptomatic because collateral circulation has devel-
(P=0.0001).8 (The data from these 2 studies oped, in which case intervention may not be required.
relate specifically to atheroma thickness of ≥4 More commonly, patients with chronic aortic occlusion
mm, which does not align precisely with the most present with lower extremity claudication that may be
commonly used grading systems for severity of accompanied by buttock claudication, central core mus-
aortic atherosclerosis, which define severe ath- cle weakness, and impotence in males caused by pelvic
eromas by a thickness of >5 mm.) Although anti- malperfusion. These patients often have cardiopulmonary
platelet therapy is commonly used in patients with comorbidities and multifocal atherosclerotic disease, and
aortic atheromas, there is no evidence to support these issues should be addressed preoperatively to miti-
the use of prophylactic anticoagulation in this gate potential complications.
population. Revascularization options include endovascular,2
open aortic (eg, aortobifemoral bypass),3 or extra-ana-
9.3.1. Aortic Thrombus
tomic (eg, axillofemoral bypass), and hybrid options (eg,
Aortic mural thrombus is typically associated with under-
iliofemoral endarterectomy and patch plus iliac stenting).
lying aortic pathology, such as aneurysm, aortitis, athero-
The preferred revascularization strategy is informed by
sclerosis, dissection, and aortic graft material.1-3 Because
the arterial anatomy, the severity of disease and symp-
such thrombi arise in the setting of underlying aortic
toms, the patient’s substrate, and the expected proce-
pathology, the thrombi can be considered “secondary,”
dural durability. No RCTs have shown an advantage for
and they most often appear in the descending thoracic
any given revascularization procedure, and all perform
and abdominal aorta.1-3 In contrast, “primary” thrombus
well in early follow-up. Open aortic reconstruction has
occurs in a normal or minimally atherosclerotic aorta and,
improved long-term patency compared with less inva-
rather than being mural, are often pedunculated and pro-
sive options3 but at a cost of a higher risk of periopera-
trude into the aortic lumen. Most often, primary aortic
tive complications.
thrombi are idiopathic, but some have been associated
with hypercoagulable states (eg, malignancy, heparin-
9.3.3. Porcelain Aorta
induced thrombocytopenia, and the antiphospholipid

“Porcelain” aorta refers to the extensive, eggshell-like,
syndrome).2-6
near-circumferential or circumferential calcification of
Aortic thrombus is most often asymptomatic but may
the intima or media of the aortic wall in the ascend-
present with limb ischemia, visceral ischemia, or stroke2-7
ing aorta or aortic arch. It is most often associated with
from embolization. The diagnosis is often typically con-
late-stage atherosclerosis, although it can also be a
firmed by either CTA or TEE.8,9 Asymptomatic patients
late consequence of aortitis. It generally occurs in older
with secondary mural thrombus are usually managed
patients with atherosclerotic disease elsewhere and
conservatively, but patients with primary aortic thrombus
carries an increased risk for cardiovascular events and
or those presenting with embolic events are often man-
mortality.1 Porcelain aorta is best seen on a noncontrast
aged with anticoagulation, endovascular intervention, or
CT scan, although very thin calcification may only be
open surgical therapy; such treatments are informed by
detected intraoperatively with epi-aortic ultrasound or
the patient’s history and the location, size, and mobil-
manual palpation.Impenetrable ascending aortic calci-
ity of the thrombus.2-7,10,11 Long-term anticoagulation is
fication makes it difficult, if not impossible, to perform
most often considered in patients with thrombus in the
central aortic cannulation for cardiopulmonary bypass,
ascending aorta and aortic arch, because of the increased
the anastomosis of proximal coronary bypass grafts to
risk of stroke from potential embolization should aortic
the aorta, aortotomy during aortic valve replacement,
thrombus recur.5-7,10
and graft-aorta anastomoses during aortic replace-
9.3.2. Aortic Occlusion ment. Additionally, performing aortic cross-clamping for
Aortic occlusion, which occurs most often secondary to cardiopulmonary bypass can crack the calcified wall,
extensive atherosclerotic disease, can present along a increasing the risk of stroke from embolization, or imme-
spectrum of acute and chronic clinical courses. CTA is diate exsanguination. Surgical management strategies
most useful in identifying the occlusion, determining its have included use of alternative sites for cannulation
cause, and defining the extent of associated aortic and and proximal bypass grafts with off-pump or beating
branch arterial disease. Aortic occlusion typically occurs heart techniques,2-4 balloon occlusion of the aorta,5
below the renal arteries but rarely can arise above this and the use of circulatory arrest with ascending aortic
level, leading to renal and possibly visceral malperfusion. replacement.6

9.4. Coarctation of the Aorta (CoA) and 20% to 60% of cases and is known as a Kommerell
CLINICAL STATEMENTS
Congenital Abnormalities of the Arch diverticulum.15,18 Such Kommerell diverticula may lead
AND GUIDELINES
to aortic dissection, rupture, or embolization.18-20 Indica-
CoA is a narrowing of the aorta occurring most often tions for treatment of ASCA relate to symptoms and
just distal to the left subclavian artery, typically with an aneurysm size.
aneurysmal aortic segment immediately beyond the
stenosis, but variants are frequent.1 Significant CoA 9.4.1. Coarctation of the Aorta
presents with upper extremity hypertension and lower Recommendations for CoA
extremity hypotension (Table 37). MRI and CT are both Referenced studies that support the recommendations are
useful to evaluate the extent of aortic narrowing and
dilation, as well as the presence of collaterals,2 whereas COR LOE Recommendations
TTE is useful for evaluating the gradient across the 1. In patients with CoA, including those who
have undergone surgical or endovascular
CoA, as well as identifying a coexisting BAV (pres- 1 B-NR intervention, an MRI or CT is recommended
ent in 50%) and other potential congenital defects.3 for initial, surveillance, and follow-up aortic
Untreated CoA may be complicated by aortic dissec- imaging.1-4
tion, heart failure, ruptured cerebral aneurysm, dis- 2. In patients with CoA, BPs should be mea-
tal hypoperfusion, or the consequences of significant 1 C-EO sured in both arms and one of the lower
extremities.
hypertension. Late complications following surgical or
3. In patients with significant native or recurrent
endovascular CoA repair may include undersized grafts,
CoA (Table 37) and hypertension, endovascular
recurrent stenosis, aneurysm or pseudoaneurysm for- 1 B-NR
stenting or open surgical repair of the coarcta-
mation, and rupture, which are typically treated with tion is recommended.2,3,5-12
endovascular procedures unless anatomic features dic- 4. In patients with CoA, guideline-directed medi-
tate open or hybrid surgery.4-11 Hypertension is common 1 C-EO cal therapy is recommended for the treatment
of hypertension.13
after CoA repair, especially during exercise, and when
the repair is performed in adults.12,13 Ambulatory BP 5. In adult patients with CoA, screening for
2b B-NR intracranial aneurysms by MRI or CT may be
monitoring and exercise testing are useful in diagnosis reasonable.14-18
and management.12,13 Patients with CoA undergo life-
long follow-up and imaging because of the associated
cardiovascular risks and the potential requirement for Synopsis
repeat intervention.6,14 CoA may have many anatomic variants and occurs most
An aberrant subclavian artery (ASCA) is commonly commonly at the level of the ductus arteriosus and dis-
an incidental finding but may present with compressive tal to the left subclavian artery. Echocardiogram is indi-
symptoms (including dysphagia and dyspnea) because cated in the evaluation of patients with CoA because a
it courses posterior to the esophagus and trachea and BAV coexists in at least 50% of cases, and CoA may
may associate with aneurysm disevase.15-18 A normal associate with complex congenital heart disease.4 Upper
left aortic arch with a right ASCA occurs in ∼1% of the extremity hypertension and lower extremity hypoperfu-
population, whereas a right aortic arch with a left ASCA sion are the hallmarks of CoA. Intracranial aneurysms
is much rarer and may form a vascular ring.17,18 Dilation may occur in adults with CoA.14-16 Ascending aortic
of the origin of either a right or left ASCA occurs in aneurysms may occur in those with BAV, and aneu-
rysms may be present in the distal arch and descend-
ing aorta.2,11,19,20 Untreated CoA may be complicated
Table 37. Criteria for Significant CoA11,28
by aortic dissection, heart failure, ruptured cerebral
The presence of significant CoA is based on evidence of upper extremity aneurysm, or complications from hypertension. Repair
hypertension (at rest, on ambulatory BP monitoring, or with pathologic
blood pressure response to exercise) or left ventricular hypertrophy and of CoA is performed by endovascular, open surgical,
evidence for 1 of these gradient measurements: and hybrid procedures, depending on patient-specific
1. A noninvasive blood pressure difference of >20 mm Hg between the and anatomic features.2,3,5,8-12 In patients with previous
upper and lower extremities procedures, late complications may include recurrent
2. A peak-to-peak gradient of >20 mm Hg across the coarct by catheter- stenosis, aneurysm or pseudoaneurysm formation, rup-
ization; or a peak-to-peak gradient of >10 mm Hg across the coarct by ture, and persistent hypertension.2,3,6,8,12,21 Hypertension
catheterization in the setting of decreased left ventricular systolic func-
tion or significant collateral flow
is common after CoA repair, especially during exercise,
and ambulatory monitoring and exercise testing may be
3. A mean gradient of >20 mm Hg across the coarct by Doppler echo-
cardiography; or a mean gradient of >10 mm Hg across the coarct by useful in diagnosis and management.3,6,7,22-24 Lifelong
Doppler echocardiography in the setting of decreased left ventricular clinical and imaging follow-up is important to evaluate
systolic function or significant collateral flow for hypertension, recurrent coarctation, and aortic wall
CoA indicates coarctation of the aorta. abnormalities after repair.1,2,6,24

