HCM Guidelines Slide Set GL HCM

2020 AHA/ACC Guideline for the Diagnosis and
Treatment of Patients With Hypertrophic

Cardiomyopathy
Developed in collaboration with and endorsed by the American Society of Echocardiography,

American Academy for Thoracic Surgery, Heart Failure Society of America, Heart Rhythm Society,
Society for Cardiovascular Angiography and Interventions, and Society for Cardiovascular
Magnetic Resonance
Citation
This slide set is adapted from the 2020 AHA/ACC Guideline for the Diagnosis and Treatment of
Patients with Hypertrophic Cardiomyopathy. Published online ahead of print November 20, 2020,
available at: Circulation. https://www.ahajournals.org/doi/10.1161/CIR.0000000000000937
and Journal of the American College of Cardiology published online ahead of print Nov. 20, 2020.
J Am Coll Cardiol. http://www.jacc.org/doi/10.1016/j.jacc.2020.08.045
2020 Writing Committee Members*
Steve R. Ommen, MD, FACC, FAHA, Chair†
Seema Mital, MD, FACC, FAHA, FRCPC, Vice Chair†
Michael A. Burke, MD† Michelle Kittleson, MD, PhD, FACC†

Sharlene M. Day, MD† Mark S. Link, MD, FACC¶
Anita Deswal, MD, MPH, FACC, FAHA‡§ Martin S. Maron, MD#
Perry Elliott, MD, FRCP, FACC† Matthew W. Martinez, MD, FACC†
Lauren L. Evanovich, PhD† Christina Y. Miyake, MD, MS†
Judy Hung, MD, FACC║ Hartzell V. Schaff, MD, FACC**
José A. Joglar, MD, FACC, FAHA† Christopher Semsarian, MBBS, PhD, MPH, FAHA†
Paul Kantor, MBBCh, MSc, FRCPC† Paul Sorajja, MD, FACC, FAHA, FSCAI††
Carey Kimmelstiel, MD, FACC, FSCAI†
*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. †ACC/AHA Representative. ‡ACC/AHA Joint Committee on Clinical Practice Guidelines Liaison. §HFSA Representative. ║ASE
Representative. ¶ HRS Representative. # SCMR Representative. **AATS Representative. ††SCAI Representative. ‡‡Former Joint Committee on Clinical Practice
Guidelines member; current member during the writing effort.
3
Table 2. ACC/AHA Applying Class of
Recommendation and Level of
Evidence to Clinical Strategies,
Interventions, Treatments, or
Diagnostic Testing in Patient Care
(Updated May 2019)*
4
Top 10 Take-Home Messages
2020 Hypertrophic Cardiomyopathy Guidelines
5
Top 10 Take Home Messages
1.Shared decision-making, a dialogue between patients and their care team that
includes full disclosure of all testing and treatment options, discussion of the
risks and benefits of those options and, importantly, engagement of the
patient to express their own goals, is particularly relevant in the management
of conditions such as hypertrophic cardiomyopathy (HCM).
6
2. Although the primary cardiology team can initiate evaluation, treatment, and
longitudinal care, referral to multidisciplinary HCM centers with graduated
levels of expertise can be important to optimizing care for patients with HCM.
Challenging treatment decisions—where reasonable alternatives exist, where the
strength of recommendation is weak (e.g., any Class 2b decision) or is
particularly nuanced, and for invasive procedures that are specific to patients
with HCM—represent crucial opportunities to refer patients to these HCM
centers.
7
3. Counseling patients with HCM regarding the potential for genetic transmission
of HCM is one of the cornerstones of care. Screening first-degree family
members of patients with HCM, using either genetic testing or an
imaging/electrocardiographic surveillance protocol, can begin at any age and
can be influenced by specifics of the patient/family history and family
preference. As screening recommendations for family members hinge on the
pathogenicity of any detected variants, the reported pathogenicity should be
reconfirmed every 2 to 3 years.
8
4. Optimal care for patients with HCM requires cardiac imaging to confirm the diagnosis,
characterize the pathophysiology for the individual, and identify risk markers that may
inform decisions regarding interventions for left ventricular outflow tract obstruction and
SCD prevention. Echocardiography continues to be the foundational imaging modality
for patients with HCM. Cardiovascular magnetic resonance imaging will also be helpful
in many patients, especially those in whom there is diagnostic uncertainty, poor
echocardiographic imaging windows, or where uncertainty persists regarding decisions
around ICD placement.
9
5. Assessment of an individual patient’s risk for SCD continues to evolve as new

markers emerge (e.g., apical aneurysm, decreased left ventricular systolic
function, and extensive gadolinium enhancement). In addition to a full
accounting of an individual’s risk markers, communication with patients
regarding not just the presence of risk markers but also the magnitude of their
individualized risk is key. This enables the informed patient to fully participate
in the decision-making regarding ICD placement, which incorporates their own
level of risk tolerance and treatment goals.
10
6. The risk factors for SCD in children with HCM carry different weights than
those observed in adult patients; they vary with age and must account for
different body sizes. Coupled with the complexity of placing ICDs in young
patients with anticipated growth and a higher risk of device complications,
the threshold for ICD implantation in children often differs from adults.
These differences are best addressed at primary or comprehensive HCM
centers with expertise in children with HCM.
11
7. Septal reduction therapies (surgical septal myectomy and alcohol septal

ablation), when performed by experienced HCM teams at dedicated centers,
continue to improve in safety and efficacy such that earlier intervention may
be possible in select patients with drug-refractory or severe outflow tract
obstruction causing signs of cardiac decompensation. Given the data on the
significantly improved outcomes at comprehensive HCM centers, these
decisions represent an optimal referral opportunity.
12
8. Patients with HCM and persistent or paroxysmal atrial fibrillation have a

sufficiently increased risk of stroke such that oral anticoagulation with direct
oral anticoagulants (or alternatively warfarin) should be considered the
default treatment option independent of the CHA 2DS2VASc score. As rapid
atrial fibrillation is often poorly tolerated in patients with HCM, maintenance
of sinus rhythm and rate control are key pursuits in successful treatment.
13
9. Heart failure symptoms in patients with HCM, in the absence of left

ventricular outflow tract obstruction, should be treated similarly to other
patients with heart failure symptoms, including consideration of advanced
treatment options (e.g., cardiac resynchronization therapy, left ventricular
assist device transplantation). In patients with HCM, an ejection fraction
<50% connotes significantly impaired systolic function and identifies
individuals with poor prognosis and who are at increased risk for SCD.
14
10. Increasingly, data affirm that the beneficial effects of exercise on general health can
be extended to patients with HCM. Healthy recreational exercise (moderate intensity)
has not been associated with increased risk of ventricular arrhythmia events in recent
studies. Whether an individual patient with HCM wishes to pursue more rigorous
exercise/training is dependent on a comprehensive shared discussion between that
patient and their expert HCM care team regarding the potential risks of that level of
training/participation but with the understanding that exercise-related risk cannot be
individualized for a given patient.
15
Shared Decision Making
16
Shared Decision-Making
COR LOE Recommendation
1. For patients with HCM or at risk for HCM, shared decision-making is
recommended in developing a plan of care (including but not limited to decisions
regarding genetic evaluation, activity, lifestyle, and therapy choices) that includes
1 B-NR
a full disclosure of the risks, benefits, and anticipated outcomes of all options, as
well the opportunity for the patient to express their goals and concerns.
17
Multidisciplinary Hypertrophic
Cardiomyopathy Centers
18
Multidisciplinary Hypertrophic Cardiomyopathy Centers
COR LOE Recommendations

