Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Edzel Lorraine Co, DMD, MD[2]
2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy [1]
Recommendation for Shared Decision-Making
| Class I
|
Level of Evidence
|
| 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 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.
|
B-NR
|
Recommendation for Multidisciplinary HCM Centers
Recommendation for Diagnosis, Initial Evaluation, and Follow-up
Recommendation for Echocardiography
| Class I
|
Level of Evidence
|
| 1. In patients with suspected HCM, a trans-thoracic echocardiogram (TTE) is recommended in the initial evaluations.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In patients with HCM with no change in clinical status or events, repeat TTE is recommended every 1 to 2 years to assess the degree of myocardial hypertrophy, dynamic left ventricular outflow tract obstruction (LVOTO), mitral regurgitation, and myocardial function.
|
B-NR children
|
| C-LD adult
|
| Class I
|
Level of Evidence
|
| 3. For patients with HCM who experience a change in clinical status or a new clinical event, repeat TTE is recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 4. For patients with HCM and resting left ventricular outflow tract gradient <50 mm Hg, a TTE with provocative maneuvers is recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 6. For symptomatic patients with HCM who do not have a resting or provocable outflow tract gradient ≥50 mm Hg on TTE, exercise TTE is recommended for the detection and quantification of dynamic LVOTO.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 7. For patients with HCM undergoing surgical septal myectomy, intraoperative transesophageal echocardiogram (TEE) is recommended to assess mitral valve anatomy and function and adequacy of septal myectomy.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 8. For patients with HCM undergoing alcohol septal ablation, TTE or intraoperative TEE with intracoronary ultrasound-enhancing contrast injection of the candidate’s septal perforator(s) is recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 9. Screening: In first-degree relatives of patients with HCM, a TTE is recommended as part of initial family screening and periodic follow-up.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 10. Screening: In individuals who are genotype-positive or phenotype-negative, serial echocardiography is recommended at periodic intervals depending on age (1 to 2 years in children and adolescents, 3 to 5 years in adults) and change in clinical status.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 11. For patients with HCM, TEE can be useful if TTE is inconclusive in clinical decision-making regarding medical therapy, and in situations such as planning for myectomy, exclusion of subaortic membrane or mitral regurgitation secondary to structural abnormalities of the mitral valve apparatus, or in the assessment of the feasibility of alcohol septal ablation.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 12. For patients with HCM in whom the diagnoses of apical HCM, apical aneurysm, or atypical patterns of hypertrophy is inconclusive on 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.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 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 for the detection and quantification of dynamic LVOTO.
|
C-LD
|
Recommendation for Cardiovascular Magnetic Resonance Imaging
| Class I
|
Level of Evidence
|
| 1. For patients suspected to have HCM in whom echocardiography is inconclusive, CMR imaging is indicated for diagnostic clarification.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. For patients with left ventricular hypertrophy in whom there is a suspicion of alternative diagnoses including infiltrative or storage disease as well as athlete’s heart, CMR imaging is useful.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 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 implantable cardioverter defibrillator (ICD) remains uncertain after clinical assessment that includes personal/family history, echocardiography, and ambulatory electrocardiographic monitoring, 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.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 4. For patients with obstructive HCM in whom the anatomic mechanism of obstruction is inconclusive on echocardiography, CMR imaging is indicated to inform the selection and planning of SRT.
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 5. For patients with HCM, repeat contrast-enhanced CMR imaging on a periodic basis (every 3 to 5 years) for the purpose of SCD risk stratification may be considered to evaluate changes in late gadolinium enhancement and other morphologic changes, including EF, development of apical aneurysm, or LV wall thickness.
|
C-EO
|
Recommendation for Heart Rhythm Assessment
| Class I
|
Level of Evidence
|
| 1. In patients with HCM, a 12-lead ECG is recommended in the initial evaluation and as part of periodic follow-up (every 1 to 2 years)
|
B-NR
|
| Class I
|
Level of Evidence
|
| 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 SCD and guide management of arrhythmias.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 3. In patients with HCM who develop palpitations or lightheadedness, extended (>24 hours) electrocardiographic monitoring or event recording is recommended, which should not be considered diagnostic unless patients have had symptoms while being monitored.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 4. In first-degree relatives of patients with HCM, a 12-lead ECG is recommended as a component of the screening algorithm.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 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 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)
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 5. In adult patients with HCM without risk factors for AF and who are eligible for anticoagulation, extended ambulatory monitoring may be considered to assess for asymptomatic paroxysmal AF as part of initial evaluation and periodic follow-up (every 1 to 2 years).
