Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

Contents lists available at ScienceDirect

Journal of the Society for Cardiovascular

Angiography & Interventions
journal homepage: www.jscai.org

Comprehensive Review

Transcatheter Interventions in Patients With Adult Congenital Heart Disease

Weiyi Tan, MD, MPH a, *, Ada C. Stefanescu Schmidt, MD, MSc b, Eric Horlick, MDCM c,
Jamil Aboulhosn, MD d
a
Division of Cardiology, University of Texas Southwestern Medical Center, Dallas, Texas; b Division of Cardiology, Massachusetts General Hospital, Harvard Medical
School, Boston, Massachusetts; c Peter Munk Cardiac Centre, University of Toronto, Toronto, Ontario, Canada; d Ahmanson/UCLA Adult Congenital Heart Disease Center,
David Geffen School of Medicine, University of California, Los Angeles, California

A B S T R A C T

Patients with congenital heart disease now live well into adulthood because of advances in surgical techniques, improvements in medical management, and the
development of novel therapeutic agents. As patients grow older into adults with congenital heart disease, many require catheter-based interventions for the treatment
of residual defects, sequelae of their initial repair or palliation, or acquired heart disease. The past 3 decades have witnessed an exponential growth in both the type
and number of transcatheter interventions in patients with congenital heart disease. With improvements in medical technology and device design, including the use of
devices designed for the treatment of acquired valve stenosis or regurgitation, patients who previously would have required open-heart surgery for various conditions
can now undergo percutaneous cardiac catheter-based procedures. Many of these procedures are complex and occur in complex patients who are best served by a
multidisciplinary team. This review aims to highlight some of the currently available transcatheter interventional procedures for adults with congenital heart disease,
the clinical outcomes of each intervention, and any special considerations so that the reader may better understand both the procedure and patients with adult
congenital heart disease.

Introduction cardiac catheter-based procedures. Many of these procedures are com-

plex and occur in complex patients, who are best served by a multidis-
The past 3 decades have witnessed an exponential growth in both the ciplinary team. We aim to highlight some of the currently available
type and number of transcatheter interventions in patients with transcatheter interventional procedures for adults with CHD, the clinical
congenital heart disease (CHD). In 2000, the first transcatheter heart outcomes of each intervention, and any special considerations so that the
valve implanted in a human was designed for children with pulmonic reader may better understand both the procedure and patients with
valve disease1,2 and contributed to a revolution in the treatment of ac- ACHD.
quired aortic stenosis (AS) with transcatheter aortic valve replacement
(TAVR).
Advances in surgical techniques, improvements in medical manage- Special needs of patients with ACHD
ment, and the development of novel therapeutic agents now allow pa-
tients with CHD to live well into adulthood. However, many of these Careful preprocedural planning is especially important for patients
patients with adult congenital heart disease (ACHD) require catheter- with ACHD because their complex anatomy and physiology, comorbid
based interventions for the treatment of residual defects, sequelae of conditions, and previous experiences may lead to complications or un-
their initial repair or palliation, or acquired heart disease.3,4 successful procedures. The development of skills required for indepen-
With improvements in medical technology and device design, dent practice in ACHD interventions requires dedicated fellowship
including the use of devices designed for the treatment of acquired valve training and can be achieved by a combination of pathways, as described
stenosis or regurgitation, patients who previously would have required in a recent position statement by the Society for Cardiovascular Angi-
open-heart surgery for various conditions can now undergo percutaneous ography and Interventions.5 Although the position statement by the

Abbreviations: ACHD, adult congenital heart disease; ASD, atrial septal defect; CHD, congenital heart disease; CoA, coarctation of the aorta; PDA, patent ductus
arteriosus; PFO, patent foramen ovale; SVASD, sinus venosus defect; TGA, transposition of the great arteries; TPVR, transcatheter pulmonary valve replacement; VSD,
ventricular septal defect.
Keywords: adult congenital heart disease; congenital cardiac interventions; stenting; transcatheter interventions; transcatheter valve replacement.
* Corresponding author: [email protected] (W. Tan).

https://doi.org/10.1016/j.jscai.2022.100438
Received 17 March 2022; Received in revised form 17 July 2022; Accepted 1 August 2022
Available online 24 August 2022
2772-9303/© 2022 The Author(s). Published by Elsevier Inc. on behalf of the Society for Cardiovascular Angiography and Interventions Foundation. This is an open
access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

Society for Cardiovascular Angiography and Interventions should be medical records and increased resources available for quality improve-
viewed as the absolute minimum training required, the nuances and ment and research.
complete toolbox of special skills can only truly be achieved with years of
clinical experience.
A detailed history of patients’ previous procedures and operations, Shunt-related interventions
with a review of their original reports, is imperative, especially because it
relates to vascular access and possible prior shunts. After childhood in- Atrial-level shunts
terventions (including central access catheters, cardiac catheterizations,
or surgeries), iliofemoral vein and/or artery occlusion is commonplace. Ostium secundum atrial septal defect closure. Atrial septal defect (ASD) is
Because of collateral formation, these occlusions may not always be one of the more commonly encountered congenital heart malformations,
clinically apparent on physical examination or vascular ultrasound but accounting for approximately 10% to 15% of CHD in adults.11,12 Adult
can be noted based on previous procedural notes or dedicated cross- patients who have been diagnosed with an ASD usually present with
sectional imaging. It may be possible to perform hemodynamic cathe- progressive dyspnea on exertion, palpitations, and decreased exercise
terization via venous collaterals or the azygous vein or recanalization of tolerance as a consequence of left-to-right shunting that results in
an occluded iliofemoral vein with serial balloon dilations or stenting.6 right-heart overload, dilation, and dysfunction.13 Some patients may also
Performing interventions using large-bore catheters may require addi- develop pulmonary hypertension, tricuspid regurgitation (TR), and atrial
tional consideration and thoughtfulness. Coronary artery disease is arrhythmias. Patients who are asymptomatic early in life may develop
becoming increasingly common in the population with ACHD; percuta- symptoms as the degree of left-to-right shunting increases with age
neous coronary interventions may be challenging because of anomalous because of decreased left atrial and ventricular compliance as well as
origin or unusual orientation of the coronaries (particularly in patients increased systemic arterial resistance.
with tetralogy of Fallot or transposition of the great arteries [TGA]) or The indications for ASD closure include symptoms; paradoxical
after coronary reimplantation during infancy. embolism; significant shunting (pulmonary blood flow to systemic blood
Most patients with moderate or complex ACHD have had many in- flow ratio, or Qp:Qs > 1.5:1) in the absence of symptoms; and/or the
teractions with the health care system and undergone multiple pro- presence of right-heart enlargement, detected using echocardiography
cedures, and some of these experiences may have caused anxiety or or magnetic resonance imaging (MRI) in the presence of normal or low
trauma in the patient as well as their family. Vascular access sites may pulmonary vascular resistance (less than one-third of systemic vascular
have increased sensitivity because of previous injuries, scarring, or resistance).4 Patients with severe pulmonary hypertension are usually
fibrosis, and, therefore, higher doses of local anesthetic and conscious not candidates for ASD closure; however, the use of pulmonary vasodi-
sedation are often needed. There is often higher anxiety associated with lator therapy may reduce the pulmonary arterial pressure and resistance
procedures in patients and their families because they may think of enough to permit ASD closure, with or without the use of a fenestrated
previous complications, worry about the risk of adverse outcomes, or device.14,15 Although the surgical outcomes of ASD closure are excellent,
worry whether the information obtained at the time of catheterization surgical closure of an ostium secundum ASD has a higher complication
will lead to a surgery or prolonged hospital stay. In addition, young rate than transcatheter closure,16 and thus transcatheter closure has
adults who are being treated for the first time in an adult catheterization become the standard of care for most ostium secundum ASDs in adults.
laboratory often have different expectations from the procedural Preprocedural evaluation using transesophageal echocardiography
workflow, such as the use of preprocedural sedation, general anesthesia (TEE) or cross-sectional imaging (cardiac computed tomography angi-
vs conscious sedation, the ability to have their parents present or ography [CTA] or MRI) is used to screen for associated defects, rule
involved, and the level of discomfort to expect. Discussion of the risks out partial anomalous pulmonary venous connections, and assess the
and benefits of the procedure, expectations, and questions is recom- rim size of ASDs.17-19 The procedure is usually performed under fluo-
mended during a clinic visit prior to the procedure, particularly for a roscopic and imaging guidance using either TEE or intracardiac
new patient. echocardiography.20,21
Multidisciplinary team discussion is also recommended prior to Procedural success (complete closure with stable positioning of the
complex procedural planning, including topics such as anticoagulation device) has been reported in 94% to 98% of cases,22,23 with a low risk of
management, intracardiac shunts, mechanical support, and backup sur- embolization, thrombus formation, aortic root perforation or
gical options. Some patients may have congenital comorbid conditions erosion,24,25 pericardial effusion, and arrhythmias; follow-up using
that may affect the airway (craniofacial abnormalities and cervical spinal echocardiography on postprocedural day 1, at 1 to 3 months, and then at
instability in patients with Down syndrome). Acquired comorbidities, 12 months is recommended. The most commonly used device in the
such as airway stenoses or hypoplasia, pulmonary hypertension, renal United States is the Amplatzer septal occluder (Abbott). Amplatzer septal
and hepatic dysfunction, prior sensitization to blood products, and occluders (Figure 1) with sizes ranging from 4 to 38 mm have been
polycythemia, with the risk of hyperviscosity, may also affect procedural approved by the United States Food and Drug Administration (FDA), with
planning and need discussion prior to the procedure. For example, pa- excellent long-term results.23,26 Of 1000 patients enrolled in a post-
tients with cyanosis, low muscle mass, or hepatic congestion due to right- approval study, the Amplatzer device caused complications in only
heart disease or Fontan circulation have more renal and hepatic 0.65% (n ¼ 6), with a risk of cardiac erosion of 0.3% over 2 years.23 The
dysfunction than is apparent using standard laboratory evaluation and Gore Cardioform ASD occluder (Gore Medical; size, 27-48 mm) and the
have a higher risk of bleeding.7 Gore Cardioform septal occluder (size, 20-30 mm) have also been
The American College of Cardiology National Cardiovascular Data approved by the FDA.23 Occlutech has an ASD and patent foramen
Registry (IMproving Pediatric and Adult Congenital Treatments Registry) ovale (PFO) occluder that is currently available in Canada but not in the
is a powerful quality improvement tool for pediatric and adult congenital United States. The device is being studied in active clinical trials
cardiac catheterizations8; the other multicenter collaborations include (NCT04291898).
the Congenital Cardiac Interventional Study Consortium9 and Congenital In older patients, the presence of left atrial and/or left ventricular
Cardiac Catheterization Project on Outcomes.10 Patient heterogeneity, diastolic dysfunction may result in increased left atrial pressure after
variability in procedural selection and techniques, the need for extensive device closure of ASD.27 Therefore, a careful hemodynamic assessment
and detailed data entry, and rapid changes in available technologies have (this requires a second venous access) of the left atrial pressure at base-
long been a significant challenge in registry and outcome research in line and during temporary balloon occlusion of the ASD is important in
ACHD; however, this has been somewhat mitigated by the electronic such patients, and fenestrated ASD devices may be optimal.28