1. In patients with HCM in whom SRT is indicated, the procedure should
be performed at experienced centers (comprehensive or primary HCM
1 C-LD centers) with demonstrated excellence in clinical outcomes for these
procedures.
2. In patients with HCM, consultation with or referral to a

comprehensive or primary HCM center is reasonable to aid in
2a C-LD
complex disease-related management decisions.
19
Diagnosis, Initial Evaluation, and Follow-up
20
Clinical Diagnosis

1. In patients with suspected HCM,
comprehensive physical examination and
complete medical and 3-generation family
1 B-NR
history is recommended as part of the initial
diagnostic assessment.
21
Table 5. Clinical Features in Patients with “HCM* Phenocopies (Mimics)”
Typical Presentation
Systemic Features Possible Etiology Diagnostic Approach
Age
Infants (0-12 mo) and Dysmorphic features, failure to thrive,  RASopathies  Geneticist assessment
toddlers metabolic acidosis  Glycogen storage diseases, other  Newborn metabolic screening
metabolic or mitochondrial diseases  Specific metabolic assays
 Infant of a mother with diabetes  Genetic testing
Early childhood Delayed or abnormal cognitive  RASopathies  Biochemical screening

development, visual or hearing impairment  Mitochondrial diseases  Genetic testing

School age and Skeletal muscle weakness or movement  Friedrich ataxia, Danon disease  Biochemical screening
adolescence disorder  Mitochondrial disease  Neuromuscular assessment
 Genetic testing
Adulthood Movement disorder, peripheral neuropathy,  Anderson-Fabry disease, Friedrich  Biochemical screening,
renal dysfunction ataxia, infiltrative disorders (e.g.,  Neuromuscular assessment
amyloidosis), glycogen storage diseases  Genetic testing
*HCM indicates hypertrophic cardiomyopathy

22
Figure 1. Evaluation and Testing for HCM
Colors correspond to the Class of

Recommendation
*The interval may be extended,
particularly in adult patients who
remain stable after multiple
evaluations.

CMR indicates cardiovascular magnetic
resonance; CPET, cardiopulmonary
exercise test; ECG,
electrocardiography/electrocardiogram
; HCM, hypertrophic cardiomyopathy;
HF, heart failure; ICD, implantable
cardioverter-defibrillator; LVOTO, left
ventricular outflow tract obstruction;
P/LP, pathogenic or likely pathogenic
variant; SCD, sudden cardiac death;
and VUS, variant of unknown
significance.
23
Figure 1. cont. Screening Asymptomatic First-Degree Relatives of Patients with HCM
24
Echocardiography
1. In patients with suspected HCM, a transthoracic echocardiogram (TTE)
1 B-NR
is recommended in the initial evaluation.
B-NR 2. In patients with HCM with no change in clinical status or events, repeat
children TTE is recommended every 1 to 2 years to assess the degree of
1 myocardial hypertrophy, dynamic left ventricular outflow tract

C-LD obstruction (LVOTO), mitral regurgitation (MR), and myocardial
adults function.
3. For patients with HCM who experience a change in clinical status or a

1 B-NR new clinical event, repeat TTE is recommended.
25
Echocardiography
4. For patients with HCM and resting left ventricular outflow tract (LVOT) gradient
1 B-NR <50 mm Hg, a TTE with provocative maneuvers is recommended.
5. For symptomatic patients with HCM who do not have a resting or provocable outflow
tract gradient ≥50 mm Hg on transthoracic echocardiogram (TTE), exercise TTE is
1 B-NR recommended for the detection and quantification of dynamic left ventricular
outflow tract obstruction (LVOTO).
6. For patients with HCM undergoing surgical septal myectomy, intraoperative

transesophageal echocardiogram (TEE) is recommended to assess mitral valve
1 B-NR
anatomy and function and adequacy of septal myectomy.
26
Echocardiography
7. For patients with HCM undergoing alcohol septal ablation,
transthoracic echocardiogram (TTE) or intraoperative trans-
1 B-NR esophageal echocardiogram (TEE) with intracoronary ultrasound-
enhancing contrast injection of the candidate septal perforator(s) is
recommended.
8. For patients with HCM who have undergone septal reduction

therapy (SRT), TTE within 3 to 6 months after the procedure is
1 B-NR
recommended to evaluate the procedural results.
27
Echocardiography
9. Screening: In first-degree relatives of patients with HCM, a
1 B-NR transthoracic echocardiogram (TTE) is recommended as part of

initial family screening and periodic follow-up.
10. Screening: In individuals who are genotype-positive or phenotype-

negative, serial echocardiography is recommended at periodic
1 B-NR intervals depending on age (1 to 2 years in children and
adolescents, 3 to 5 years in adults) and change in clinical status.
28
Echocardiography
11. For patients with HCM, trans-esophageal echocardiogram (TEE)
can be useful if transthoracic echocardiogram (TTE) is inconclusive
in clinical decision-making regarding medical therapy, and in
situations such as planning for myectomy, exclusion of subaortic
2a C-LD membrane or mitral regurgitation (MR) secondary to structural
abnormalities of the mitral valve apparatus, or in the assessment of
the feasibility of alcohol septal ablation.
29
Echocardiography
12. For patients with HCM in whom the diagnoses of apical HCM, apical aneurysm,
or atypical patterns of hypertrophy is inconclusive on transthoracic
2a B-NR echocardiogram (TTE), the use of an intravenous ultrasound-enhancing agent is
reasonable, particularly if other imaging modalities such as cardiovascular
magnetic resonance (CMR) are not readily available or contraindicated.
13. For asymptomatic patients with HCM who do not have a resting or provocable
outflow tract gradient ≥50 mm Hg on standard TTE, exercise TTE is reasonable
2a C-LD for the detection and quantification of dynamic left ventricular outflow tract
obstruction (LVOTO).
30
Table 6. Screening with Electrocardiography and 2D Echocardiography in Asymptomatic Family
Members*
Repeat ECG,
Age of First-Degree Relative Initiation of Screening
Echo
Pediatric
Children and adolescents from genotype-

At the time HCM is diagnosed in another family
positive families, and families with early Every 1-2 y
member
onset disease
At any time after HCM is diagnosed in a family

All other children and adolescents Every 2-3 y
member but no later than puberty
At the time HCM is diagnosed in another family