|
B-NR
|
Recommendation for Angiography and Invasive Hemodynamic Assessment
Recommendation for Exercise Stress Testing
Recommendation for Genetics and Family Testing
| Class I
|
Level of Evidence
|
| 1. In patients with HCM, evaluation of familial inheritance, including a 3-generation family history, is recommended as part of the initial assessment.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In patients with HCM, genetic testing is beneficial to elucidate the genetic basis to facilitate the identification of family members at risk for developing HCM (cascade testing).
|
B-NR
|
| Class I
|
Level of Evidence
|
| 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 HCM and other genetic causes of unexplained cardiac hypertrophy (“HCM phenocopies”) is recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 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 risks, benefits, results, and their clinical significance can be reviewed and discussed with the patient in a shared decision-making process.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 5. When performing genetic testing in an HCM proband, the initial tier of genes tested should include genes with strong evidence to be disease-causing in HCM.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 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 has been identified in the proband) should be offered.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 7. In families where a sudden unexplained death has occurred with a postmortem diagnosis of HCM, postmortem genetic testing is beneficial to facilitate cascade genetic testing and clinical screening in first-degree relatives.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 8. In patients with HCM who have undergone genetic testing, serial reevaluation of the variant(s) identified is recommended to assess for variant reclassification, which may impact diagnosis and cascade genetic testing in family members.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 9. In affected families with HCM, preconception and prenatal reproductive and genetic counseling should be offered.
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 10. In patients with HCM, the usefulness of genetic testing in the assessment of risk of SCD is uncertain.
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 11. In patients with HCM who harbor a variant of uncertain significance, the usefulness of clinical genetic testing of phenotype-negative relatives for the purpose of variant reclassification is uncertain.
|
B-NR
|
| Class III: No Benefit
|
Level of Evidence
|
| 12. For patients with HCM who have undergone genetic testing and were found to have no pathogenic variants (ie, harbor only benign/likely benign variants), cascade genetic testing of the family is not useful.
|
B-NR
|
| Class III: No Benefit
|
Level of Evidence
|
| 13. Ongoing clinical screening is not indicated in genotype-negative relatives in families with genotype-positive HCM, unless the disease-causing variant is downgraded to variant of uncertain significance, likely benign, or benign variant during follow-up.
|
B-NR
|
Recommendations for Individuals Who Are Genotype-Positive, Phenotype-Negative
Recommendations for SCD Risk Assessment
| Class I
|
Level of Evidence
|
| 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 arrest, or sustained ventricular arrhythmias
d. Maximal LV wall thickness, EF, LV apical aneurysm
e. Non-sustained ventricular tachycardia episodes on continuous ambulatory electrocardiographic monitoring
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. For patients with HCM who are not otherwise identified as high risk for SCD, or in whom a decision to proceed with ICD placement remains uncertain after clinical assessment that includes personal/]]family history]], echocardiography, and ambulatory electrocardiographic monitoring, CMR imaging is beneficial to assess for maximum LV wall thickness, EF, LV apical aneurysm, and extent of myocardial fibrosis with late gadolinium enhancement
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 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 gradient to aid in calculating an estimated 5-year sudden death risk that may be useful during shared decision-making for ICD placement
|
B-NR
|
Recommendations for ICD Placement in High-Risk Patients With HCM
| Class I
|
Level of Evidence
|
| 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 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.
|
C-EO
|
| Class I
|
Level of Evidence
|
| 2. For patients with HCM, and previous documented cardiac arrest or sustained ventricular tachycardia, ICD placement is recommended.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 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 left ventricular hypertrophy ≥30 mm in any left ventricular segment;
c. ≥1 Recent episodes of syncope suspected by clinical history to be arrhythmic (ie, unlikely to be of neurocardiogenic [[[vasovagal]]] etiology, or related to LVOTO);
d. LV apical aneurysm, independent of size;
e. LV systolic dysfunction (EF <50%)
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 4. For children with HCM who have ≥1 conventional risk factors, including unexplained syncope, massive left ventricular hypertrophy, non-sustained ventricular tachycardia, or family history of early HCM-related SCD, ICD placement is reasonable after considering the relatively high complication rates of long-term ICD placement in younger patients.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 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 rates can be useful during the shared decision-making process for ICD placement.