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

Figure 1. Transcatheter occlusion of an ostium secundum atrial septal defect (ASD) using the Amplatzer septal occluder. (A) The top panel shows fluoroscopic
images of a sizing balloon across the ASD to measure the waist of the defect, whereas the bottom panel shows the simultaneous transesophageal echocardiographic
(TEE) images. (B) The device is now deployed across the ASD, as observed using fluoroscopy (top panel), and is seen to be in a stable position, as detected using TEE
imaging (bottom panel). (C) After release of the device from the delivery cable, the septal occluder has aligned with the atrial septum in a more natural way (top panel)
and is seen to be still in a stable position across the ASD while straddling the retroaortic rim using TEE imaging (bottom panel).

PFO closure. Patent foramen ovale is a common anatomic variant (pre- Transcatheter correction of superior sinus venosus atrial septal defects. Sinus
sent in ~25% of the general population)29 that is a remnant of fetal venosus atrial septal defects (SVASDs) (superior or inferior type) are rare,
circulation. The indications for PFO closure are symptoms such as cryp- comprising approximately 5% to 10% of all ASDs.41 Superior SVASD is a
togenic stroke30-32 and paradoxical emboli; in select cases, pla- deficiency of the common wall between the superior vena cava (SVC) and
typnea-orthodeoxia,33 persistent hypoxia due to TR shunting blood right-sided pulmonary veins and is associated with an anomalous right
across the PFO,34 and decompression illness may be considered.35 upper pulmonary vein in up to 90% of cases. Inferior SVASD is a defi-
Transcatheter PFO closure was introduced in the early 2000s36 and has ciency of the wall between the right atrium (RA) or inferior vena cava
become more prevalent since 2017, when 3 trials showed a reduction in and the left atrium and can be associated with anomalous pulmonary
the risk of recurrent events after an index cryptogenic stroke in carefully venous drainage of the right lower pulmonary veins to the inferior vena
selected patients (using clinical and anatomic features such as Risk of cava or RA.42 Patients with SVASDs usually present with symptoms
Paradoxical Embolism or RoPE score and PFO-Associated Stroke Causal similar to patients with ostium secundum ASDs, although they may
Likelihood [PASCAL] Classification System)29,37 receiving antiplatelet present earlier because of increased left-to-right shunting from both an
therapy.30-32 atrial-level shunt and 1 anomalous right-sided pulmonary veins that
Two devices, the Amplatzer PFO occluder and the Gore Cardioform drain into the SVC. Treatment is indicated to reverse right-heart
septal occluder, that have been approved by the FDA for PFO closure. enlargement and the following symptoms: dyspnea on exertion,
Although these devices have excellent procedural outcomes30-32 and decreased exercise tolerance, or right-heart failure.43 The standard of
helped reduce the absolute risk of stroke by 3.3% in 1 meta-analysis care is surgical repair4 using the Warden procedure or 2-patch repair.
(with a number needed to treat of 30),38 an increased risk of new-onset This repair can be technically challenging, and the operative complica-
atrial fibrillation (3.4%) was noted, with, however, a very low associ- tions include pulmonary vein stenosis (which may lead to dyspnea,
ated incidence of stroke and resolution of arrhythmia within the first 3 pulmonary edema, or infarction), SVC stenosis, or sinus node
months.39 Other complications, such as pericardial effusion,24 device dysfunction.41,44
embolization, and device erosion, are extremely rare. A new device, However, over the last several years, transcatheter correction of su-
NobleStitch,40 is being studied, and a clinical trial is underway in the perior SVASDs using covered stents (Figure 2) has become more estab-
United States (NCT04339699) to compare its effectiveness with that of lished.43,45-48 The procedure is technically challenging and is successful
the established Amplatzer device; the Occlutech Flex II device is being only in patients with suitable anatomy. Cardiac CTA is performed, and
used in Europe and Canada and is under investigation in the United virtual or 3-dimensionally printed models are used to help with visuali-
States (NCT05069558). zation of SVASDs, the anomalous pulmonary veins, and surrounding