Adults Every 3-5 y
member
*Includes all asymptomatic, phenotype-negative first-degree relatives deemed to be at-risk for developing HCM based on family history or genotype status
and may sometimes include more distant relatives based on clinical judgment. Screening interval may be modified (e.g., at onset of new symptoms or in
families with a malignant clinical course or late-onset HCM).
ECG indicates electrocardiogram; Echo, echocardiogram; and HCM, hypertrophic cardiomyopathy. 31
Cardiovascular Magnetic Resonance Imaging

1. For patients suspected to have HCM in whom echocardiography is
inconclusive, cardiovascular magnetic resonance (CMR) imaging is indicated
1 B-NR
for diagnostic clarification
2. For patients with left ventricular hypertrophy (LVH) in whom there is a
suspicion of alternative diagnoses, including infiltrative or storage disease as
1 B-NR
well as athlete’s heart, CMR imaging is useful.
3. For patients with HCM who are not otherwise identified as high risk for sudden
cardiac death (SCD), or in whom a decision to proceed with ICD remains
uncertain after clinical assessment that includes personal/family history,
echocardiography, and ambulatory electrocardiographic monitoring, CMR
1 B-NR imaging is beneficial to assess for maximum left ventricular (LV) wall thickness,
ejection fraction (EF), LV apical aneurysm, and extent of myocardial fibrosis
with late gadolinium enhancement (LGE).
32
Cardiovascular Magnetic Resonance Imaging

4. For patients with obstructive HCM in whom the anatomic mechanism of
obstruction is inconclusive on echocardiography, CMR imaging is indicated to
1 B-NR
inform the selection and planning of septal reduction therapy (SRT).
5. For patients with HCM, repeat contrast-enhanced cardiovascular magnetic

resonance (CMR) imaging on a periodic basis (every 3 to 5 years) for the
purpose of sudden (SCD) risk stratification may be considered to evaluate
2b C-EO changes in late gadolinium enhancement (LGE) and other morphologic changes,
including ejection fraction (EF), development of apical aneurysm, or left
ventricular (LV) wall thickness.
33
Cardiac Computed Tomography (CT)

1. In adult patients with suspected HCM, cardiac
computed tomography (CT) may be considered for
diagnosis if the echocardiogram is not diagnostic
2b C-LD and cardiovascular magnetic resonance (CMR)
imaging is unavailable.
34
Heart Rhythm Assessment
1. In patients with HCM, a 12-lead electrocardiogram (ECG) is recommended in
1 B-NR the initial evaluation and as part of periodic follow-up (every 1 to 2 years).
2. In patients with HCM, 24- to 48-hour ambulatory electrocardiographic

monitoring is recommended in the initial evaluation and as part of periodic
follow-up (every 1 to 2 years) to identify patients who are at risk for sudden
1 B-NR cardiac death (SCD) and guide management of arrhythmias.
35
3. In patients with HCM who develop palpitations or
lightheadedness, extended (>24 hours) electrocardiographic
monitoring or event recording is recommended, which should not
1 B-NR
be considered diagnostic unless patients have had symptoms while
being monitored.
4. In first-degree relatives of patients with HCM, a 12-lead ECG is

1 B-NR recommended as a component of the screening algorithm.
36

5. In patients with HCM who have additional risk factors for atrial fibrillation
(AF), such as left atrial dilatation, advanced age, and New York Heart
Association (NYHA) class III to class IV heart failure (HF), and who are
2a B-NR eligible for anticoagulation, extended ambulatory monitoring is reasonable to
screen for AF as part of initial evaluation and periodic follow-up (every 1 to 2
years).
6. In adult patients with HCM without risk factors for AF and who are eligible
for anticoagulation, extended ambulatory monitoring may be considered to
2b B-NR
assess for asymptomatic paroxysmal AF as part of initial evaluation and
periodic follow-up (every 1 to 2 years).
37
Angiography and Invasive Hemodynamic Assessment

1. For patients with HCM who are candidates for septal reduction therapy
(SRT) and for whom there is uncertainty regarding the presence or severity
1 B-NR of left ventricular outflow tract obstruction (LVOTO) on noninvasive

imaging studies, invasive hemodynamic assessment with cardiac
catheterization is recommended.
2. In patients with HCM with symptoms or evidence of myocardial ischemia,

1 B-NR
coronary angiography (CT or invasive) is recommended.
3. In patients with HCM who are at risk of coronary atherosclerosis, coronary
1 B-NR angiography (CT or invasive) is recommended before surgical myectomy.
38
Exercise Stress Testing
1. For symptomatic patients with HCM who do not have resting or provocable
outflow tract gradient ≥50 mm Hg on TTE, exercise TTE is recommended for
1 B-NR
the detection and quantification of dynamic LVOTO.
2. In patients with nonobstructive HCM and advanced HF (NYHA functional

class III to class IV despite GDMT), cardiopulmonary exercise stress testing
should be performed to quantify the degree of functional limitation and aid
1 B-NR
in selection of patients for heart transplantation or mechanical circulatory
support.
39
3. In patients with HCM, exercise stress testing is reasonable to determine
functional capacity and to provide prognostic information as part of
2a B-NR
initial evaluation.
4. For asymptomatic patients with HCM who do not have a resting or

provocable outflow tract gradient ≥50 mm Hg on standard transthoracic
echocardiogram (TTE), exercise TTE is reasonable for the detection and
2a C-LD
quantification of dynamic left ventricular outflow tract obstruction
(LVOTO).
40
5. In patients with obstructive HCM who are being considered for
septal reduction therapy (SRT) and in whom functional
2b C-EO capacity or symptom status is uncertain, exercise stress testing
may be reasonable.
6. In patients with HCM in whom functional capacity or symptom

status is uncertain, exercise stress testing may be considered
2b C-EO
every 2 to 3 years.
41
Genetics and Family Screening
1. In patients with HCM, evaluation of familial inheritance, including a 3-generation
1 B-NR family history, is recommended as part of the initial assessment.
2. In patients with HCM, genetic testing is beneficial to elucidate the genetic basis to
1 B-NR facilitate the identification of family members at risk for developing HCM
(cascade testing).
3. In patients with an atypical clinical presentation of HCM or when another genetic

condition is suspected to be the cause, a work-up including genetic testing for
1 B-NR
HCM and other genetic causes of unexplained cardiac hypertrophy (“HCM
phenocopies”) is recommended.
42
4. In patients with HCM who choose to undergo genetic testing, pre- and posttest genetic
counseling by an expert in the genetics of cardiovascular disease is recommended so that
1 B-NR risks, benefits, results, and their clinical significance can be reviewed and discussed with the
patient in a shared decision-making process.
5. When performing genetic testing in an HCM proband, the initial tier of genes tested should
1 B-NR
include genes with strong evidence to be disease-causing in HCM*.
6. In first-degree relatives of patients with HCM, both clinical screening (ECG and 2D
echocardiogram) and cascade genetic testing (when a pathogenic/likely pathogenic variant
1 B-NR
has been identified in the proband) should be offered.
*Strong evidence HCM genes include, at the time of this publication: MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, and ACTC1.
43
7. In families where a sudden unexplained death has occurred with a postmortem
diagnosis of HCM, postmortem genetic testing is beneficial to facilitate cascade
1 B-NR
genetic testing and clinical screening in first-degree relatives.
8. In patients with HCM who have undergone genetic testing, serial reevaluation of
the clinical significance of the variant(s) identified is recommended to assess for
1 B-NR variant reclassification, which may impact diagnosis and cascade genetic testing in
family members.
9. In affected families with HCM, preconception and prenatal reproductive and