Recommendation-Specific Supportive Text grafting, or bypass grafting, with the choice of pro-
CLINICAL STATEMENTS
cedure informed by patient- and anatomic-specific

1. In patients with CoA, both MRI and CT are can
AND GUIDELINES
characteristics.5,11 In adults who have undergone

detect coexistent BAV, examine the full thoracic
a previous open surgical CoA repair and develop
aorta for coexistent aneurysm disease or arch
recoarctation, aneurysm, or pseudoaneurysm, an
abnormalities, and assist in treatment planning.4,25
endovascular approach (assuming there is ade-
TTE is also can detect the gradients across the
quate iliofemoral access and absence of involve-
site of the coarctation and assess for recoarctation
ment of the supra-aortic trunks) avoids the need
(recurrence of a significant coarct). After repair of
for reoperation.2,3,9,12,29
CoA, complications may occur, including recoarc-
4. Patients with CoA are at risk for complications of
tation, aortic aneurysm, pseudoaneurysm, and aor-
hypertension, including heart failure, stroke, coro-
tic dissection.2,3,11,12,26 Arch and descending aortic nary artery disease, and aortic complications, so
complications are better visualized by MRI or CT hypertension should be assessed and in accor-
than TTE. The optimal imaging frequency after dance with current guidelines.13 Multiple studies
repair of CoA is not well established and is best have shown that persistent hypertension is com-
individualized based on the type of repair, physical mon after CoA correction.3,6,7,23,24 Ambulatory BP
examination findings, and previous imaging find- monitoring and exercise testing may be useful in
ings.27 After establishing stable aortic imaging after the evaluation and treatment of hypertension in
CoA repair, surveillance imaging is often obtained patients with native CoA and after repair.3,22,24
every 3 to 5 years.20,28-30 Recoarctation occurs in 5. Screening studies suggest that adults with CoA
about 10%6,8 after surgical repair and about 8% have an 10% prevalence of intracranial aneurysms
after balloon dilation.21 After endovascular repair (compared with a prevalence of 2% in the normal
of CoA, MRI or CT can evaluate for complications, adult population), with the greatest risk among
recoarctation, or endoleaks.2,3,11 older adults and those with hypertension.14-16,18
2. Patients with a significant CoA typically have Cost-effective analysis supports screening for
hypertension in the upper extremities and a reduc- intracranial aneurysms in adults with CoA, but
tion in BP in the lower extremities. The location of preferred screening strategies remain unknown.17
the CoA will inform any BP differential between Because many of the intracranial aneurysms
the left and right arms. Physical examination may detected by screening will be very small and not
reveal a delay in timing and a decreased amplitude require treatment, shared decision-making about
of the femoral pulse. After CoA repair, recurrent screening may be informed by age, risk factors,
coarctation may occur. Obtaining the BP in the and anticoagulation considerations.18,30
upper and lower extremities assesses for native
and recurrent coarctation. 9.4.2. Other Arch Abnormalities
3. CoA presents with upper extremity hypertension, 9.4.2.1. Aberrant Subclavian Artery, Kommerell’s
lower extremity hypoperfusion, and imaging confir- Diverticulum
mation of narrowing of the aorta that may include
Recommendations for Aberrant Subclavian Artery, Kommerell’s
collateral formation.2,3,6,11 Significant native or reco- Diverticulum
arctation has been variably defined, but commonly COR LOE Recommendations
used criteria are listed in Table 37.7,11 The pres-
1. In patients discovered to have an ASCA
ence of left ventricular hypertrophy is an important in the absence of thoracic aortic imaging,
2a C-LD
marker of disease.28 In addition to abnormal aortic dedicated imaging to assess for TAA is
gradients, anatomic evidence for CoA is necessary reasonable.1,2
and is well characterized by MRI or CT. Adult con- 2. In patients with Kommerell’s diverticulum,
depending on patient anatomy and comor-
genital guidelines have reported the best evidence bidities, repair may be reasonable when
to proceed with intervention to correct CoA, includ- 2b C-LD the diverticulum orifice is >3.0 cm, the
ing hypertension, BP differential between upper combined diameter of the diverticulum and
adjacent descending aorta is >5.0 cm, or
and lower extremities, and TTE-derived gradients both (Figure 27).3
across the coarctation.11 For individuals with native
or recurrent CoA and appropriate anatomic char-
acteristics, endovascular treatment with stenting is Synopsis
typically performed.2,3,6,9-12,29 Open surgical repair of Anomalies of the aortic arch are usually detected inci-
CoA may include subclavian flap aortoplasty, resec- dentally on a CT of the chest or neck ordered for other
tion and end-to-end anastomosis, interposition reasons. An ASCA arises as the fourth branch from the