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

Figure 2. Transcatheter stenting of a superior sinus venosus defect with associated partial anomalous pulmonary venous connection. (A) Three-dimensional
rendering from a computed tomography angiogram demonstrating a superior sinus venosus defect with the associated partial anomalous pulmonary venous
connection. (B) Deployment of a covered stent within the superior vena cava to exclude the sinus venosus defect, which allows systemic venous blood from the
superior vena cava to drain into the right atrium while pulmonary venous blood can drain into the left atrium from the partial anomalous pulmonary venous
connection. (C) Angiogram of the partial anomalous pulmonary venous connection demonstrating unobstructed blood flow back to the left atrium from behind the
covered stent and through the sinus venosus defect. Ao, aorta; CS, covered stent; IV, innominate vein; LA, left atrium; PA, pulmonary artery; PAPVC, partial anomalous
pulmonary venous connection; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

structures45 to assess whether the anatomy is favorable for the placement infective endocarditis may also be candidates for VSD closure. Patients
of a covered stent that will direct SVC blood to the RA, allowing for with irreversible pulmonary hypertension or Eisenmenger syndrome are
unobstructed anomalous pulmonary vein flow to then drain into the left not candidates for VSD closure.4
atrium through the residual SVASD. The procedure is usually performed The types of VSDs that are amenable to transcatheter closure are
with the patient under general anesthesia given the need for intra- primarily muscular or apical VSDs and some membranous VSDs,49,52,53
procedural imaging guidance using TEE (Figure 3). whereas defects in close proximity to valves or ventricular-free walls
A description of the largest cohort to date demonstrated that the require surgical repair. The only device that has been approved by the
procedure has good short-term outcomes, with no incidence of sinus FDA for transcatheter VSD closure is the Amplatzer muscular or PI
node dysfunction.48 However, one of the drawbacks of this procedure is (postmyocardial infarction) muscular VSD occluder (Abbott), and it has
difficulty in using just 1 stent to adequately cover the SVC superiorly and been demonstrated to be effective in the treatment of both congenital and
the SVC-RA junction inferiorly while maintaining stent stability. Forty acquired (postmyocardial infarction) muscular VSDs.54,55 Other devices,
percent (n ¼ 10) of the patients in the study required covered Chea- such as the Amplatzer ductal occluder I and Amplatzer ductal occluder II
tham-Platinum stents (B. Braun Inc) that were longer than 6 cm, which (Abbott), can be used in an off-label fashion to close certain types of VSDs
are currently not available commercially in the United States. Further- that have favorable anatomy (such as a membranous VSD with a
more, 52% (n ¼ 13) of the patients in the study required additional stents windsock-shaped aneurysmal sac).49 Closure of membranous VSDs is
to secure the covered stent, and 24% of the patients (n ¼ 6) had covered feasible; however, the development of conduction abnormalities is a
stents that either migrated or embolized during the procedure. If the stent major concern, with rates reported to be as high as 6% of cases,49,52,53
is not long enough, there may still be residual shunting at the inferior although membranous VSDs associated with aneurysms of the ventricu-
margin of the stent, as noted in 44% (n ¼ 11) of the patients in the study lar septum can usually be treated, with a low risk of conduction system
by Hansen et al.48 In fact, the last 9 patients in the study had abnormalities.56 The long-term results are favorable, with procedural
custom-made 7- or 8-cm-long, covered 10-zig Cheatham-Platinum stents success of >90% and complications such as heart block (up to 6%), he-
to increase the zone of apposition in the SVC to prevent migration or molysis (1%-2%), and embolization (1%-2%) being relatively rare.49
embolization and reduce the chance of residual leaks around the stent at
the SVC-RA junction. Larger studies and more experience with this
technique will lead to further iterations and improvements of the pro- Patent ductus arteriosus closure
cedure; however, a select subset of patients with superior SVASDs may
benefit from transcatheter correction of the defect, and this may help The patent ductus arteriosus (PDA) is a vestige from fetal circulation
avoid a surgical option. that connects the descending aorta and pulmonary artery (PA).57-59 The
incidence of an isolated PDA in term infants is approximately 2.9 per 10,
000 live births,60 and it is more common in patients with genetic syn-
Ventricular septal defect closure dromes such as DiGeorge or CHARGE (coloboma, heart defects, choanal
atresia, growth retardation, genital abnormalities, and ear abnormal-
Ventricular septal defects (VSDs) comprise approximately 20% of all ities).61 Patients with a small PDA (Qp:Qs < 1.5) may have no symptoms,
congenital heart lesions49,50 and can occur anywhere along the ventric- and its diagnosis may be incidental; however, some may have a contin-
ular septum. Patients with unrepaired VSDs are usually asymptomatic if uous murmur on examination. Patients with a moderately sized PDA
the shunt is small or may present with evidence of left-heart volume (Qp:Qs, 1.5:1-2.2:1) may have exercise intolerance, heart failure, and
overload (pulmonary venous congestion, shortness of breath, or dyspnea evidence of left-sided (left atrial and/or left ventricular) volume overload
on exertion) due to increased left-to-right shunting and pulmonary detected using echocardiography. Adult patients with a large PDA (Qp:Qs
overcirculation. Adult patients with very large unrepaired VSDs usually > 2.2:1) will most likely have evidence of irreversible pulmonary hy-
present with irreversible pulmonary hypertension and right-to-left pertension and differential cyanosis (normal oxygenation of the upper
shunting, with cyanosis, or Eisenmenger syndrome.4,51 The indication extremities, with cyanosis of the lower extremities due to the flow of
for VSD repair is evidence of left ventricular volume overload and a he- deoxygenated blood from the PDA), consistent with the diagnosis of
modynamically significant shunt with a Qp:Qs ratio of 1.5:1 as long as Eisenmenger syndrome. However, these patients may not have a murmur
the pulmonary pressure or pulmonary vascular resistance is normal or because of equalization of pressures on both the right and left sides of the
low.4 Patients with worsening aortic valve regurgitation or a history of heart.

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Figure 3. Transesophageal echocardiography during

stenting of a superior sinus venosus defect. (A) Traditional
bicaval view of the superior sinus venosus defect, with superior
vena cava blood draining into both the left and right atriums.
(B) Color Doppler imaging demonstrating blood flow with
mainly left-to-right shunting across the superior sinus venosus
defect. (C) Traditional bicaval view of the superior sinus
venosus defect after deployment of a covered stent to direct
superior vena cava blood to the right atrium while excluding
the superior sinus venosus defect to prevent shunting of left
atrial blood to the right atrium. (D) Color Doppler imaging
demonstrating only a trace residual left-to-right shunt around
the covered stent at the end of the case. CS, covered stent; LA,
left atrium; RA, right atrium; SVC, superior vena cava. The
asterisk indicates the superior sinus venosus defect.

Patent ductus arteriosus closure is indicated in patients with left-to- Creation of shunts
right shunting with symptoms or asymptomatic patients with left-heart
enlargement. PDA closure should not be attempted in those with Eisen- Although many interventions in patients with ACHD are aimed at
menger syndrome.4 A history of infective endocarditis involving the PDA closing shunts, in some cases, palliation with transcatheter shunt creation
is rare and is an indication for PDA closure.62 In adults (and children) with can be considered. In patients with severe pulmonary arterial hyperten-
PDA, the outcomes of closure have been very good, including in select sion but low left atrial pressure, creation of an atrial-level shunt may help
patients with moderately elevated pulmonary arterial pressure and decompress the right ventricle (RV) (at the expense of lowering systemic
resistance.63 The closure of most PDAs can be accomplished via cathe- saturation) and treat the following symptoms: end-stage right-heart
terization, with minimal morbidity and a high rate of success.58 In most failure and syncope.69 Transcatheter creation of a Potts shunt (from the
patients, a single venous access alone is required to cross the PDA in an descending aorta to the left PA) has also been described in select patients
antegrade fashion and deploy a device. In patients with elevated pulmo- with severe pulmonary hypertension as a way to preserve oxygenated
nary resistance, pulmonary bed vasoreactivity to pulmonary vasodilatory flow to the coronaries and cerebral circulation while allowing
agents or reduction in pulmonary arterial pressure and resistance during right-to-left shunting from the PA to the descending aorta.70 Creation of
test occlusion may predict a favorable outcome with device occlusion.58 an atrial-level shunt was thought to be a promising therapy in patients
The currently available devices include coils, which are used for with diastolic dysfunction and heart failure with preserved ejection
closure of small-sized PDAs, and multiple occlusion devices, including fraction71; however, the results of a recent randomized control trial did
the Amplatzer duct occluder (approved in the United States) and the not demonstrate a benefit with the creation of an atrial-level shunt over a
Occlutech PDA device.58,64-67 The risks of PDA closure are low and sham intervention.72 In select patients, creation or enlargement of a VSD
include device embolization, hemolytic anemia due to high-pressure via a transcatheter or hybrid technique may be helpful in improving
residual shunting across the PDA, vascular access complications, and cardiac output, such as in patients with a double-outlet RV and restrictive
infection.58,67,68 VSDs.73,74

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Valve interventions in medical device technology have improved the delivery profile of
the balloons used for the intervention and made vascular access site
Balloon angioplasty of native pulmonary valve stenosis injury less likely.