1 B-NR
genetic counseling should be offered.
44
10. In patients with HCM, the usefulness of genetic testing in
2b B-NR the assessment of risk of SCD is uncertain.
11. In patients with HCM who harbor a variant of

uncertain significance, the usefulness of clinical genetic
2b B-NR testing of phenotype-negative relatives for the purpose of
variant reclassification is uncertain.
45
12. For patients with HCM who have undergone genetic testing and
3: B-NR were found to have no pathogenic variants (i.e., harbor only
No benefit
benign/likely benign variants), cascade genetic testing of the family
is not useful.
13. Ongoing clinical screening is not indicated in genotype-negative

relatives in families with genotype-positive HCM, unless the
3: B-NR
No benefit disease-causing variant is downgraded to variant of uncertain
significance, likely benign, or benign variant during follow-up.
46
Figure 2. HCM Index Case Targeted Gene Testing
Colors correspond to the

Class of Recommendation in
Table 2.
HCM indicates hypertrophic
cardiomyopathy; LB/B,
likely benign/benign; LP/P,
likely pathogenic or
pathogenic; and VUS,
variant of unknown
significance.
47
Genotype-Positive, Phenotype-Negative
1. In individuals who are genotype-positive, phenotype-negative for HCM, serial
clinical assessment, electrocardiography, and cardiac imaging are recommended
at periodic intervals depending on age (every 1 to 2 years in children and
1 B-NR
adolescents, and every 3 to 5 years in adults) and change in clinical status.
2. In individuals who are genotype-positive, phenotype-negative for HCM,

2a C-LD participation in competitive athletics of any intensity is reasonable.
3: 3. In individuals who are genotype-positive, phenotype-negative for HCM, ICD is

No benefit B-NR not recommended for primary prevention.
48
Sudden Cardiac Risk (SCD) Assessment and
Prevention
49
SCD Risk Assessment
1. In patients with HCM, a comprehensive, systematic noninvasive SCD risk assessment at
initial evaluation and every 1 to 2 years thereafter is recommended and should include
evaluation of these risk factors:
a. Personal history of cardiac arrest or sustained ventricular arrhythmias
b. Personal history of syncope suspected by clinical history to be arrhythmic
c. Family history in close relative of premature HCM-related sudden death, cardiac
1 B-NR
arrest, or sustained ventricular arrhythmias
d. Maximal left ventricular (LV) wall thickness, ejection fraction (EF), LV apical
aneurysm
e. Non-sustained ventricular tachycardia (NSVT) episodes on continuous ambulatory
electrocardiographic monitoring
50
SCD Risk Assessment
2. For patients with HCM who are not otherwise identified as high risk for
SCD, or in whom a decision to proceed with implantable cardioverter-
defibrillator (ICD) placement remains uncertain after clinical
assessment that includes personal/family history, echocardiography, and
ambulatory electrocardiographic monitoring, cardiovascular magnetic
1 B-NR
resonance (CMR) imaging is beneficial to assess for maximum left
ventricular (LV) wall thickness, ejection fraction (EF), LV apical
aneurysm, and extent of myocardial fibrosis with late gadolinium
enhancement (LGE).
51
SCD Risk Assessment
3. For patients who are ≥ 16 years of age with HCM, it is
reasonable to obtain echocardiography-derived left atrial
diameter and maximal left ventricular outflow tract (LVOT)
gradient to aid in calculating an estimated 5-year sudden
2a B-NR
death risk that may be useful during shared decision-making
for implantable cardioverter-defibrillator (ICD) placement.
52
Patient Selection for ICD Placement
1. In patients with HCM, application of individual clinical judgment is
recommended when assessing the prognostic strength of conventional
risk marker(s) within the clinical profile of the individual patient, as
1 C-EO well as a thorough and balanced discussion of the evidence, benefits,
and estimated risks to engage the fully informed patient’s active
participation in ICD decision-making.
2. For patients with HCM, and previous documented cardiac arrest or

1 B-NR
sustained VT, ICD placement is recommended.
53
3. For adult patients with HCM with ≥1 major risk factors for SCD, it is
reasonable to offer an ICD. These major risk factors include:
a) Sudden death judged definitively or likely attributable to HCM in ≥1
first-degree or close relatives who are ≤50 years of age;
b) Massive LVH ≥30 mm in any LV segment;
2a B-NR c) ≥1 Recent episodes of syncope suspected by clinical history to be
arrhythmic (i.e., unlikely to be of neurocardiogenic vasovagal, etiology,
or related to LVOTO);
d) LV apical aneurysm, independent of size;
e) LV systolic dysfunction (EF<50%).
54
4. For children with HCM who have ≥1 conventional risk factors, including
unexplained syncope, massive LVH, NSVT, or family history of early
2a B-NR HCM-related SCD, ICD placement is reasonable after considering the
relatively high complication rates of long-term ICD placement in younger
patients.
5. For patients ≥16 years of age with HCM and with ≥1 major SCD risk
factors, discussion of the estimated 5-year sudden death risk and mortality
2a B-NR rates can be useful during the shared decision-making process for ICD
placement.
55
6. In select adult patients with HCM and without major SCD risk factors after
clinical assessment, or in whom the decision to proceed with ICD placement
2b B-NR remains otherwise uncertain, ICD may be considered in patients with

extensive LGE by contrast-enhanced CMR imaging or NSVT present on
ambulatory monitoring.
7. In select pediatric patients with HCM in whom risk stratification is

otherwise less certain, it may be useful to consider additional factors such
2b C-LD
as extensive LGE on contrast-enhanced CMR imaging and systolic
dysfunction in risk stratification.
56
3: 8. In patients with HCM without risk factors, ICD

B-NR placement should not be performed.
Harm
9. In patients with HCM, ICD placement for the