and hybrid approaches depending on patient anatomy
CLINICAL STATEMENTS
and comorbidities.3
AND GUIDELINES
1. Variant aortic arch anatomy has been found to be
significantly associated with TAA in several single-
center retrospective observational series,1 with
33% of patients with right-sided aortic arch having
concomitant TAA.2 Left-sided aortic arch with aber-
rant right subclavian artery was also significantly
associated with TAD but only occurred in 2% to
8% of those patients.1 Consequently, if the imag-
ing study that detected the ASCA did not include
imaging of the thoracic aorta, then a dedicated CT
or MRI to evaluate for an associated aortic aneu-
rysm is reasonable.
2. Case series have reported rupture and/or dissec-
tion of Kommerell’s diverticulum for diverticula
ranging from 4.0 cm to 10 cm (mean size, 5.0 cm).3
The measurement of the Kommerell’s diverticulum
may be difficult, and various strategies to stan-
dardize measures have been proposed.3 Based on
CT, 2 diameter measurements should be obtained
(Figure 27) using cross-sectional imaging: the
diverticulum orifice (radially and longitudinally at
the aortic wall) and the combined diameter of the
Figure 27. Measurements of Kommerell’s Diverticulum. diverticulum and adjacent descending thoracic
Two diameter measurements should be obtained using cross- aorta (measured from the tip of the diverticulum
sectional imaging: the diverticulum orifice (radially and longitudinally

at the aortic wall) and the combined diameter of the diverticulum and
to the opposite aortic wall6). Repair of Kommerell’s
adjacent descending thoracic aorta (measured from the apex of the diverticulum has been suggested when the orifice
diverticulum to the opposite aortic wall). ARSA indicates aberrant right diameter is >3 cm or the combined diameter of
subclavian artery; LCA, left common carotid artery; LSA, left subclavian the diverticulum and adjacent descending thoracic
artery; and RCA, right common carotid artery. Adapted from Erben aorta is >5.0 cm.3,4,7
et al,7 Copyright 2020, with permission from Elsevier, Inc., and the
Society for Vascular Surgery.
9.4.2.2. Aberrant Left Vertebral Artery Origin
Recommendation for Aberrant Left Vertebral Artery Origin
aorta, distal to the left subclavian artery (or right sub- COR LOE Recommendation
clavian artery in the case of a right-sided aortic arch). It 1. In patients with an aberrant left vertebral
courses through the posterior mediastinum behind the artery origin arising directly from the thoracic
esophagus in its path to perfuse the arm and can cause 2a C-EO
aorta who require aortic repair involving recon-
struction or coverage of the vertebral artery
a vascular ring around the trachea and esophagus that origin, revascularization of the vertebral artery
results in dysphagia, respiratory symptoms, or recur- is reasonable.
rent laryngeal nerve palsy. Kommerell’s diverticulum is a
persistent remnant of the fourth primitive dorsal aortic
arch because of failed regression3 and may be present Synopsis
in 20% to 60% of patients with an aberrant right or left The most common anatomic variant for the left verte-
subclavian artery. The risk of rupture or dissection of a bral artery is arising directly from the aortic arch; 6%
Kommerell’s diverticulum has been reported to be as of adults have a left vertebral artery that arises from
high as 50% in case series, although high-quality data on the arch between the left carotid and left subclavian
the natural history are very limited. The 2020 SVS clinical arteries,1,2 rather than of a branch of the left subcla-
practice guidelines recommend surgical intervention for vian artery. There is a paucity of data on the manage-
Kommerell’s diverticulum when the diverticulum orifice is ment of the left vertebral artery arising from the aortic
>3.0 cm, the combined diameter of the diverticulum and arch in patients undergoing thoracic aortic repair.
adjacent descending aorta is >5.0 cm, or both.4 Success- For patients undergoing elective open surgical par-
ful repair has been described using open, endovascular, tial or total arch repair or undergoing TEVAR for TAA

or dissection, revascularization of the left subclavian Synopsis

CLINICAL STATEMENTS
artery is recommended to preserve left vertebral artery

The most common anatomic pattern of great vessel ori-
AND GUIDELINES
perfusion and reduce the risk of symptomatic verte-

gin, occurring in approximately 70% of adults, is a type I
brobasilar insufficiency, SCI, and stroke.3 This may be
arch, in which the 3 great vessels originate directly from
particularly important in patients with a dominant left
the aorta.4 Bovine arch variants are the most common
vertebral artery or a nonintact circle of Willis. Verte-
arch anomalies, and 2 types are described: In type II-A,
bral artery revascularization via either an open bypass
found in 9% of the population, the left common carotid
or transposition technique can be accomplished with
artery arises directly from the innominate artery (Figure
good outcomes.3,4
28); in type II-B, found in 13% of the population, the
innominate and left common carotid arteries arise from
Recommendation-Specific Supportive Text a common origin (Figure 28).5 The term “bovine arch” is
1. In patients undergoing elective TEVAR with planned a misnomer, because the arch vasculature in cattle has a
left subclavian artery coverage, preoperative revas- single, large brachiocephalic vessel that subsequently tri-
cularization of the left subclavian artery has been furcates into 2 subclavian arteries and a bicarotid trunk.5
shown to decrease the risk of stroke and SCI,5-8 Others have referred to the bovine aortic arch pattern as
presumably by maintaining perfusion through the an aortic arch with a common origin of the innominate
posterior circulation via the left vertebral artery. In and left carotid artery.
a small series of 9 patients with an aberrant left Some authors have suggested that a bovine arch
vertebral artery origin undergoing open aortic arch increases the risk of aortic dissection, but the data are
replacement, no neurologic complications were limited.2,6 Among patients with acute type A aortic dis-
reported among patients who first underwent section, a bovine arch was highly predictive of an arch
revascularization of the left vertebral artery.4 tear (OR, 5.9; 95% CI, 2.89–12.04; P<0.001) and
increased perioperative stroke (OR, 2.69; 95% CI, 1.2–
9.4.2.3. Bovine Arch (Common Innominate and Left 6.0; P=0.016) based on multivariable analysis, although
Carotid Artery) it was not associated with worse long-term survival.7
Recommendation for Bovine Arch (Common Innominate and Left
Carotid Artery)

1. In patients with bovine arch (common 1. A bovine aortic arch appears to be a marker for
2b C-LD
innominate and left carotid artery), TAD and more rapid aortic expansion.1 Among
imaging to assess for TAA may be patients with TAD, the prevalence of a bovine arch
r easonable. 1-3
was 26.3%, compared with 16.4% in controls
Figure 28. Normal and Bovine Aortic Arch Configurations.

(A) Type I aortic arch: The normal aortic arch configuration. (B) Type II-A aortic arch: The left common carotid artery originates from the
innominate artery. (C) Type II-B aortic arch: The innominate and left common carotid arteries share a common origin. LCA indicates left common
carotid artery; LSA, left subclavian artery; LVA, left vertebral artery; RCA, right common carotid artery; RSA, right subclavian artery; and RVA, right
vertebral artery. Adapted from Layton et al.5 Copyright 2006, American Society of Neuroradiology. Used with permission from Mayo Foundation
for Medical Education and Research, all rights reserved.