Pulmonary valve stenosis is fairly common, comprising approxi-

mately 7% of all congenital heart defects in some epidemiologic Pulmonary valve replacement
studies.75 Pulmonic stenosis can occur at 3 locations: at the valvular,
subvalvular, or supravalvular level. Valvular stenosis is the most Transcatheter pulmonary valve replacement (TPVR) is the most
amenable to transcatheter intervention. Valvular pulmonic stenosis commonly performed transcatheter valve procedure in patients with
mainly occurs as an isolated lesion but can also be associated with certain ACHD for the treatment of dysfunction of a native pulmonary valve, a
conditions such as tetralogy of Fallot, congenital rubella syndrome, and valve within a homograft or RV-PA conduit, or a prosthetic pulmonary
Noonan syndrome. Patients with pulmonary valve stenosis present with valve.83 The largest group of patients with ACHD requiring TPVR are
exertional dyspnea and may show signs of right ventricular hypertrophy those with repaired tetralogy of Fallot83; patients with a pulmonary
during transthoracic echocardiography. The indications for intervention atresia-intact ventricular septum, pulmonary stenosis with surgical val-
for pulmonic stenosis include symptomatic patients with votomy, repaired truncus arteriosus with dysfunctional RV-PA conduits,
moderate-to-severe pulmonic stenosis (a peak Doppler velocity of >3 m/s congenital aortic valve stenosis due to the Ross procedure and subse-
and a peak gradient of 36 mm Hg during echocardiography) or quent homograft dysfunction, and double-outlet RV after Rastelli pro-
asymptomatic patients with severe pulmonic stenosis (a peak Doppler cedure can also require TPVR.
velocity of 4 m/s, a peak gradient of 64 mm Hg, or a mean gradient of In patients with predominant PR, the 2018 American College of Cardi-
35 mm Hg).4 ology or American Heart Association guidelines on the management of
Balloon valvuloplasty is the treatment of choice for isolated pul- patients with ACHD recommend consideration of valve replacement in
monary valve stenosis if the valve is mobile and doming, with a lower symptomatic patients (dyspnea, chest pain, and/or exercise intolerance
chance of success in those with dysplastic (seen in 15% of cases) or referable to PR or otherwise unexplained); asymptomatic patients with ev-
calcified valves.76 Compared with surgical valvotomy, transcatheter idence of RV or left ventricle (LV) systolic dysfunction, severe RV enlarge-
balloon valvuloplasty results in lower mortality and morbidity and is ment (an indexed RV end-diastolic volume of >160 mL/m2 or an indexed
now considered the standard of care, with surgery relegated to those RV end-systolic volume of >80 mL/m2), and an RV systolic pressure of more
in whom transcatheter interventions have failed or those with than two-third of systemic pressure; or those with objective, progressive
incompatible anatomy. The original catheter-based technique was reduction in exercise capacity.4 Patients with sustained tachyarrhythmias
described by Rubio and Limon-Lason77 in 1956, whereas the currently and moderate-to-severe PR may also benefit from TPVR.4 In patients with
employed percutaneous static balloon valvuloplasty technique was prosthetic or homograft pulmonic stenosis, the guidelines recommend
first reported by Kan et al78 in 1982. The outcomes of the procedure intervention in a fashion similar to that in patients with native pulmonic
are excellent, with a low risk of complications4,76,79; the use of a stenosis.4
balloon-to-annulus ratio of 1.2:1.25 is preferred.80 Postprocedural In the current era of TPVR (Central Illustration), the most widely used
mild pulmonary regurgitation (PR) is usually well tolerated, and there valves in patients with ACHD in the United States are the Melody valve2
will be a low rate of restenosis over the next decade,81,82 although (Medtronic Inc) and Edwards Sapien valve84,85 (Edwards Lifesciences).
approximately 25% of patients at 20-year follow-up will require pul- The Melody valve is indicated for the treatment of pulmonary valve
monary valve replacement. The technique for balloon valvuloplasty annuli with diameters ranging from 16 to 22 mm, whereas the Sapien
has not changed significantly over the years. However, advancements valve is indicated for the treatment of valve annuli with diameters

Central Illustration. Timeline of the evolution of the transcatheter pulmonary valve technologies. CE, European Commission; FDA, Food and Drug Administration;
HDE, humanitarian device exemption; TPV, transcatheter pulmonary valve; US, United States.

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

ranging from 20 to 29 mm. Both the valve platforms are associated with conduits that will be dilated using a balloon and stented. Coronaries
excellent short- and intermediate-term outcomes and are comparable adjacent to bioprosthetic valves are usually at a low risk of compression
with surgical valves in terms of longevity and the risk of unless valve fracture is required. Preprocedural computed tomography
reintervention.83,85-87 or MRI can be used to assess the coronary distance to the proposed
The acute procedural complications include injury to the tricuspid landing zone of the device (which requires a collaborative review be-
valve, valve embolization, injury to the PA, coronary compression, or tween interventionalists and radiologists) and identify high-risk (<3
delivery system fracture due to retained equipment in the patient.88 The mm) and low-risk (>15 mm) anatomies99; most patients are in an in-
use of a long sheath (>60 cm) can prevent damage to the tricuspid valve termediate risk zone and require compression testing at the time of the
while crossing it using the valve delivery system.89 The long-term procedure.
complications include valve dysfunction90 and infective endocarditis. Patients with native RVOTs (such as patients with tetralogy of Fallot
Infective endocarditis is a serious concern and may have occurred in up and those who have undergone transannular patch repair) have a dilated
to 10% of patients with dysfunctional homografts being considered for pulmonary valve annulus, and, thus, there is often no landing zone for a
intervention; a history of endocarditis, immunocompromised state, and balloon-expandable transcatheter pulmonary valve. The treatment of
residual stenosis after TPVR are risk factors for endocarditis following large-diameter (>30 mm), native RVOTs is especially challenging given
TPVR.91-94 Stent fracture has been noted to be a problem with the that the largest, commercially available, balloon-expandable TPVR
Melody valve, especially when prestenting is not performed within platforms are the 29-mm Sapien 3 or Ultra valves. Hybrid surgical
conduits or native right ventricular outflow tracts (RVOTs). Prestenting plication of the PA or the use of a PA band can be considered via ster-
does not appear to be necessary for bioprosthetic valves for notomy or thoracotomy in order to establish a “landing zone” for
valve-in-valve procedures, given the presence of a metallic or plastic TPVR.100,101 The Venus P valve (Venus Medtech) and the Harmony valve
ring, which protects the stent platform from compressive forces.95 The (Medtronic) are self-expanding, covered, hourglass-shaped, RVOT
cobalt chromium stent frame of the Sapien valve is significantly more reducer platforms, with the valve in the central waist,102 and the Har-
durable, can withstand high compressive forces, and is not prone to mony valve has now been approved by the FDA for use in patients with
fracture; therefore, prestenting prior to Sapien valve implantation does ACHD with severe PR (Figure 4). Edwards Lifesciences has developed the
not appear necessary as long as there is no proximal or distal stenosis at Alterra self-expanding RVOT reducer (Figure 5), in which the 29-mm
the valve location and an adequate valve size for the patient’s body Sapien S3 valve is subsequently implanted (during the same procedure
surface area can be placed.88,96,97 Evaluation for coronary arterial or a separate procedure); this system also had excellent early results and
compression prior to valve implantation is a critical portion of the has now been approved by the FDA for native or surgically repaired
procedure because coronary artery compression can occur in up to 5% RVOTs with severe PR.103 A detailed analysis of the anatomy of RVOTs
of patients, especially in those with abnormal coronary anatomy or using gated cardiac CTA is necessary to establish candidacy and a target
reimplanted coronary arteries98 and those with stenotic homografts or landing zone for these devices.