3: sole purpose of participation in competitive
B-NR
Harm athletics should not be performed.
57
Table 7. Established Clinical Risk Factors for HCM Sudden Death Risk Stratification
Family history of sudden death Sudden death judged definitively or likely attributable to HCM in ≥1 first-degree or close relatives who are ≤50 years of age. Close relatives would
from HCM generally be second- degree relatives; however, multiple SCDs in tertiary relatives should also be considered relevant.
Massive LVH Wall thickness ≥30 mm in any segment within the chamber by echocardiography or CMR imaging; consideration for this morphologic marker is
also given to borderline values of 28 mm in individual patients at the discretion of the treating cardiologist. For pediatric patients with HCM, an
absolute or z-score threshold for wall thickness has not been established; however, a maximal wall that corresponds to a z-score ≥20 (and >10 in
conjunction with other risk factors) appears reasonable.
Unexplained syncope ≥1 Unexplained episodes involving acute transient loss of consciousness, judged by history unlikely to be of neurocardiogenic (vasovagal) etiology,
nor attributable to LVOTO, and especially when occurring within 6 months of evaluation (events beyond 5 years in the past do not appear to have
relevance).
HCM with LV systolic dysfunction Systolic dysfunction with EF <50% by echocardiography or CMR imaging.
LV apical aneurysm Apical aneurysm defined as a discrete thin-walled dyskinetic or akinetic segment of the most distal portion of the LV chamber; independent of size.
Extensive LGE on CMR imaging Diffuse and extensive LGE, representing fibrosis, either quantified or estimated by visual inspection, comprising ≥15% of LV mass (extent of LGE
conferring risk has not been established in children).
NSVT on ambulatory monitor It would seem most appropriate to place greater weight on NSVT as a risk marker when runs are frequent (≥3), longer (≥10 beats), and faster (≥200
bpm) occurring usually over 24 to 48 hours of monitoring. For pediatric patients, a VT rate that exceeds the baseline sinus rate by >20% is
considered significant.
58
CMR indicates cardiovascular magnetic resonance; ICD, implantable cardioverter-defibrillator; LGE, late gadolinium enhancement; LV, left ventricular; LVH, left ventricular hypertrophy; LVOTO, left ventricular
outflow tract obstruction; NSVT, nonsustained ventricular tachycardia; and SCD, sudden cardiac death.
Device Selection Considerations
59
Selection of ICD Device Type
1. In patients with HCM who are receiving an ICD, either a single
chamber transvenous ICD or a subcutaneous ICD is recommended
after a shared decision-making discussion that takes into
1 B-NR
consideration patient preferences, lifestyle, and expected potential
need for pacing for bradycardia or VT termination.
2. In patients with HCM who are receiving an ICD, single-coil ICD

1 B-NR leads are recommended in preference to dual-coil leads.
60
3. In patients with HCM who are receiving an ICD, dual-
chamber ICDs are reasonable for patients with a need
for atrial or atrioventricular sequential pacing for
2a B-NR bradycardia/conduction abnormalities, or as an attempt
to relieve symptoms of obstructive HCM (most
commonly in patients >65 years of age).
61
4. In selected adult patients with nonobstructive HCM receiving an ICD who
have NYHA class II to ambulatory class IV HF, left bundle branch block
2a C-LD (LBBB), and LV ejection fraction (LVEF) <50%, cardiac resynchronization
therapy (CRT) for symptom reduction is reasonable.
5. In patients with HCM in whom a decision has been made for ICD
implantation and who have paroxysmal atrial tachycardias or AF, dual-
2b C-LD
chamber ICDs may be reasonable, but this decision must be balanced
against higher complication rates of dual-chamber devices.
62
Figure 3. ICD Patient Selection
Colors correspond to the Class of Recommendation in Table

2.
*ICD decisions in pediatric patients with HCM are based on

≥1 of these major risk factors: family history of HCM SCD,
NSVT on ambulatory monitor, massive LVH, and
unexplained syncope.
†In patients >16 years of age, 5-year risk estimates can be

considered to fully inform patients during shared decision-
making discussions.
‡It would seem most appropriate to place greater weight on

frequent, longer, and faster runs of NSVT.
CMR indicates cardiovascular magnetic resonance; EF,

ejection fraction; FH, family history; HCM, hypertrophic
cardiomyopathy; ICD, implantable cardioverter-
defibrillator; LGE, late gadolinium enhancement; LVH, left
ventricular hypertrophy; NSVT, nonsustained ventricular
tachycardia; SCD, sudden cardiac death; VF, ventricular
fibrillation; and VT, ventricular tachycardia.
63
Management of Symptomatic Patients with
Obstructive Hypertrophic Cardiomyopathy
64
Pharmacologic Management of Patients With Obstructive HCM

1. In patients with obstructive HCM and symptoms* attributable to LVOTO,
nonvasodilating beta-blockers, titrated to effectiveness or maximally tolerated
1 B-NR
doses, are recommended.
Verapamil B- 2. In patients with obstructive HCM and symptoms* attributable to LVOTO, for
NR whom beta-blockers are ineffective or not tolerated, substitution with non-
1 dihydropyridine calcium channel blockers (e.g., verapamil, diltiazem) is
Diltiazem C- recommended.
LD
*Symptoms include effort-related dyspnea or chest pain; and occasionally other exertional symptoms (e.g., syncope, near
syncope) that are attributed to LVOTO and interfere with everyday activity or quality of life.
65
3. For patients with obstructive HCM who have persistent severe symptoms*
attributable to LVOTO despite beta-blockers or non-dihydropyridine calcium
channel blockers, either adding disopyramide in combination with 1 of the other
1 B-NR
drugs, or SRT performed at experienced centers,† is recommended.
4. For patients with obstructive HCM and acute hypotension who do not respond to
fluid administration, intravenous phenylephrine (or other vasoconstrictors
1 C-LD
without inotropic activity), alone or in combination with beta-blocking drugs, is
recommended.
*Symptoms include effort-related dyspnea or chest pain; and occasionally other exertional symptoms
(e.g., syncope, near syncope) that are attributed to LVOTO and interfere with everyday activity or quality of life.
66
5. For patients with obstructive HCM and persistent dyspnea with clinical evidence of volume
overload and high left- sided filling pressures despite other HCM guideline-directed
2b C-EO management and therapy (GDMT), cautious use of low-dose oral diuretics may be considered.
6. For patients with obstructive HCM, discontinuation of vasodilators (e.g., angiotensin-

converting enzyme inhibitors, angiotensin receptor blockers, dihydropyridine calcium channel
2b C-EO blockers) or digoxin may be reasonable because these agents can worsen symptoms caused by
dynamic outflow tract obstruction.
7. For patients with obstructive HCM and severe dyspnea at rest, hypotension, very high resting
3: gradients (e.g., >100 mm Hg), as well as all children <6 weeks of age, verapamil is potentially
C-LD
Harm harmful.
67
Invasive Treatment of Symptomatic Patients
with Obstructive HCM
1. In patients with obstructive HCM who remain severely symptomatic
1 B-NR despite GDMT, SRT in eligible patients,* performed at experienced
centers,† is recommended for relieving LVOTO.
2. In symptomatic patients with obstructive HCM who have associated