(P<0.001). Moreover, among patients with TAA, the Recommendations for Physical Activity and Quality of Life (Continued)
CLINICAL STATEMENTS
annual aortic growth rate was 0.29 cm/y among COR LOE Recommendations
AND GUIDELINES
those with a bovine arch versus 0.09 cm/y among
4. For patients with clinically significant
those with normal arch anatomy. A recent meta- aortic disease, it is reasonable to screen
analysis found that the proportion of TAD among for anxiety, depression, and posttraumatic
patients with bovine arch was 41.5%, compared stress disorder and, when indicated, provide
2a C-LD resources for support7,8; it is also reason-
with 34.0% among patients with standard arch con- able to provide education and resources to
figuration.3 If aortic dilation or aneurysm is found on minimize patients’ concerns, support opti-
imaging, subsequent surveillance imaging may be mal decision-making, and enhance quality
of life.5,9-11
obtained.
Synopsis
9.5. Tumors
As surgical outcomes for aortic disease improve, a focus
Tumors of the thoracic aorta are usually secondary, on patient-reported health-related quality of life (HRQOL)
resulting from contiguous or metastatic spread of primary outcomes is becoming increasingly important,10 because
malignancies, especially lung and esophageal.1,2 Primary patients have become increasingly concerned about
malignant tumors of the aorta, which are extremely rare, HRQOL issues such as returning to work, pain manage-
are most often primary sarcomas that protrude into the ment, risk of infection, activity recommendations and
lumen but leave the aortic wall intact. Aortic sarcomas restrictions, and neurologic complications. The most com-
are aggressive tumors with a propensity for arterial mon measures of HRQOL are generic patient-reported
embolization, disseminated metastases, and rapid clini- outcome measures (eg, SF-36), although validated aneu-
cal deterioration,3,4 usually with limited survival after initial rysm-specific measures have been developed.7,12,13
diagnosis.5,6 Tumors of the thoracoabdominal aorta may In patients with Marfan syndrome in the GenTAC reg-
exhibit nonspecific symptoms. On imaging, aortic tumors istry, HRQOL was slightly below the population norm.
are often initially mistaken for atherosclerosis or aneurys- Better HRQOL was independently associated with
mal disease7 (although PET imaging may suggest tumor socioeconomic factors (eg, private insurance, active
metabolic activity over metabolically quiescent athero- employment) but not factors related to disease sever-
sclerosis), so the diagnosis is often made by histologic ity or comorbidities.14,15 Although aneurysms are usually
examination of embolic debris or surgical specimens8-10; asymptomatic before diagnosis, surgical aortic repair
in some cases, the diagnosis is made postmortem. Com- is associated with an initial deterioration in HRQOL at
bined therapy with surgery (resection and reconstruc- 3 months, including decreased physical, cognitive, and
tion of the segment of aorta containing the neoplasm) social function that generally returns to preoperative
and chemoradiotherapy provide the best survival results, levels after 6 to 12 months.11 Standardized reporting
although the overall prognosis remains poor. of preoperative and postoperative HRQOL measures is
needed to guide further improvements in interventional
strategies and improve the overall patient experience.16
10. PHYSICAL ACTIVITY AND QUALITY Patients with aortic aneurysms, who have adequate
OF LIFE BP control, may have improvements in overall cardio-
vascular health when undertaking moderate intensity
Recommendations for Physical Activity and Quality of Life
aerobic activity at least 3 to 4 days per week, 30 to
COR LOE Recommendations 60 minutes per session.17-19 Although resistance training
1. For patients with significant aortic dis- may be beneficial to patients with cardiovascular disease,
ease, education and guidance should be
provided about avoiding intense isomet-
it increases central aortic BP and, therefore, benefits for
1 C-EO ric exercises (eg, heavy weightlifting or those with aortic aneurysm are less well understood
activities requiring the Valsalva maneuver), because, theoretically, increases in BP could contribute
burst exertion and activities, and collision
sports. 1,2
to subsequent aortic growth, complications, or both. Fur-
ther longitudinal study is warranted.20-22
2. For patients who have undergone sur-
gery for aortic aneurysm or dissection,
1 C-EO
postoperative cardiac rehabilitation is
recommended.3,4 Recommendation-Specific Supportive Text
3. In patients with thoracic or abdominal aortic 1. In patients with aortic disease, limited data are
aneurysms whose BP is adequately con-
available to guide recommendations regarding the
trolled, it is reasonable to encourage 30 to
2a C-LD
60 minutes of mild-to-moderate intensity forms of exercise that are safe and promote car-
aerobic activity at least 3 to 4 days per diovascular health versus those that pose an acute
week.5,6
or long-term risk of aortic growth or rupture. But

evidence exists regarding the physiologic ben- to improve overall cardiovascular health, includ-
CLINICAL STATEMENTS
efits of exercise and the hemodynamic conse- ing among patients with TAA32-34 and AAA.20,35,36
AND GUIDELINES
quences of various form of exercise and exertion A recent meta-analysis suggests that that higher
in case series and relevant animal models. There physical activity is associated with a reduced risk
has been a uniform consensus among numer- of AAA.37 In 1 study of a mouse model of Marfan
ous expert committees on aortic disease that it is syndrome, rates of aortic root growth were signifi-
wise to avoid intense isometric exertion or exer- cant slower in mice that exercised daily on a tread-
cises that require the Valsalva maneuver, given mill compared with sedentary mice.38 In another
that heavy lifting with Valsalva can produce acute study of mice with Marfan syndrome, both mild and
increases in SBP to >300 mm Hg. There is also a moderate—but not strenuous—aerobic exercise
consensus that light weightlifting and low-intensity protected the structural integrity of the aortic wall,
aerobic exercise are safe and likely improve both as evidenced by reduced elastin fragmentation and
physical and mental health. No uniform consen- reduced expression of matrix metalloproteinases 2
sus exists about the safety of intermediate-level and 9 within the aortic wall, compared with seden-
static and aerobic exercise. Recommendations for tary controls.39
exercise intensity are best individualized, informed 4. Depression and anxiety often occur in patients
by multiple factors that include underlying aortic with aortic disease, regardless of surgical status.
pathology, aortic diameter and ASI, aortic growth Posttraumatic stress disorder after dissection is
rate, age, family history, and any other high-risk a particular risk.8 Screening patients and provid-
features (eg, uncontrolled hypertension). Ongoing ing resources for assistance may prevent mental
investigation is needed to better define the levels health issues from becoming more severe and
of resistance activities that would be considered lead to an increased HRQOL.9,40 The SF-36 is a
low-risk for adverse aortic events, favoring greater common tool for assessing mental health for these
exercise restrictions among patients at higher risk patients7,11,12,41 but may not cover all patient con-
of dissection.17,23-26,27 cerns, such as activity restriction, family life, and
2. Although data are limited, cardiac rehabilitation has losing ability to earn income.16 More studies are
been shown to be useful and safe for patients after needed with both pre- and postoperative HRQOL
aortic surgery.4,5,27,28 A randomized trial of exercise- data to improve shared decision-making and
based cardiac rehabilitation in patients who have patient outcomes.12,13,41 Exercise may decrease
undergone surgery for type A aortic dissection depression.9 Education before procedures helps
showed improved peak oxygen uptake, maximal most patients feel more satisfied with their pro-
workload, and HRQOL.3 Fear of a repeat cardiac cedures16 and improve postoperative HRQOL.41
event can cause patients who are post–aortic dis- Patients and clinicians can define surgery success
section to decrease or stop exercise and sexual differently, showing the importance of discussing
activity, but mild-to-moderate intensity exercise expectations and risks.
may be cardioprotective. Because of decondition-
ing, patients may be unable to exercise initially at
the recommended level.27 An intensity of 3 to 5 11. COST AND VALUE CONSIDERATIONS
metabolic equivalents of task is recommended, Although assessment of cost and value in development
while avoiding strenuous lifting, lifting to the point of guidelines is of growing importance, studies are lim-
of exhaustion, or other activities that entail maximal ited on the cost-effectiveness of aortic disease treat-
exertion.6,29 In a retrospective study, patients with ment and lack standard methods for comparison.1
small AAA went through a modified cardiac rehabil- Screening for AAA among men ≥65 years of age has
itation program before surgery, and the rate of aor- been shown to be cost-effective,2,3 although data for
tic growth was slower in the rehabilitation group.28 screening women are less clear. Women have a lower
3. High-intensity athletic training in 1 study has been incidence of AAA but higher risk of rupture and longer
shown to be an independent predictor of aortic life expectancy, so incremental cost-effectiveness is sim-
growth, although these data were limited to the ilar to men and may justify screening, especially in those
aortic root and did not include AAA.30 In a recent with a history of smoking.4
study in 442 athletes of mean age 61 years, aortic In patients with AAA, studies comparing EVAR to
root enlargement by z-score was present in 24% open surgical repair generally show lower initial costs for
of participants and, after multivariate analysis, elite EVAR based on shorter hospital stays; however, ongoing
competitor status was found to be an independent expenses for EVAR surveillance and reinterventions may
predictor of aortic growth.31 Less is known about minimize long-term cost advantages after 2 to 5 years.5-9
the potential effects of mild-to-moderate intensity In addition, significant variability in costs across organiza-
aerobic activity on aortic growth, but it is known tions and countries, and changing efficiencies in tech-