Figure 4. Harmony valve implantation. (A) Using a pre-

procedural computed tomography angiogram of the chest, the
operator receives an analysis of the patient’s pulmonary artery
and right ventricular outflow tract, which can help predict the
appropriate location for deployment of the valve. (B) Medtronic
uses the computed tomography angiogram data to create a
perimeter plot, which helps predicts the potential fit of a Har-
mony transcatheter pulmonary valve within the pulmonary ar-
tery. (C) Three-dimensional rendering of how the valve would
fit within the pulmonary artery. (D) Fluoroscopic image of the
Harmony valve after deployment. Angiography with a pigtail
catheter demonstrates no residual pulmonary valve regurgita-
tion. The arrow indicates the Harmony valve. LPA, left pulmo-
nary artery; LZ, landing zone; MPA, main pulmonary artery; PA,
pulmonary artery; RPA, right pulmonary artery; RV, right
ventricle; TPV, transcatheter pulmonary valve.

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

Percutaneous repair of native atrioventricular valve patients with ACHD with severe valvular regurgitation, especially those
regurgitation: Edge-to-edge repair who are at a prohibitively high risk of surgical repair or replacement.
Early feasibility studies of new clip devices, such as the Edwards PASCAL
Severely regurgitant atrioventricular valves can develop in many system (Edwards Lifesciences), have also shown promising results.114
patients with ACHD as sequelae of their underlying congenital anatomy
or as residual from their surgeries. For example, patients with congeni-
tally corrected TGA can develop significant systemic TR as they get older, Prosthetic atrioventricular valve replacement: Valve-in-valve,
and the systemic ventricle starts to dilate and fail.104 Patients with un- valve-in-ring, or novel techniques for the treatment of regurgitant
balanced atrioventricular septal defects and single-ventricle physiology or stenotic valve
also develop significant atrioventricular valve regurgitation over time.105
For patients with systemic atrioventricular valve regurgitation, the ACHD Tricuspid valve
guidelines4 recommend extrapolation of the American College of Car-
diology/American Heart Association guidelines for the management of Primary tricuspid valve dysfunction in patients with CHD is often
valvular heart disease for mitral valve regurgitation, which include in- associated with Ebstein anomaly or congenital dysplasia, which may
terventions for severe regurgitation in the presence of symptoms (heart result in chronic severe TR.115,116 Secondary TR occurs with progressive
failure, dyspnea, and decreased exercise tolerance), or in asymptomatic RV and tricuspid annular dilation, which is usually due to RV volume or
patients with evidence of left ventricular dysfunction.106 pressure overload. The indications for intervention include regurgitation
Although surgery for repairing or replacing severely regurgitant or stenosis with symptoms of right-heart failure as well as arrhythmias.4
atrioventricular valves is an acceptable strategy, some patients may be at Transcatheter tricuspid valve replacement (TTVR) is feasible, and
a prohibitively high surgical risk, and percutaneous transcatheter edge- there is growing evidence for the use of the Melody and Sapien valves in
to-edge repair has been successful in this patient population.106-108 The both surgical rings and failing tricuspid valve bioprostheses.116-119 The
use of MitraClips (Abbott) has been established as an effective therapy in procedural success rate is high, and short-term hemodynamic benefits are
adult patients with primary and secondary mitral valve regurgita- evident. A registry of 306 patients undergoing TTVR demonstrated a
tion107,108 but has also been demonstrated to be effective in a select cumulative 3-year incidence of death of 17%, reintervention rate of 12%,
group of patients with CHD, mainly those with systemic atrioventricular and valve-related adverse outcomes (endocarditis, thrombosis, or
valves (dextro-TGA [D-TGA]) and atrial switch or those with congenitally dysfunction) in 8% of the patients. Eight patients (2.6%) developed valve
corrected TGA with systemic TR) or a common atrioventricular thrombosis during study follow-up.118 Perivalvular regurgitation is
valve.109,110 Experience with this technology is still limited in the common when TTVR is performed in surgical bands and rings but is mild
congenital space, and the procedure can be technically challenging, in most cases and can be frequently managed with a variety of trans-
especially given the differences in anatomy and the need for excellent catheter occlusion devices.117
transesophageal echocardiographic images to guide the intervention.111 Several devices are being developed for transcatheter replacement of
There have been <25 cases reported in the literature so far.109,112,113 native tricuspid valves, such as the EVOQUE valve120 (Edwards Life-
Nevertheless, it is a new technique that has the potential to help many sciences). Clinical trials are ongoing to evaluate the efficacy of the device
(NCT04482062) in the treatment of functional TR.

Mitral valve

Congenital mitral valve disease results in stenotic, regurgitant, or

mixed disease. Stenotic lesions of the mitral valve can occur at the supra-
annular, annular, valvular, or subvalvular level, such as in the cor tria-
triatum, supramitral ring, double-orifice mitral valve, parachute mitral
valve, or hammock mitral valve.116 Regurgitant lesions are usually due to
issues of the mitral valve apparatus, such as in partial or complete
atrioventricular septal defects, myxomatous mitral valve leaflets, or flail
or ruptured chordae. Secondary mitral valve regurgitation usually occurs
in the setting of progressive annular dilation because of poor ventricular
function or cardiomyopathy.
The field of transcatheter mitral valve replacement is still in its early
phase. However, many devices in early clinical trials have shown
encouraging results, especially in patients who are too high risk of sur-
gery.121,122 Most procedures are performed in the valve-in-valve and
valve-in-ring population123 and in those with calcified mitral annuli to
allow for anchoring, most commonly using the Melody and Sapien
valves.116,123 Preprocedural imaging is important for predicting and
evaluating strategies to mitigate the risk of left ventricular outflow tract
Figure 5. Alterra prestent and Sapien valve deployment. (A) A lateral pro- (LVOT) obstruction by the valve scaffold. The procedure can often be
jection of a right ventriculogram demonstrating a dilated pulmonary artery that performed via the transseptal approach; TEE is required for procedural
is suitable for an Alterra prestent. (B) Three-dimensional rendering using imaging guidance.123,124 Although technical procedural success is usu-
computed tomography angiogram data simulating placement of the Alterra
ally very high (95% of cases), the use of these valves in transcatheter
prestent within the dilated pulmonary artery. (C) A lateral projection of the
mitral valve replacement has been limited to patients who are at a pro-
deployed Alterra prestent in a stable position as well as the Edwards Sapien S3
valve within the Alterra prestent. (D) A computed tomography angiogram of the hibitively high risk of surgery because the outcomes of transcatheter
pulmonary artery in a simulated lateral projection after the prestent and valve mitral valve replacement are relatively poor (with a 30-day mortality rate
have been deployed demonstrating stable position of both the valve within the of 8.2%), mainly because of LVOT obstruction.125 Trials for the devel-
prestent and the prestent within the pulmonary artery. MPA, main pulmonary opment of transcatheter valve delivery systems meant specifically for the
artery; RV, right ventricle; S3, Edwards Sapien S3 valve. mitral valve are currently ongoing for both transapical and transseptal

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

approaches.122,124 As the technology improves, widespread adoption of Aortic interventions

this technology as an alternative to conventional surgery may be the
future. Balloon angioplasty and stenting of coarctation of the aorta