cardiac disease requiring surgical treatment (e.g., associated anomalous
papillary muscle, markedly elongated anterior mitral leaflet, intrinsic
1 B-NR mitral valve disease, multivessel CAD, valvular aortic stenosis), surgical
myectomy, performed at experienced centers,† is recommended.
68
*General eligibility criteria for septal reduction therapy:
a) Clinical: Severe dyspnea or chest pain (usually NYHA functional class III or class IV), or occasionally
other exertional symptoms (e.g., syncope, near syncope), when attributable to LVOTO, that interferes
with everyday activity or quality of life despite optimal medical therapy.
b) Hemodynamic: Dynamic LVOT gradient at rest or with physiologic provocation with approximate peak
gradient of ≥50 mm Hg, associated with septal hypertrophy and SAM of mitral valve.
c) Anatomic: Targeted anterior septal thickness sufficient to perform the procedure safely and effectively
in the judgment of the individual operator.
†Comprehensive or primary HCM centers with demonstrated excellence in clinical outcomes for these procedures
69
3. In adult patients with obstructive HCM who remain
severely symptomatic, despite GDMT and in whom
surgery is contraindicated or the risk is considered
1 C-LD unacceptable because of serious comorbidities or
advanced age, alcohol septal ablation in eligible patients,*
performed at experienced centers,† is recommended.
70
71
4. In patients with obstructive HCM, earlier (NYHA class II) surgical myectomy
performed at comprehensive HCM centers may be reasonable in the presence of
additional clinical factors, including:
a) Severe and progressive pulmonary hypertension thought to be
attributable to LVOTO or associated MR.
2b B-NR b) Left atrial enlargement with ≥1 episodes of symptomatic AF.
c) Poor functional capacity attributable to LVOTO as documented on
treadmill exercise testing.
d) Children and young adults with very high resting LVOT gradients
(>100 mm Hg).
72

5. For severely symptomatic patients with obstructive
HCM, SRT in eligible patients,* performed at
experienced centers† may be considered as an
2b B-NR alternative to escalation of medical therapy after shared
decision-making including risks and benefits of all
treatment options.
73
74
6. For patients with HCM who are asymptomatic and have
3: Harm C-LD normal exercise capacity, SRT is not recommended.
7. For symptomatic patients with obstructive HCM in whom

SRT is an option, mitral valve replacement should not be
3: Harm B-NR
performed for the sole purpose of relief of LVOTO.
75
Figure 4. Management of Symptoms Recommendation in Table 2.
in Patients With HCM GL indicates guideline; HCM,

hypertrophic cardiomyopathy;
HFpEF, heart failure with preserved
ejection fraction; HFrEF, heart failure
with reduced ejection fraction; and
SRT, septal reduction therapy.
76
Management of Patients with Nonobstructive
Hypertrophic Cardiomyopathy with Preserved
Ejection Fraction (EF)
77
Management of Patients with Nonobstructive HCM
with Preserved EF
1. In patients with nonobstructive HCM with preserved EF and
symptoms of exertional angina or dyspnea, beta- blockers or non-
1 C-LD dihydropyridine calcium channel blockers are recommended.
2. In patients with nonobstructive HCM with preserved EF, it is

reasonable to add oral diuretics when exertional dyspnea persists
2a C-EO despite the use of beta-blockers or non-dihydropyridine calcium
channel blockers.
78
with Preserved EF
3. In patients with nonobstructive HCM with

preserved EF, the usefulness of angiotensin-
converting enzyme inhibitors and angiotensin
2b C-LD receptor blockers in the treatment of symptoms
(angina and dyspnea) is not well established.
79
with Preserved EF
4. In highly selected patients with apical HCM with severe dyspnea or
angina (NYHA class III or class IV) despite maximal medical therapy,
and with preserved EF and small LV cavity size (LV end-diastolic
2b C-LD volume <50 mL/m2 and LV stroke volume <30 mL/m2), apical
myectomy by experienced surgeons at comprehensive centers may be
considered to reduce symptoms.
5. In asymptomatic patients with nonobstructive HCM, the benefit of

2b C-EO beta-blockers or calcium channel blockers is not well established.
80
Management of Patients with Hypertrophic
Cardiomyopathy and Atrial Fibrillation (AF).
81
Management of Patients with HCM and AF
1. In patients with HCM and clinical AF, anticoagulation is
recommended with direct-acting oral anticoagulants (DOAC) as first-
1 B-NR line option and vitamin K antagonists as second-line option,
independent of CHA2DS2-VASc score.
2. In patients with HCM and subclinical AF detected by internal or

external cardiac device or monitor of >24 hours’ duration for a given
1 C-LD episode, anticoagulation is recommended with DOAC as first-line

option and vitamin K antagonists as second-line option, independent
of CHA2DS2-VASc score.
82

3. In patients with AF in whom rate control strategy is planned, either beta-blockers,
verapamil, or diltiazem are recommended, with the choice of agents according to
1 C-LD
patient preferences and comorbid conditions.
4. In patients with HCM and subclinical AF detected by internal or external device

or monitor, of >5 minutes’ but <24 hours’ duration for a given episode,
anticoagulation with DOAC as first-line option and vitamin K antagonists as
2a C-LD second-line option can be beneficial, taking into consideration duration of AF
episodes, total AF burden, underlying risk factors, and bleeding risk.
83
5. In patients with HCM and poorly tolerated AF, a rhythm control
strategy with cardioversion or antiarrhythmic drugs can be beneficial
2a B-NR with the choice of an agent according to AF symptom severity, patient
preferences, and comorbid conditions.
6. In patients with HCM and symptomatic AF, as part of an AF rhythm

control strategy, catheter ablation for AF can be effective when drug
2a B-NR
therapy is ineffective, contraindicated, or not the patient’s preference.
84

7. In patients with HCM and AF who require
surgical myectomy, concomitant surgical AF
2a B-NR ablation procedure can be beneficial for AF

rhythm control.
85
Table 8. Antiarrhythmic Drug Therapy Options for Patients With HCM and AF
Antiarrhythmic Drug Efficacy for AF Side Effects Toxicities Use in HCM
Disopyramide Modest Anticholinergic Prolonged QTc Particularly with early onset

HF AF
Flecainide and propafenone ? Proarrhythmia Not generally recommended
in the absence of an ICD
Sotalol Modest Fatigue Prolonged QTc Reasonable
Bradycardia Prolonged QTc
Proarrhythmia
Dofetilide Modest Headache Proarrhythmia Reasonable
Dronedarone Low HF Prolonged QTc ?
Amiodarone Modest-high Bradycardia Liver, lung, thyroid, skin, Reasonable
neurologic
86
AF indicates atrial fibrillation; HCM, hypertrophic cardiomyopathy; HF, heart failure; and ICD, implantable cardioverter-defibrillator.
Cardiopathy (HCM) and Ventricular
Arrhythmias (VA)
87
Management of Patients with HCM and Ventricular
Arrhythmias