niques, makes it difficult to make recommendations on whose expression can be modified through targeted
CLINICAL STATEMENTS
preferred interventional approaches based primarily on mechanisms and present opportunities to identify new
AND GUIDELINES
relative costs.6,10,11 treatment options for patients with aortic disease.1-7 In
Findings are mixed but similar for descending TAA, addition, developing image-based cardiac and aortic
with trends toward lower initial hospital costs with markers derived from large-scale imaging studies with
TEVAR compared with open surgery stemming from automated machine learning–based analysis might pro-
shorter length of stay, but the long-term results are more vide a wealth of information for guiding the optimal care
neutral.12-14 of these patients.
Few data examine the cost-effectiveness of manage-
ment strategies of TAA. For the management of AAS, the
costs are not easily modifiable. However, for manage- 12.2. Genetic and Nongenetic Factors
ment of chronic TAD, patients often see a host of spe- Various genes have been associated with and linked to
cialists, including both cardiologists and surgeons, have TAA and dissection. Consequently, genetic testing can
follow-up visits with specialists in both the community identify pathogenic mutations in specific genes that
and at tertiary or quaternary centers, or both. Moreover, increase a patient’s risk of aneurysm, dissection, or both
diagnostic imaging is often duplicated because of differ- and may inform the optimal timing of aortic repair. As
ences in imaging protocols or quality, or simply because the prevalence of genetic testing increases, the discov-
images are not readily transferrable. Consequently, there ery of more genes will help in the earlier diagnosis of
are likely opportunities for significant cost savings if asymptomatic nonsyndromic TAA. In addition to the con-
redundant clinician visits and imaging could be reduced tribution of genetic variants, environmental factors and
through common protocols, common imaging platforms, lifestyle habits may contribute to aortic aneurysm forma-
and coordinated care.15 tion. Further research on these factors may provide evi-
dence to guide lifestyle modifications that could reduce
a patient’s lifelong aortic risk. Recent evidence suggests
12. EVIDENCE GAPS AND FUTURE that fluoroquinolone use is associated with an increased
DIRECTIONS risk of aortic aneurysm and dissection, but the pathways
Most of the current recommendations for patients with through which this effect is mediated are unknown.
aortic disease are based on expert opinion and data Future research investigating the potentially protective
or harmful effects of other pharmacologic agents on aor-
from observational studies, large registries, and pro-

spective studies, but few are from randomized clinical tic health might further elucidate the pathophysiology of
trials. More data are needed from basic science stud- aortic disease.1-10
ies and RCTs to guide prevention, early diagnosis, and
advanced treatment for aortic disease. In the future, 12.3. Biomechanics of the Aorta
precision medicine and patient-centered approaches
Emerging evidence suggests that aortic diameter alone
will enable clinicians to develop care plans to optimize
is an insufficient predictor of risk for aortic dissection.
outcomes for each patient. Future research should
Understanding the distribution of biomechanical wall
include diverse populations and examine race, ethnicity,
and sex differences to ensure that all patient groups are stress in the various anatomic locations of the aorta,
represented and that questions pertinent to their aortic as well as potential contributions of hemodynamic flow
health are answered. disturbances such as those from aortic valve stenosis or
regurgitation, or even from a well-functioning BAV, may
improve risk stratification strategies and, in turn, patient
12.1. Biomarker Studies outcomes, filling a knowledge gap on wall stress distribu-
Although interest in using circulating biomarkers for risk tion in patients with aortic aneurysms.1-4
stratification of patients with aortopathy has increased,
biomarker expression has not been clearly associated
with relevant clinical aortic events. Most studies have 12.4. Sex, Race, and Ethnicity
focused on protein-based biomarkers and noncoding Conflicting data exist in the literature on the associa-
RNAs in patients with bicuspid aortopathy. These emerg- tion between sex and outcome in patients with aortic
ing biomarkers and other better, early-stage biomarkers, disease. Studies have shown different rates of aortic
along with advanced noninvasive imaging modalities, aneurysm growth and dissection risk in male versus
may help us precisely identify the risk associated with female patients. Nevertheless, the data are inconsis-
adverse outcomes in these patients. In addition, noncod- tent, because some outcome studies indicate that sex
ing RNAs such as microRNA are biological molecules affects prognosis, whereas others show no impact of

sex. Clearly, further research is needed to elucidate Continued evolution in stent-graft design, focused on
CLINICAL STATEMENTS
the impact of sex on the incidence and progression flexibility and durability, improved vascular imaging tech-
AND GUIDELINES
of aortic disease, the risk of aortic dissection, and the nology, and advances in simulation training for operators,
outcomes of intervention. Even more challenging is the will likely further reduce the risk of reinterventions and
fact that few studies have been published on racial and improve long-term outcomes.1-6
ethnic disparities among patients with aortic disease
and those undergoing aortic intervention. Moreover, it is
unclear that all patients with aortic disease have equal 12.7. Optimal Exercise and Rehabilitation
access to skilled practitioners to care for them, so it is Protocols
imperative that we seek ways to actively minimize such Very limited research has been conducted on optimal
health care disparities.1-17 Similarly, efforts should be exercise in patients with aortic disease. Moreover, no
made to broaden clinical trials to represent the diverse specific rehabilitation strategies exist for patients who
populations that we treat; study design, methodology, still have untreated diseased aortic segments after sur-
reporting, and implementation should be designed to be gical aortic repair and who do not meet the surgical
more inclusive.18,19 threshold for intervention. Developing patient-centric
rehabilitation protocols and individualized exercise pro-
grams for patients with aortic disease is an unmet need
12.5. Quality of Life in Patients With Aortic that requires further study.1-4
Disease
Baseline HRQOL assessment in patients with aortic
12.8. Equitable Care and Training Opportunities
disease is lacking, and the few studies that have tar-
geted HRQOL have been conducted only in patients Sociodemographic disparities can pose challenges to
receiving endovascular or open aortic repair. The impact patients and clinicians who seek and offer cardiovascular
of physical, mental, emotional, sexual, and professional and aortic care. Market competition, a relatively modern
status on the psychosocial well-being, tolerance of med- phenomenon, and physician market concentration can
ical therapies, and recovery from aortic intervention has drive decision-making and subsequently affect optimal
not been well studied. The long-term effects on physical care. Providing optimal cardiovascular and aortic care
and mental HRQOL after aortic repair are unknown. In will depend on widespread regional quality improvement
addition, evidence-based knowledge on studies target- projects to determine best practices, minimize varia-
ing quality of life in patients with heritable TAA is narrow tions in areas where evidence-based medicine has finite
or limited only to patients with Marfan syndrome; almost benchmarks, and standardize patient selection and case
no studies have been performed in patients with Loeys- management. Physician participation in these programs
Dietz syndrome or vascular Ehlers-Danlos syndrome, should be encouraged, and educational interventions
for example. Furthermore, only scattered studies have and training should be provided to disseminate knowl-
examined strategies for boosting the psychological edge and improve performance, which will help increase
health of patients with aortic disease and those under- awareness for patients and physicians in less-populated,
going aortic surgery. Aortic diseases require a lifetime underserved areas.1-3
of treatment and surveillance, so research is needed
on ways to improve and sustain patient engagement,
especially among those who are disadvantaged or at a PEER REVIEW COMMITTEE MEMBERS
lower educational level.1-8 David P. Faxon, MD, FACC, Chair; Gilbert R. Upchurch Jr,
MD, FAHA, FACC, Vice Chair; Aaron W. Aday, MD, MSc,
FAHA; Ali Azizzadeh, MD; Michael Boisen, MD*; Beau
12.6. New Endovascular Technology Hawkins, MD, FACC†; Christopher M. Kramer, MD, FACC,
Advances in endovascular technology have dramatically FAHA; Jessica G.Y. Luc, MD; Thomas E. MacGillivray,
impacted treatment strategies in patients with aortic MD‡; S. Christopher Malaisrie, MD, FACC§; Kathryn
disease requiring intervention. Despite this significant Osteen, PhD, RN, CMSRN, CNE; Himanshu J. Patel, MD,
progress, current endovascular designs are limited in FACC, FAHA; Parag J. Patel, MD||; Wanda M. Popescu,
their application because of the differing hemodynamic MD; Evelio Rodriguez, MD, FACC; Rebecca Sorber, MD;
and anatomic challenges presented by each segment of Philip S. Tsao, PhD; Annabelle Santos Volgman, MD,
the aorta and individual differences in aortic anatomy. In FACC, FAHA
addition, operator knowledge and experience, as well as
methodical patient selection, are important for obtain- *SCA representative. †SCAI representative. ‡AHA/ACC Joint Committee on
ing optimal outcomes from endovascular procedures. Clinical Practice Guidelines liaison. §AATS representative. ||SIR representative.