Coarctation of the aorta (CoA) is a narrowing of the descending aorta,

Aortic valve replacement usually at the insertion point of the ductus arteriosus, just distal to the left
subclavian artery. CoA has a prevalence of 2.7 to 4.4 per 10,000 children,
Aortic stenosis or aortic regurgitation (AR) occurs in many accounting for 2% to 5% of all congenital heart defects.12,60 Most cases
different congenital cardiac conditions and can be due to primary occur sporadically but are also associated with certain genetic conditions,
valvular lesions or secondary dysfunction. The spectrum of clinical such as Turner syndrome (in as many as 5%-15% of women with CoA),133
disease of AS varies from the bicuspid aortic valve causing isolated and are often seen in conjunction with other congenital heart defects,
disease to complex lesions, such as hypoplastic left-heart syndrome, with the most common being the bicuspid aortic valve (in 30%-50% of
which is associated with mitral valve hypoplasia or atresia in addition patients with CoA).134 Up to 10% of adults with bicuspid aortic valve and
to aortic valve stenosis or atresia. The estimated prevalence of CoA are found to have intracranial aneurysms.135
congenital AS is between 1.1 to 4.9 per 10,000 live births.12,60 AR Depending on the severity of coarctation, patients can present at birth
can occur in conjunction with AS in patients with degenerative with heart failure and/or shock when the ductus arteriosus starts to close. If
bicuspid aortic valves or in isolation because of secondary aortic root the coarctation is not as severe, then patients may be asymptomatic and can
dilation. AR can also occur because of aortic valve prolapse in present later in childhood or even in adulthood. Careful history taking may
conjunction with membranous VSDs or subvalvular-aortic stenosis. In reveal chest pain, cold extremities, and claudication with physical exertion.
patients with a history of aortic valve surgery with an autograft (Ross Adults with CoA most often present with hypertension. The natural history
procedure), homograft, or bioprosthetic valve, all of these valves can of untreated CoA is quite grim, with an average survival of 35 years and a
degenerate and develop AS and/or AR. Prosthetic aortic valves, mortality of 75% by 46 years of age.136 Surgical or transcatheter treatment
especially in patients with ACHD, degenerate more rapidly in young of CoA is recommended if patients have hypertension and a significant
adults than in older patients.126 When patients become symptomatic, gradient across the coarctation (an upper or lower extremity resting cath-
develop decreased LV function or objectively decreased exercise eterization peak-to-peak gradient of >20 mm Hg, a mean echo Doppler
tolerance, or have severe AR with increasing LV size, intervention is gradient of >20 mm Hg, an upper or lower extremity resting peak-to-peak
recommended.106 gradient of >10 mm Hg, a mean Doppler gradient of >10 mm Hg in the
Transcatheter aortic valve replacement has become a widely used presence of decreased LV function, AR, or collateral flow).4 When CoA is
alternative to surgical aortic valve replacement predominantly in pa- treated early on prior to the development of significant complications, the
tients with calcific aortic valve stenosis, with overall good outcomes.127 long-term survival is excellent, reaching up to 90% at 20 years in a study of
The potential complications of TAVR include perivalvular leaks, con- patients with childhood cardiac interventions.137
duction abnormalities, thromboembolism, stroke, valve embolization, For adult patients with CoA, transcatheter treatment is usually the
aortic annular rupture, coronary occlusion, renal injury, bleeding, and first line, although a surgical approach may be preferred for hypoplastic
vascular access injury.128 TAVR in patients with AS due to the bicuspid aortic arch, genetic conditions such as Turner syndrome and other
aortic valve has been shown to be feasible and effective, with favorable connective tissue disorders, or other congenital cardiac lesions
valve performance, even in low-risk patients,129 but does have an requiring treatment. Transcatheter balloon angioplasty of aortic
increased risk of stroke, pacemaker implantation, and perivalvular coarctation was initially performed in 1982 by Singer et al138 and has
leaks,130-132 especially in patients with significantly calcified bicuspid been performed for the treatment of discrete native and recurrent
aortic valves.131 For patients with primarily AR, TAVR is generally not narrowing of the descending aorta, with a high immediate success rate;
recommended with the currently available devices because the lack of however, aortic wall complications occurred in 10% of patients and
leaflet calcification makes valve anchoring difficult; the Jena valve is in reobstruction occurred in 32% of patients.139 Thus, the preferred
clinical trials (NCT04415047). method of transcatheter treatment of CoA is stent implantation
Transcatheter aortic valve replacement has also been used in pa- (Figure 6), which was first reported by de Lezo et al140 in 1995. Open-
tients with D-TGA after arterial switch and valve sparing aortic root and closed-cell-design stents as well as covered stent platforms are now
repair with recurrent AR116 and in those with existing bioprosthetic widely used in preference to balloon angioplasty alone. The immediate
aortic valves.123 As technology improves, more and more patients un- results are excellent, with a low risk of complications (<5%) and a low
dergoing TAVR are receiving moderate sedation and going home in 1 to risk of aortic wall injury (3.1%). The intermediate reobstruction rate is
3 days, with >95% of all procedures being performed via the trans- 15%; however, the majority of these were only mild restenosis.139 The
femoral approach.128 Nevertheless, given the various anatomic varia- intermediate results of 1 study demonstrated that 4% of patients with
tions and considerations, such as aortic root size, coronary artery stenting of the CoA underwent an unplanned reintervention because of
anomalies, and aortic valve or annular calcification, a nuanced heart recurrent coarctation or pseudoaneurysm formation, with an average
team approach should be used for each congenital cardiac case with AS time to reintervention of 2.84 years, whereas another study found a
or AR to evaluate whether TAVR or surgical aortic valve replacement cumulative aneurysm incidence of 6% at 5 years.9,139 The availability of
would be the best option for that patient at that point in their life, covered stents (balloon-expandable or self-expanding) platforms may
especially because most patients require several interventions over the further improve the safety profile of stents, reduce catastrophic aortic
course of their lifespan.132 wall injury, and allow the treatment of CoA-associated pseudoaneur-
A critical benefit of multidisciplinary team discussion involving ysms or aneurysms.141,142
ACHD interventionalists with experience in TAVR and surgeons includes
surgical planning to increase the chances of successful valve-in-valve
TAVR in the future (such as the size of implanted surgical prosthesis PA interventions
and coronary distance), which may also impact the feasibility of valve-in-
valve TAVR vs surgical aortic valve replacement as the next procedure. Balloon angioplasty and stenting of PA stenosis
Newer devices for TAVR are constantly under development with the aim
to improve deliverability, durability, deployment, and positioning. This Stenosis of the proximal or branch PAs may occur in isolation but is
will hopefully allow TAVR technology to expand to congenital patients usually associated with other cardiac lesions such as tetralogy of Fallot,
with predominant AR without calcific AS.116 Alagille syndrome, Williams syndrome, Noonan syndrome, or congenital

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

Figure 6. Stenting of a coarctation of the aorta. (A) 3-dimensional rendering using a preprocedural computed tomography angiogram of the chest. There is a tight
coarctation of the aorta that appears to be remote from the left subclavian artery. (B) Lateral fluoroscopic projection of an aortogram demonstrating a tortuous,
discrete coarctation. (C) Placement of a covered stent across the coarctation with resolution of the waist. (D) Aortogram after stent deployment demonstrating no
residual coarctation and no evidence of dissection, hematoma, or rupture of the aorta.