1. In patients with HCM and recurrent poorly tolerated
life-threatening ventricular tachyarrhythmias
refractory to maximal antiarrhythmic drug therapy
1 B-NR and ablation, heart transplantation assessment is
indicated in accordance with current listing criteria.
88
Arrhythmias
Amiodarone,
B-NR 2. In adults with HCM and symptomatic ventricular
arrhythmias or recurrent ICD shocks despite beta-
Dofetilide, blocker use, antiarrhythmic drug therapy listed is
C-LD
recommended, with the choice of agent guided by age,
1
Mexiletine, underlying comorbidities, severity of disease, patient
C-LD
preferences, and balance between efficacy and safety.
Sotalol,
C-LD
89
Arrhythmias
3. In children with HCM and recurrent ventricular arrhythmias despite
beta-blocker use, antiarrhythmic drug therapy (amiodarone,
mexiletine, sotalol) is recommended, with the choice of agent guided
1 C-LD by age, underlying comorbidities, severity of disease, patient
preferences, and balance of efficacy and safety.
4. In patients with HCM and pacing-capable ICDs, programming

1 C-LD antitachycardia pacing is recommended to minimize risk of shocks.
90
Arrhythmias

5. In patients with HCM and recurrent symptomatic
sustained monomorphic VT, or recurrent ICD shocks
despite optimal device programming, and in whom
2a C-LD antiarrhythmic drug therapy is either ineffective, not
tolerated, or not preferred, catheter ablation can be useful
for reducing arrhythmia burden.
91
Cardiomyopathy and Advanced Heart Failure
92
Management of Patients with HCM and HF
1. In patients with HCM who develop systolic dysfunction
1 C-LD with an LVEF <50%, guideline-directed therapy for HF

with reduced EF is recommended.
2. In patients with HCM and systolic dysfunction, diagnostic

testing to assess for concomitant causes of systolic
1 C-LD
dysfunction (such as CAD) is recommended.
93
3. In patients with nonobstructive HCM and advanced HF (NYHA functional
class III to class IV despite guideline-directed therapy), CPET should be
1 B-NR performed to quantify the degree of functional limitation and aid in selection
of patients for heart transplantation or mechanical circulatory support.
4. In patients with nonobstructive HCM and advanced HF (NYHA class III to

class IV despite guideline-directed therapy) or with life-threatening
1 B-NR ventricular arrhythmias refractory to maximal guideline-directed therapy,

assessment for heart transplantation in accordance with current listing
criteria is recommended.
94
5. For patients with HCM who develop systolic dysfunction (LVEF <50%),
it is reasonable to discontinue previously indicated negative inotropic
2a C-EO agents (specifically, verapamil, diltiazem, or disopyramide).
6. In patients with nonobstructive HCM and advanced HF (NYHA

functional class III to class IV despite GDMT) who are candidates for
2a B-NR heart transplantation, continuous-flow LVAD therapy is reasonable as a

bridge to heart transplantation.
95

7. In patients with HCM and LVEF <50%, ICD placement
2a C-LD can be beneficial.
8. In patients with HCM and LVEF <50%, NYHA functional

class II to class IV symptoms despite guideline-directed
2a C-LD therapy, and LBBB, CRT can be beneficial to improve
symptoms.
96
Recommendation in Table 2.
ACE indicates angiotensin-
Figure 5. Heart
converting enzyme; ARB, angiotensin Failure Algorithm
receptor blocker; ARNI, angiotensin
receptor-neprilysin inhibitors; CRT,
cardiac resynchronization therapy;
EF, ejection fraction; GDMT,
guideline-directed management and
therapy; HCM, hypertrophic
cardiomyopathy; LBBB, left bundle
branch block; LVAD, left ventricular
assist device; LVEF, left ventricular
ejection fraction; MRA,
mineralocorticoid receptor
antagonist; and NYHA, New York
Heart Association.
97
Lifestyle Considerations for Patients with Hypertrophic
Cardiomyopathy
98
Table 9. Lifestyle Considerations for
Patients With HCM
Lifestyle
Considerations*
Sports/activity For most patients with HCM, mild- to moderate-intensity recreational exercise is beneficial to improve
cardiorespiratory fitness, physical functioning, and quality of life, and for their overall health in
keeping with physical activity guidelines for the general population.
Pregnancy For women with clinically stable HCM who wish to become pregnant, it is reasonable to advise that
pregnancy is generally safe as part of a shared discussion regarding potential maternal and fetal risks,
and initiation of guideline-directed therapy.
Comorbidities The clinician should monitor and counsel patients on prevention and treatment of comorbid conditions
that can worsen severity of HCM (atherosclerotic cardiovascular disease, obesity, hypertension, sleep-
disordered breathing)
*Shared decision-making is an important component of counseling and lifestyle modifications.

HCM indicates hypertrophic cardiomyopathy 99
Sports and Activity

1. For most patients with HCM, mild- to moderate-intensity recreational*
exercise is beneficial to improve cardiorespiratory fitness, physical
1 B-NR functioning, and quality of life, and for their overall health in keeping with
physical activity guidelines for the general population.
2. For athletes with HCM, a comprehensive evaluation and shared discussion

of potential risks of sports participation by an expert provider is
1 C-EO
recommended.
*Recreational exercise is done for the purpose of leisure with no requirement for systematic training and without the purpose to excel or compete against others.
100
Sports and Activity

3. For most patients with HCM, participation in low-
2a C-EO
intensity competitive sports is reasonable.
4. In individuals who are genotype-positive, phenotype-

negative for HCM, participation in competitive
2a C-LD
athletics of any intensity is reasonable.
101
Sports and Activity

5. For patients with HCM, participation in high-intensity recreational
activities or moderate- to high-intensity competitive sports activities may
be considered after a comprehensive evaluation and shared discussion,
repeated annually with an expert provider who conveys that the risk of
sudden death and ICD shocks may be increased, and with the
2b C-LD
understanding that eligibility decisions for competitive sports
participation often involve third parties (e.g., team physicians,
consultants, and other institutional leadership) acting on behalf of the
schools or teams.
102
Sports and Activity
6. In patients with HCM, ICD placement for

the sole purpose of participation in
3:
B-NR competitive athletics should not be
Harm
performed.
103
Occupation

1. For patients with HCM, it is reasonable to follow Federal
Motor Carrier Safety Administration cardiovascular
disease guidelines that permit driving commercial motor
2a C-EO vehicles, if they do not have an ICD or any major risk
factors for SCD and are following a guideline-directed
management plan.
104
Occupation

2. For pilot aircrew with a diagnosis of HCM, it is
reasonable to follow Federal Aviation Administration
guidelines that permit consideration of multicrew flying
2a C-EO duties, provided they are asymptomatic, are deemed

low risk for SCD, and can complete a maximal
treadmill stress test at 85% peak heart rate
105
Occupation
3. Patients with HCM may consider occupations that require
manual labor, heavy lifting, or a high level of physical
performance after a comprehensive clinical evaluation, risk
stratification for SCD, and implementation of guideline-directed
2a C-EO management. Before a shared decision between a clinician and
patient is reached, the clinician should convey that risks
associated with the physical requirements of these occupations
are uncertain.
106
Pregnancy
1. For pregnant women with HCM and AF or other indications for
anticoagulation, low-molecular-weight heparin or vitamin K
1 B-NR antagonists (at maximum therapeutic dose of <5 mg daily) are
recommended for stroke prevention.
2. In pregnant women with HCM, selected beta-blockers should be

administered for symptoms related to outflow tract obstruction or
1 C-LD
arrhythmias, with monitoring of fetal growth.
107
Pregnancy
3. In most pregnant women with HCM, vaginal delivery is
recommended as the first-choice delivery option.
1 C-LD
4. In affected families with HCM, preconceptional and