AHA/ACC JOINT COMMITTEE MEMBERS ARTICLE INFORMATION
CLINICAL STATEMENTS
This document was approved by the American College of Cardiology Clinical Pol-
Joshua A. Beckman, MD, MS, FAHA, FACC, Chair; Cath-
AND GUIDELINES
icy Approval Committee and the American Heart Association Science Advisory
erine M. Otto, MD, FAHA, FACC, Chair-Elect; Patrick and Coordinating Committee in July 2022, the American College of Cardiology
T. O’Gara, MD, MACC, FAHA, Immediate Past Chair¶; Science and Quality Committee in August 2022, and the American Heart As-
sociation Executive Committee in September 2022.
Anastasia Armbruster, PharmD, FACC; Kim K. Birtcher,
This article has been copublished in the Journal of the American College of
PharmD, MS, FACC¶; Lisa de las Fuentes, MD, MS, Cardiology.
FAHA; Anita Deswal, MD, MPH, FACC, FAHA; Dave L. Copies: This document is available on the websites of the American College of
Cardiology (www.acc.org) and the American Heart Association (professional.heart.
Dixon, PharmD, FACC, FAHA¶; Bulent Gorenek, MD,
org). A copy of the document is also available at https://professional.heart.org/
FACC; Norrisa Haynes, MD, MPH; Adrian F. Hernandez, statements by selecting the “Guidelines & Statements” button. To purchase addi-
MD, MHS, FAHA; José A. Joglar, MD, FACC, FAHA¶; tional reprints, call 215-356-2721 or email [email protected].
The expert peer review of AHA-commissioned documents (eg, scientific
W. Schuyler Jones, MD, FACC; Daniel Mark, MD, MPH,
statements, clinical practice guidelines, systematic reviews) is conducted by the
FACC, FAHA¶; Debabrata Mukherjee, MD, FACC, FAHA, AHA Office of Science Operations. For more on AHA statements and guidelines
FSCAI; Latha Palaniappan, MD, MS, FACC, FAHA; Mari- development, visit https://professional.heart.org/statements. Select the “Guide-
lines & Statements” drop-down menu near the top of the webpage, then click
ann R. Piano, RN, PhD, FAHA; Tanveer Rab, MD, FACC;
“Publication Development.”
Erica S. Spatz, MD, MS, FACC; Jacqueline E. Tamis- Permissions: Multiple copies, modification, alteration, enhancement, and dis-
Holland, MD, FAHA, FACC, FSCAI; Y. Joseph Woo, MD, tribution of this document are not permitted without the express permission of the
American Heart Association. Instructions for obtaining permission are located at
FACC, FAHA
https://www.heart.org/permissions. A link to the “Copyright Permissions Request
Form” appears in the second paragraph (https://www.heart.org/en/about-us/
statements-and-policies/copyright-request-form).
PRESIDENTS AND STAFF
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aortic diseases of the thoracic and abdominal aorta of the adult. The Task 8. IMPROVE Trial Investigators. Comparative clinical effectiveness and cost
Force for the Diagnosis and Treatment of Aortic Diseases of the European effectiveness of endovascular strategy v open repair for ruptured abdominal
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Society of Cardiology (ESC). Eur Heart J. 2014;35:2873–2926. aortic aneurysm: three year results of the IMPROVE randomised trial. BMJ.
AND GUIDELINES
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AND GUIDELINES
cific alcoholic beverages, and abdominal aortic aneurysm. Circulation.
2014;130:646–652. 12.5. Quality of Life in Patients With Aortic Disease
1. Archer S, Pinto A, Vuik S, et al. Surgery, complications, and quality of life:
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Appendix 1. Author Relationships With Industry and Other Entities (Relevant)—2022 2022 ACC/AHA Guideline for the Diagno-
sis and Management of Aortic Disease
CLINICAL STATEMENTS
AND GUIDELINES
Institutional,
Ownership/ Organizational,
Committee Speakers Partnership/ Personal or Other Finan- Expert
Member Employment Consultant Bureau Principal Research cial Benefit Witness
Eric M. Isselbacher, Massachusetts General Hospi- None None None None None None
Chair tal—Director, Healthcare Trans-
formation Lab; Co-Director,
MGH Thoracic Aortic Center;
Harvard Medical School—Asso-
ciate Professor of Medicine
James Hamilton Johns Hopkins Medicine—Pro- None None None None None None
Black III, Vice Chair fessor of Surgery
Ourania Preventza, Baylor College of Medicine; • Terumo Aortic None None None None None
Vice Chair Baylor St. Luke’s Medical Cen- (Bolton Medical)
ter—Professor of Surgery, Divi- • W.L. Gore &
sion of Cardiothoracic Surgery Associates
John G. University of Pennsylvania—Pro- None None None None None None
Augoustides fessor, Department of Anesthe-
siology and Critical Care
Adam W. Beck University of Alabama at Bir- • Cook Medical None None • Cook Medical None None
mingham—Professor and Direc- • Cryolife • Medtronic
tor of Vascular Surgery and
• Endologix • Terumo Aortic
Endovascular Therapy, Depart-
• Philips (Bolton
ment of Surgery
Medical)
• Terumo Aortic
(Bolton Medical) • W.L. Gore &
Associates
Michael A. Bolen Cleveland Clinic, Main Campus— None None None None None None
Associate Professor of Radiol-
ogy, Division of Radiology
Alan C. Braverman Washington University School None None None None None None
of Medicine—Alumni Endowed
Professor in Cardiovascular
Diseases; Director, Marfan
Syndrome and Aortopathy
Clinic Cardiovascular Division,
Department of Medicine
Bruce E. Bray University of Utah—Professor, None None None None None None
Department of Internal Medi-
cine and Biomedical Informatics
Maya M. Patient advocate None None None None None None
Brown-Zimmerman
Edward P. Chen Duke University Medical Cen- None None None • Bolton Medical* • Bolton Medi- None
ter—Professor and Division Chief, cal†
Division of Cardiovascular and
Thoracic Surgery
Tyrone J. Collins Ochsner Medical Center—Sec- • InspireMD None None None • W.L. Gore & None
tion Head, Interventional Cardiol- Associates†
ogy, Co-Director, Cardiac Cathe-
terization Laboratory, Department
of Interventional Cardiology
Abe DeAnda Jr UTMB-Galveston—Professor None None None None None None
and Chief, Division of Cardio-
vascular and Thoracic Surgery,
Division of Surgery
Christina L. Fanola University of Minnesota—Assis- • Janssen Pharma- None None None None None
tant Professor, Department of ceuticals
Cardiovascular Medicine
Leonard N. Girardi Weill Cornell Medicine—Profes- None None None None None None
sor and Chairman, Department
of Cardiothoracic Surgery
(Continued )