rubella syndrome.143,144 Branch PA stenosis often occurs following sur- ventricle status were significantly associated with the risk of any
gical shunt placement. The diagnosis of peripheral PA stenosis in adults is adverse event, which included vessel rupture, stent embolization, and
rare but is underrecognized as a cause of right ventricular hypertension, death.145 Another study reported a complication rate of 3%, with no
progressive dyspnea, and fatigue on exertion.144 deaths in their cohort of 183 patients.148
The indication for the treatment of PA stenosis is when the lesion
results in elevation of right ventricular systolic pressure or reduces
perfusion to a lung segment distal to the stenosis.145 Surgical repair of PA Special populations
stenosis is possible; however, the results are frequently suboptimal,
particularly if the lesions are peripheral. Lock et al146 described a Patients with single-ventricle physiology (Fontan procedure)
percutaneous static balloon angioplasty technique for the treatment of
peripheral PA stenosis in 1983. An adequate result depends on the use of Univentricular heart defects are rare and account for 1 to 1.5 per
sufficiently large high-pressure balloons that tear the vascular intima and 10,000 live births in some studies.12,60 Over the past 3 decades, advances
a part of the media, leaving a slim safety margin for this procedure.147 in surgical techniques and refinements in the Fontan operation procedure
Elastic recoil of the PA segment undergoing balloon angioplasty is have led to improved short- and medium-term survival of patients with
common, hence prompting the use of stents.145 The success rate of single-ventricle physiology.149 The overall survival rates at 20 years after
transcatheter intervention for peripheral PA stenosis is increased from the Fontan procedure are as high as 87% in some cohorts. Nevertheless,
70% with balloon angioplasty alone to 90% with the use of stents.148 In freedom from late complications of the Fontan procedure is low, and in 1
general, the first and second arcade branches of the PAs are usually study, it was observed that 50% of patients with Fontan circulation
effectively treated with stents. Diffuse peripheral PA stenosis can be a experienced a complication related to the Fontan circuit over a period of
very difficult problem, for which there is no surgical option (short of lung 20 years.149 In order to improve survival and quality of life, many of
transplantation). Patients with multiple distal stenoses often require these adult patients with Fontan circulation undergo catheter-based in-
multiple balloon dilations.145 In some patients, thorough and aggressive terventions for the treatment of these complications. We will highlight a
dilation of these stenoses can lead to lasting and significant improvement few of these interventions and the reasons for these interventions.
of right ventricular pressure and function; however, care must be taken to Patients with obstructions in the Fontan pathway can have significant
avoid rupture of the distal PA vasculature. Proximal branch PA stenoses symptoms because they lack a subpulmonary ventricle. Their pulmonary
are also difficult to treat because of PA bifurcation. V-stenting or stenting blood flow relies on a high systemic venous pressure driving flow
the branch PA through a side strut of the first stent in a modified culotte through a low-resistance circuit, in addition to respirophasic fluctuations
technique are options for the treatment of the bifurcation.145 in pressure; any obstruction in this circuit, even with only a low (1-3 mm
There are patients who have had stents placed in their PAs during Hg) gradient, may be hemodynamically significant. Thus, interventions
childhood but have not had further dilation of the stents since and now such as balloon angioplasty or stenting of the Fontan circuit, the branch
present with stenosis at the site of stent implantation because of somatic PAs, or residual aortic obstructions such as CoA, are often performed for
growth. These stents either need to be dilated or intentionally fractured the treatment of heart failure or complications of a failing Fontan circuit,
using high-pressure balloon inflation to reach an appropriate adult such as protein-losing enteropathy, plastic bronchitis, or Fontan-
size.145 In cases of bilateral PA stenoses, lung perfusion scans or cardiac associated liver disease. These interventions have been previously
MRI imaging may be helpful to assess the distribution of flow. described in earlier sections.
Complications with the procedure are uncommon; however, major In certain patients with Fontan circulation, transseptal punctures are
complications occurred in approximately 9% of all PA stenting proced- necessary in order to access the pulmonary venous atrium (eg, for an
ures in the IMproving Pediatric and Adult Congenital Treatments regis- electrophysiologic study or creation of an atrial-level shunt).150,151
try. A patient weight of <4 kg, emergency procedures, and single- Although the techniques for transseptal punctures are varied, the use of

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W. Tan et al. Journal of the Society for Cardiovascular Angiography & Interventions 1 (2022) 100438

TEE or intracardiac echocardiography is important to help with a safe removal of pacing wires, stenting, and replacement of the pacing wire is
puncture.21 necessary.164 Pulmonary venous baffle obstructions are also seen to occur
The transcatheter creation of a Fontan fenestration was described as de novo or following compression due to stenting of a systemic venous
early as 1997 to aid in improving cardiac output and decreasing central baffle limb. Sometimes, stents are required for both systemic venous and
venous pressure at the expense of systemic desaturation.152 The tech- pulmonary venous baffles to relieve obstructions in each pathway.165
nique of using a transseptal puncture needle to cross into the pulmonary Three-dimensionally printed models can be made to better understand
venous atrium and the use of serial balloon dilation over a wire to allow the complex intracardiac anatomy of these patients and help with plan-
for a long sheath to cross for the delivery of a stent has been well ning an intervention.166
established.153 However, successful variations of this technique, such as Some patients with D-TGA treated with an atrial-switch procedure
with the new atrial flow regulator (Occlutech), for the creation of a stable have LVOT obstructions or proximal PA stenosis. In most cases, a mild or
fenestration154 as well as creation of a fenestration from the PA to the moderate degree of obstruction is hemodynamically advantageous
pulmonary venous atrium in patients with unusually thick Fontan con- because the elevated subpulmonary left ventricular pressure maintains
duits155 have been described. the interventricular septum in a midline position that leads to a favorable
Many patients with Fontan circulation eventually develop heart failure interventricular interaction, a reduced right ventricular size, and less TR.
and are referred for heart transplantation or even combined heart-liver Acute systemic right ventricular dysfunction and worsening of TR after
transplantation. Preoperative hemodynamic catheterization includes the the treatment of LVOT obstructions have been described,167 and, thus,
assessment of the Fontan pathway, as described above, evaluation and intervention for subpulmonic obstructions in these patients should be
coiling or embolization of large aortopulmonary or venovenous collaterals reserved for severe or critical stenosis or left ventricular dysfunction.
that may lead to life-threatening intraoperative bleeding, and, in some
cases, transjugular liver biopsy for risk stratification (in addition to
noninvasive methods such as hepatic FibroScan). Agitated saline in- Pregnant patients
jections in the Fontan circuit and in the branch PAs, with transthoracic
echocardiogram during the procedure, are useful for evaluating right-to- Patients with ACHD have more maternal and fetal morbidity and
left shunts in patients with cyanotic Fontan circulation. The treatment of mortality than women without CHD168; during pregnancy, the cardiac
venovenous collaterals improves cyanosis156 and prevents perioperative output and intravascular volume increase, which can lead to heart failure
bleeding at the time of transplantation but at the expense of increased as well as atrial and ventricular arrhythmias.169 Because pregnancy leads
systemic venous pressure and reduction in systemic ventricular preload. to increased cardiac output and circulating blood volume, the hemody-
Some studies have shown an increase in long-term mortality after these namics of existing lesions can change to the point where patients can
procedures157; so, the coiling of venovenous collaterals needs to be well become symptomatic. Although it is rare for a congenital patient to need
timed with the transplant listing. Aortopulmonary collaterals increase a transcatheter intervention during pregnancy, there have been reports of
pulmonary blood flow but chronically volume load the single ventricle, patients needing coarctation stenting170 and ASD closure.171 Should
which may trigger ventricular remodeling and increased end-diastolic there be a need for a transcatheter intervention during pregnancy, great
pressure. This eventually leads to ventricular dysfunction and failure. care should be taken to shield the fetus from any radiation and minimize
Although the practice variation varies across institutions,158 coiling of radiation exposure during the case.169 The use of imaging techniques,
aortopulmonary collaterals is a very common procedure to relieve over- such as intracardiac echocardiography and TEE, may be helpful in
circulation, treat hemoptysis, and prepare patients for transplantation by reducing the amount of fluoroscopy needed for such interventions.
reducing their risk of perioperative bleeding.159,160 After device implantation, most patients require antiplatelet therapy
Percutaneous lymphatic embolization is a novel and specialized or anticoagulation. Although aspirin is well tolerated during pregnancy,
technique to identify pathways of lymphatic decompression and occlude patients who require additional antiplatelet agents, such as clopidogrel,
these abnormal channels to treat protein-losing enteropathy and plastic or anticoagulation may need to alter their medication regimen during
bronchitis.161 Only a few centers are capable of specializing in these pregnancy.172,173 The special considerations may include stopping clo-
interventions because they require magnetic resonance lymphangio- pidogrel 7 days prior to delivery to allow for the possibility of epidural or
grams162; however, these procedures can be very effective. spinal catheter placement for analgesia delivery.173 There is wide prac-
tice variation among interventionalists while deciding on postprocedural
antiplatelet and/or anticoagulation regimens after an intervention, and,
D-TGA with atrial switch (as well as L-TGA with double switch) thus, more studies are needed to shed light on this important decision.