1 B-NR prenatal reproductive and genetic counseling should be
offered.
108
Pregnancy
5. For pregnant women with HCM, care should be coordinated between
their cardiologist and an obstetrician. For patients with HCM who
1 C-EO
are deemed high risk, consultation is advised with an expert in
maternal-fetal medicine.
6. For women with clinically stable HCM who wish to become pregnant,
it is reasonable to advise that pregnancy is generally safe as part of a
2a C-LD shared discussion regarding potential maternal and fetal risks, and
initiation of guideline-directed therapy.
109
Pregnancy
7. In pregnant women with HCM, cardioversion for
2a C-LD new or recurrent AF, particularly if symptomatic, is

reasonable.
8. In pregnant women with HCM, general or epidural
2a C-LD anesthesia is reasonable, with precautions to avoid

hypotension.
110
Pregnancy
9. In pregnant women with HCM, it is reasonable to perform
serial echocardiography, particularly during the second or
2a C-EO
third trimester when hemodynamic load is highest, or if
clinical symptoms develop.
10. In pregnant women with HCM, fetal echocardiography may

be considered for diagnosis of fetal HCM in the context of
2b C-EO
prenatal counseling.
111
Comorbidities
1. In patients with HCM, adherence to the guidelines on the
prevention of atherosclerotic cardiovascular disease is
1 C-EO
recommended to reduce risk of cardiovascular events.
2. In patients with HCM who are overweight or obese, counseling and

comprehensive lifestyle interventions are recommended for
1 B-NR achieving and maintaining weight loss and possibly lowering the
risk of developing LVOTO, HF, and AF.
112
Comorbidities
3. In patients with HCM and hypertension, lifestyle modifications
and medical therapy for hypertension are recommended with
1 C-LD preference for beta-blockers and non-dihydropyridine calcium
channel blockers in patients with obstructive HCM.
4. In patients with HCM, assessment for symptoms of sleep-

disordered breathing is recommended and, if present, referral to a
1 C-LD
sleep medicine specialist for evaluation and treatment.
113
Abbreviations used in this Guideline
Abbreviation Meaning/Phrase
AF atrial fibrillation
CAD coronary artery disease
CMR cardiovascular magnetic resonance
CPET cardiopulmonary exercise test
CRT cardiac resynchronization therapy
DOAC direct-acting oral anticoagulants
EF ejection fraction
GDMT guideline-directed management and therapy 114
HCM hypertrophic cardiomyopathy
HF heart failure
ICD implantable cardioverter-defibrillator
LAMP2 lysosome-associated membrane protein-2
LBBB left bundle branch block
LGE late gadolinium enhancement
LV left ventricular
LVAD left ventricular assist device 115
LVOT left ventricular outflow tract
LVOTO left ventricular outflow tract obstruction
MET metabolic equivalent
MR mitral regurgitation
NSVT nonsustained ventricular tachycardia
NYHA New York Heart Association
RCT randomized controlled trial
RV right ventricular 116
SAM systolic anterior motion
SCAF subclinical AF
SCD sudden cardiac death
SRT septal reduction therapy
TEE trans-esophageal echocardiogram
TTE transthoracic echocardiogram
VF ventricular fibrillation
VT ventricular tachycardia 117

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2020 AHA/ACC Guideline for the Diagnosis and

Treatment of Patients With Hypertrophic

Developed in collaboration with and endorsed by the American Society of Echocardiography,

Michael A. Burke, MD† Michelle Kittleson, MD, PhD, FACC†

2020 Hypertrophic Cardiomyopathy Guidelines

5. Assessment of an individual patient’s risk for SCD continues to evolve as new

7. Septal reduction therapies (surgical septal myectomy and alcohol septal

8. Patients with HCM and persistent or paroxysmal atrial fibrillation have a

9. Heart failure symptoms in patients with HCM, in the absence of left

COR LOE Recommendations

2. In patients with HCM, consultation with or referral to a

COR LOE Recommendations

Early childhood Delayed or abnormal cognitive  RASopathies  Biochemical screening

*HCM indicates hypertrophic cardiomyopathy

Colors correspond to the Class of

1 myocardial hypertrophy, dynamic left ventricular outflow tract

3. For patients with HCM who experience a change in clinical status or a

6. For patients with HCM undergoing surgical septal myectomy, intraoperative

8. For patients with HCM who have undergone septal reduction

1 B-NR transthoracic echocardiogram (TTE) is recommended as part of

10. Screening: In individuals who are genotype-positive or phenotype-

Children and adolescents from genotype-

At any time after HCM is diagnosed in a family

At the time HCM is diagnosed in another family

COR LOE Recommendations

COR LOE Recommendations

5. For patients with HCM, repeat contrast-enhanced cardiovascular magnetic

COR LOE Recommendation

2. In patients with HCM, 24- to 48-hour ambulatory electrocardiographic

4. In first-degree relatives of patients with HCM, a 12-lead ECG is

COR LOE Recommendations

COR LOE Recommendations

1 B-NR of left ventricular outflow tract obstruction (LVOTO) on noninvasive

2. In patients with HCM with symptoms or evidence of myocardial ischemia,

2. In patients with nonobstructive HCM and advanced HF (NYHA functional

4. For asymptomatic patients with HCM who do not have a resting or

6. In patients with HCM in whom functional capacity or symptom

3. In patients with an atypical clinical presentation of HCM or when another genetic

9. In affected families with HCM, preconception and prenatal reproductive and

11. In patients with HCM who harbor a variant of

13. Ongoing clinical screening is not indicated in genotype-negative

Colors correspond to the

2. In individuals who are genotype-positive, phenotype-negative for HCM,

3: 3. In individuals who are genotype-positive, phenotype-negative for HCM, ICD is

2. For patients with HCM, and previous documented cardiac arrest or

2b B-NR remains otherwise uncertain, ICD may be considered in patients with

7. In select pediatric patients with HCM in whom risk stratification is

3: 8. In patients with HCM without risk factors, ICD

9. In patients with HCM, ICD placement for the

2. In patients with HCM who are receiving an ICD, single-coil ICD

Colors correspond to the Class of Recommendation in Table

*ICD decisions in pediatric patients with HCM are based on

†In patients >16 years of age, 5-year risk estimates can be

‡It would seem most appropriate to place greater weight on

CMR indicates cardiovascular magnetic resonance; EF,

COR LOE Recommendations

6. For patients with obstructive HCM, discontinuation of vasodilators (e.g., angiotensin-

2. In symptomatic patients with obstructive HCM who have associated

COR LOE Recommendations