Appendix 1. Continued
CLINICAL STATEMENTS
Institutional,
AND GUIDELINES
Caitlin W. Hicks Johns Hopkins University None None None None None None
School of Medicine—Associate
Professor of Surgery, Division
of Surgery
Dawn S. Hui University of Texas Health San None None None • Astellas None None
Antonio— Associate Professor, Pharma*
Department of Cardiothoracic
Surgery
William Schuyler Duke University—Associate Pro- • Bayer‡ None None • Boehringer None None
Jones fessor of Medicine & Director of • Janssen Pharma- Ingelheim
Cardiac Catheterization Labo- ceuticals‡ • Bristol-Myers
ratory, Division of Medicine/ Squibb
Cardiology
Vidyasagar Cleveland Clinic—Assistant None None None None None None
Kalahasti Professor, Cleveland Clinic
Lerner College of Medicine
of the Case Western Reserve
University; Director, Marfan
Syndrome & Connective Tissue
Disorder Clinic, Aortic Center,
Heart, Vascular and Thoracic
Institute
Karen M. Kim University of Michigan—Assis- None None None None None None
tant Professor, Department of
Cardiac Surgery
Dianna M. Milewicz University of Texas Health Sci- None None None None None None
ence Center at Houston—Presi-
dent George H.W. Bush Chair
of Cardiovascular Medicine
Vice Chair, Department of Inter-

nal Medicine Director, Division
of Medical Genetics Director,
Division of Internal Medicine
Gustavo S. The University of Texas Health • Cook Medical None None • Cook Medical‡ • Centerline None
Oderich Science Center at Houston, • GE Healthcare • GE Health- Biomedical,
McGovern Medical School— care‡ Advisory
• W.L. Gore &
Professor of Surgery and Chief Board
Associates • W.L. Gore &
of Vascular and Endovascular Member*
Associates‡
Surgery, Director of Aortic • Cook
Center, Department of Cardio- Medical†
Thoracic and Vascular Surgery
• Philips, Advi-
sory Board
Member
• W.L. Gore &
Associates†
Laura Ogbechie Baylor College of Medicine— None None None None None None
Nurse Practitioner, Department
of Cardiothoracic Surgery
Susan B. Promes Penn State Health Hershey None None None None None None
Medical Center—Professor and
Chair‚ Department of Emer-
gency Medicine
Elsie Gyang Ross Stanford University School of None None None None None None
Medicine— Assistant Professor,
Department of Surgery and
Medicine
(Continued )

CLINICAL STATEMENTS
Institutional,
AND GUIDELINES
Marc L. Beth Israel Deaconess Medical None None None None • Medtronic, None
Schermerhorn Center— George H. A. Clowes Scientific
Jr. Professor of Surgery, Chief, Advisory
Division of Vascular and Endo- Board
vascular Surgery, Department Member*
of Surgery • Philips, Sci-
entific Advi-
sory Board
Member*
Sabrina Singleton American Heart Association/ None None None None None None
Times§ American College of Cardiol-
ogy Guideline Advisor
Elaine E. Tseng University of California San None None None None • Cryolife†‡ None
Francisco; San Francisco VA
Medical Center—Professor of
Surgery University of California
San Francisco; Chief of Cardio-
thoracic Surgery, San Francisco
VA Department of Surgery
Grace J. Wang University of Pennsylvania Hospi- None None None None None None
tal—Associate Professor of Sur-
gery, Division of Vascular Surgery
and Endovascular Therapy
Y. Joseph Woo Stanford University—Chair, Divi- None None None None None None
sion of Cardiothoracic Surgery
This table represents the relationships of committee members with industry and other entities that were determined to be relevant to this document. These relation-
ships were reviewed and updated in conjunction with all meetings and/or conference calls of the writing committee during the document development process. The
table does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest
represents ownership of ≥5% of the voting stock or share of the business entity, or ownership of ≥$5000 of the fair market value of the business entity; or if funds
received by the person from the business entity exceed 5% of the person’s gross income for the previous year. Relationships that exist with no financial benefit are
also included for the purpose of transparency. Relationships in this table are modest unless otherwise noted.
According to the ACC/AHA, a person has a relevant relationship IF: a) the relationship or interest relates to the same or similar subject matter, intellectual property
or asset, topic, or issue addressed in the document; or b) the company/entity (with whom the relationship exists) makes a drug, drug class, or device addressed in
the document or makes a competing drug or device addressed in the document; or c) the person or a member of the person’s household, has a reasonable potential for
financial, professional or other personal gain or loss as a result of the issues/content addressed in the document.
*No financial benefit.
†This disclosure was entered under the Clinical Trial Enroller category in the ACC’s disclosure system. To appear in this category, the author acknowledges that
there is no direct or institutional relationship with the trial sponsor as defined in the (ACCF or AHA/ACC) Disclosure Policy for Writing Committees.
‡Significant relationship.
§Sabrina Singleton Times is an AHA/ACC joint staff member and acts as the Guideline Advisor for the “2022 ACC/AHA Guideline for the Diagnosis and Manage-
ment of Aortic Disease.” No relevant relationships to report. Nonvoting author on measures and not included/counted in the RWI balance for this committee.
ACC indicates American College of Cardiology; ACCF, American College of Cardiology Foundation; AHA, American Heart Association; MGH, Massachusetts
General Hospital; RWI, relationships with industry and other entities; UTMB, University of Texas Medical Branch; and VA, Veterans Affairs.