There remains a large population of patients with D-TGA who have

undergone an atrial-switch operation, with the creation of a baffle redi- Hybrid procedures
recting systemic venous return to the subpulmonary ventricle with the
use of native atrial tissue (Senning procedure) or a prosthetic patch Many patients with ACHD are at an increased surgical risk because
(Mustard procedure). There is also a population of young adult patients of a history of multiple sternotomies, collateral formation, lung disease,
with congenitally corrected TGA who have undergone a double-switch kyphosis, or poor nutritional status. Because of the limitations of the
procedure (arterial switch and atrial switch) during early childhood to currently available transcatheter technologies, a hybrid approach is
allow the LV to be the systemic ventricle and prevent complications sometimes needed to perform safe interventions in these patients.
related to a failing systemic RV.163 Baffle leaks may occur and cause Hybrid approaches have long been a staple of congenital interventions
cyanosis or lead to paradoxical emboli due to right-to-left shunting. in the pediatric world, mainly because of the small size of vascular
Baffle leak closure with cribriform septal defect or PFO closure devices is structures in children, which limits the use of vascular access to deliver
indicated in symptomatic patients or those with right-sided pacing wires, interventional equipment.174 Hybrid approaches, such as stenting of the
which increase the risk of paradoxical emboli. ductus arteriosus, concomitant banding of the PAs, closure of VSDs via a
Stenosis of the systemic venous baffle limbs is also seen because of right ventricular puncture, or transcatheter valve replacement via
progressive fibrosis or thrombosis; in some cases, it is associated with ventricular access, have been described.174 In adults with CHD, hybrid
pacing wires. Symptoms may arise with low gradients in the venous approaches have been used to perform transcatheter valve replacement
circulation, making careful hemodynamic and angiographic assessments of the pulmonary valve in patients with large RVOTs,175,176 trans-
particularly important. Balloon dilation followed by stenting can be catheter mitral valve replacements in those with difficult anatomy for a
effective in relieving stenosis. In some cases, a hybrid procedure with transseptal approach,177 or even for paravalvular leak occlusion of the

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mitral valve via transapical access.178,179 Although these procedures 2. Bonhoeffer P, Boudjemline Y, Qureshi SA, et al. Percutaneous insertion of the
pulmonary valve. J Am Coll Cardiol. 2002;39(10):1664–1669. https://doi.org/
sometimes require sternotomy or a smaller chest incision, the use of
10.1016/S0735-1097(02)01822-3
cardiopulmonary bypass is minimized with hybrid procedures, which 3. Lui GK, Saidi A, Bhatt AB, et al. Diagnosis and management of noncardiac
leads to reduced blood transfusion requirements compared with those complications in adults with congenital heart disease: a scientific statement from
for surgery.175 Nevertheless, hybrid procedures can lead to increased the American Heart Association. Circulation. 2017;136(20):e348–e392.
4. Stout KK, Daniels CJ, Aboulhosn JA, et al. AHA/ACC guideline for the management
bleeding compared with just transcatheter procedures, and 1 series had of adults with congenital heart disease: a report of the American College of
a bleeding complication rate of 19% at 30 days.179 However, hybrid Cardiology/American Heart Association Task Force on Clinical Practice guidelines.
procedures can be a feasible option for certain high-risk patients with J Am Coll Cardiol. 2019;73(12):e81–e192. https://doi.org/10.1016/
j.jacc.2018.08.1029
ACHD who need interventions, especially at centers with a strong 5. Aboulhosn JA, Hijazi ZM, Kavinsky CJ, et al. SCAI position statement on adult
working relationship between the interventional cardiologist and congenital cardiac interventional training, competencies and organizational
cardiothoracic surgeon.180 recommendations. Catheter Cardiovasc Interv. 2020;96(3):643–650. http://www.
ncbi.nlm.nih.gov/pubmed/32272495
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defect in children and adults: results of a multicenter nonrandomized trial. J Am
Coll Cardiol. 2002;39(11):1836–1844. https://doi.org/10.1016/S0735-1097(02)
Dr Horlick reports a relationship with Abbott Cardiovascular Struc- 01862-4
tural Heart Division that includes consulting or advisory, funding grants, 17. Silvestry FE, Cohen MS, Armsby LB, et al. Guidelines for the echocardiographic
assessment of atrial septal defect and patent foramen ovale: from the American
and speaking and lecture fees; Medtronic that includes funding grants; Society of Echocardiography and Society for Cardiac Angiography and
and Edwards Lifesciences Corp that includes funding grants. Dr Aboul- Interventions. J Am Soc Echocardiogr. 2015;28(8):910–958. https://doi.org/
hosn reports a relationship with Edwards Lifesciences Corp that includes 10.1016/j.echo.2015.05.015
18. Silvestry FE, Kerber RE, Brook MM, et al. Echocardiography-guided interventions.
consulting or advisory, funding grants, speaking and lecture fees, and J Am Soc Echocardiogr. 2009;22(3):213–231;quiz 316-7. https://doi.org/10.1016/
travel reimbursement; Medtronic that includes consulting or advisory, j.echo.2008.12.013.
speaking and lecture fees, and travel reimbursement; and Abbott Car- 19. Hasco€et S, Warin-Fresse K, Baruteau AE, et al. Cardiac imaging of congenital heart
diseases during interventional procedures continues to evolve: pros and cons of the
diovascular Structural Heart Division that includes consulting or advi- main techniques. Arch Cardiovasc Dis. 2016;109(2):128–142. https://doi.org/
sory, speaking and lecture fees, and travel reimbursement. He is an 10.1016/j.acvd.2015.11.011
Editorial Board member of Adult Congenital Heart Association. Drs Tan 20. Rigatelli G. Expanding the use of intracardiac echocardiography in congenital heart
disease catheter-based interventions. J Am Soc Echocardiogr. 2005;18(11):
and Schmidt reported no financial interests. 1230–1231.
21. Basman C, Parmar YJ, Kronzon I. Intracardiac echocardiography for structural
Funding sources heart and electrophysiological interventions. Curr Cardiol Rep. 2017;19(10):
102–103.
22. Sommer RJ, Love BA, Paolillo JA, et al. ASSURED clinical study: new GORE®
This research did not receive any specific grant from funding agencies CARDIOFORM ASD occluder for transcatheter closure of atrial septal defect.
in the public, commercial, or not-for-profit sectors. Catheter Cardiovasc Interv. 2020;95(7):1285–1295.
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defects with the AMPLATZER septal occluder: a prospective, multicenter, post-
Ethics statement approval study. Circ Cardiovasc Interv. 2017;10(8):1–7.
24. Kumar P, Orford JL, Tobis JM. Two cases of pericardial tamponade due to nitinol
wire fracture of a gore septal occluder. Catheter Cardiovasc Interv. 2020;96(1):
The research reported has adhered to the relevant ethical guidelines.
219–224.
25. Amin Z, Hijazi ZM, Bass JL, Cheatham JP, Hellenbrand WE, Kleinman CS. Erosion
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