Appendix 2. Reviewer Relationships With Industry and Other Entities (Comprehensive)—2022 ACC/AHA Guideline for the
Diagnosis and Management of Aortic Disease
CLINICAL STATEMENTS
AND GUIDELINES
Institutional, Or-
Ownership/ ganizational, or
Speakers Partnership/ Other Financial Expert
Reviewer Employment Consultant Bureau Principal Personal Research Benefit Witness
David P. Faxon, Harvard University; None None • REVA • Boston Scientific None None
Peer Review Com- Brigham and Wom- Medical (DSMB)
mittee Chair en’s Hospital • CSL Behring (DSMB)
• Medtronic (DSMB)*
Gilbert R. University of Florida None None • Antyllus* • NIH, PI* • Bolton† None
Upchurch Jr, Peer College of Medicine • Cook†
Review Commit- • Gore†
tee Vice Chair • Medtronic†
Aaron W. Aday Vanderbilt University • OptumCare‡ None None • Janssen None
Medical Center • TransThera Pharmaceuti-
Sciences cals†
• University of
Manitoba†
Ali Azizzadeh Cedars Sinai None None None None • Gore† None
Michael Boisen University of None None None None None None
(representing Pittsburgh
SCA)
Mohammad H. University of None None None None None None
Eslami (represent- Pittsburgh
ing SVS)
Beau Hawkins The University of • Baim Institute None None None • Behring† None
(representing Oklahoma College for Clinical • Boston
SVM) of Medicine Research Scientific†
• Hemostemix†
• NIH†
Christopher M. University of Virginia • Bristol Myers None None • NHLBI‡ • Cytokinetics† None
Kramer Squibb‡ • NIBIB‡ • MyoKardia†
• Cytokinetics
• Eli Lilly
• Xencor
Jessica G. Y. Luc University of British None None None None None None
Columbia
Thomas E. MacGil- Houston Methodist None None None None • Xylocor† None
livary (representing
STS)
S. Christopher Northwestern Uni- • Artivion • Edwards None • Artivion‡ • Artivion† None
Malaisrie versity • Medtronic Life- • Atricure • Edwards
(representing sciences‡ • Edwards Lifesciences‡ Lifesciences†
AATS) • Terumo • Terumo‡ • Medtronic†
Kathryn Osteen Baylor University None None None None • AHA* None
Louise Herrington • Congenital
School of Nursing Heart Public
Health Con-
sortium*
• Sigma Theta
Tau-Eta
Gamma
Chapter*
• The Chil-
dren’s Heart
Foundation*
Himanshu J. Patel University of Michi- • Edwards Life- None None None • Edwards None
gan Health sciences Lifesciences†
• Gore • Gore†
• Medtronic‡ • Medtronic†
• Terumo • Nexus†
Parag J. Patel Medical College of None None None None • AHA* None
(representing SIR) Wisconsin • SIR*
(Continued )

CLINICAL STATEMENTS
Institutional, Or-
AND GUIDELINES
Ownership/ ganizational, or
Speakers Partnership/ Other Financial Expert
Reviewer Employment Consultant Bureau Principal Personal Research Benefit Witness
Wanda M. Yale School of None None None None • Ambu, Advi- None
Popescu (repre- Medicine sory Board
senting SCA) Member
Evelio Rodriguez Ascension Tennes • Abbott‡ • Abbott‡ None • Abbott • Abbott† None
see Saint Thomas • Edwards Life- • Edwards • Atricure • Atricure†
Hospital sciences‡ Life- • Boston Scientific • Boston Sci-
sciences‡ • Claret entific†
• Philips‡ • Direct Flow • Edwards
• Edwards Lifesciences‡ Lifesciences†
• Medtronic • Medtronic†
Rebecca Sorber Johns Hopkins None None None None None None
University School of
Medicine
Philip S. Tsao VA Palo Alto Health None None None None None None
Care System;
Stanford University
School of Medicine
Annabelle Santos Rush College of • Bristol Myers None None • NIH‡ • Apple* None
Volgman Medicine Squibb, • Novartis†
DCICDP
• Janssen
Pharmaceu-
ticals
• Merck
• Pfizer
• Sanofi‡
This table represents all reviewers’ relationships with industry and other entities that were reported at the time of peer review, including those not deemed to be
relevant to this document, at the time this document was under review. The table does not necessarily reflect relationships with industry at the time of publication. A
person is deemed to have a significant interest in a business if the interest represents ownership of 5% of the voting stock or share of the business entity, or ownership
of $5000 of the fair market value of the business entity; or if funds received by the person from the business entity exceed 5% of the person’s gross income for the pre-
vious year. Relationships that exist with no financial benefit are also included for the purpose of transparency. Relationships in this table are modest unless otherwise
noted. Names are listed in alphabetical order within each category of review. Please refer to https://www.acc.org/guidelines/about-guidelines-and-clinical-documents/
relationships-with-industry-policy for definitions of disclosure categories or additional information about the ACC/AHA Disclosure Policy for Writing Committees.
*No financial benefit.
†This disclosure was entered under the Clinical Trial Enroller category in the ACC’s disclosure system. To appear in this category, the author acknowledges that
there is no direct or institutional relationship with the trial sponsor as defined in the (ACCF or AHA/ACC) Disclosure Policy for Writing Committees.
‡Significant relationship.
AATS indicates American Association for Thoracic Surgery; ACC, American College of Cardiology; ACCF, American College of Cardiology Foundation; AHA,
American Heart Association; DCICDP, Diverse Clinical Investigator Career Development Program; DSMB, data and safety monitoring board; NIH, National Institutes
of Health; NHLBI, National Heart, Lung, and Blood Institute; NIBIB, National Institute of Biomedical Imaging and Bioengineering; PRC, Peer Review Committee; PI,
principal investigator; SCA, Society of Cardiovascular Anesthesiologists; SIR, Society of Interventional Radiology; STS, Society of Thoracic Surgeons; SVM, Society
for Vascular Medicine; SVS, Society for Vascular Surgery; and VA, Veterans Affairs.

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Circulation

ACC/AHA CLINICAL PRACTICE GUIDELINE

2022 ACC/AHA Guideline for the Diagnosis and

Writing Committee Members*

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<zjs;Clinical Statements and Guidelines> • <zjss;10163> • <zdoi;10.1161/CIR.0000000000001106>

CONTENTS 6.1.2. Genetic Aortopathies. . . . . . . . . . e361

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7.6. Management of PAU. . . . . . . . . . . . . . . . . . e414 9.5. Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . e441

Both . . . . . . . . . . . . . . . . . . . . . . . . . . e414 11. Cost and Value Considerations. . . . . . . . . . . . . . . e442

1. Because outcomes for patients with aortic disease

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study; randomized controlled trial; review; atherosclerotic

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1.3. Document Review and Approval Abbreviation Meaning/Phrase

1.5. Class of Recommendations and Level of LDL low-density lipoprotein

LVV large vessel vasculitis

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Table 1. Associated Guidelines

Title Organization (Reference)

and distensibility to the aorta, features that are critical to

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Figure 1. The Anatomy of the Aorta and Its Main Branches.

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risk estimates and do not inform absolute risk. It follows

in risk at a diameter of ≥4.5 cm justifies defining an aorta

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Figure 4. Freedom From Ascending

Figure 5. Relative Risk of Aortic

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Figure 6. Acute Aortic Syndromes.

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Table 3. Classification of Aortic Dissection Chronicity Based 2.4.1.1. Definition

Figure 7. Classification of Acute Aortic Dissection.

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Any of the aortic branches are at risk for malperfusion as

the false lumen expands and compresses the true lumen

and can occur in multiple vascular beds simultaneously

to 47% progress to aortic dissection if the intimal layer

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Figure 10. Classification of Thoracoabdominal Aortic Aneurysms.

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Recommendations for Aortic Imaging Techniques to Determine Recommendation-Specific Supportive Text

Presence and Progression of Aortic Disease (Continued )

COR LOE Recommendations

to the axis of blood flow.

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Figure 11. Classification of Endoleak Types.

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Reports to measure the aortic root (at end-diastole) from the

size in large population-based studies.12 Aortic length is

Table 5. Diagnostic Performance of Aortic Imaging Modalities

Parameter CT MRI TTE TEE US

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of the root and ascending aorta,1 significantly increas-

Figure 12. Aortic Imaging Techniques to Determine the

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1 C-LD2 TGFBR2 No 4.5

2a C-LD1,2 TGFBR2 Yes 4.0

2b C-EO5-7 TGFB2‡ – 4.5†

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