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 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 remains otherwise uncertain, ICD may be considered in patients with extensive late gadolinium enhancement by contrast-enhanced CMR imaging or non-sustained ventricular tachycardia present on ambulatory monitoring.
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 7. In select pediatric patients with HCM in whom risk stratification is otherwise less certain, it may be useful to consider additional factors such as extensive late gadolinium enhancement on contrast-enhanced CMR imaging and systolic dysfunction in risk stratification.
|
C-LD
|
| Class III:Harm
|
Level of Evidence
|
| 8. In patients with HCM without risk factors, ICD placement should not be performed.
|
B-NR
|
| Class III:Harm
|
Level of Evidence
|
| 9. In patients with HCM, ICD placement for the sole purpose of participation in competitive athletics should not be performed.
|
B-NR
|
Recommendations for Selection of ICD Device Type
| Class I
|
Level of Evidence
|
| 1. In patients with HCM who are receiving an ICD, either a single chamber trans-venous ICD or a subcutaneous ICD is recommended after a shared decision-making discussion that takes into consideration patient preferences, lifestyle, and expected potential need for pacing for bradycardia or ventricular tachycardia termination.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In patients with HCM who are receiving an ICD, single-coil ICD leads are recommended in preference to dual coil leads.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 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 bradycardia/conduction abnormalities, or as an attempt to relieve symptoms of obstructive HCM (most commonly in patients >65 years of age).
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 4. In selected adult patients with non-obstructive HCM receiving an ICD who have NYHA class II to ambulatory class IV HF, left bundle branch block (LBBB), and LV ejection fraction (LVEF) <50%, cardiac resynchronization therapy for symptom reduction is reasonable.
|
C-LD
|
| Class IIb
|
Level of Evidence
|
| 5. In patients with HCM in whom a decision has been made for ICD implantation and who have paroxysmal atrial tachycardias or AF, dual-chamber ICDs may be reasonable, but this decision must be balanced against higher complication rates of dual chamber devices.
|
C-LD
|
Recommendations for Pharmacologic Management of Patients With Obstructive HCM
| Class I
|
Level of Evidence
|
| 1. In patients with obstructive HCM and symptoms attributable to LVOTO, non-vasodilating beta blockers, titrated to effectiveness or maximally tolerated doses, are recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In patients with obstructive HCM and symptoms attributable to LVOTO, for whom beta blockers are ineffective or not tolerated, substitution with non-dihydropyridine calcium channel blockers (eg, verapamil, diltiazem) is recommended.
|
Verapamil
B-NR
|
| Diltiazem
C-LD
|
| Class I
|
Level of Evidence
|
| 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 drugs, or SRT performed at experienced centers,† is recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 4. For patients with obstructive HCM and acute hypotension who do not respond to fluid administration, intravenous phenylephrine (or other vasoconstrictors without inotropic activity), alone or in combination with beta-blocking drugs, is recommended.
|
C-LD
|
| Class IIb
|
Level of Evidence
|
| 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 management and therapy, cautious use of low-dose oral diuretics may be considered.
|
C-EO
|
| Class IIb
|
Level of Evidence
|
| 6. For patients with obstructive HCM, discontinuation of vasodilators (eg, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, dihydropyridine calcium channel blockers) or digoxin may be reasonable because these agents can worsen symptoms caused by dynamic outflow tract obstruction.
|
C-EO
|
| Class III: Harm
|
Level of Evidence
|
| 7. For patients with obstructive HCM and severe dyspnea at rest, hypotension, very high resting gradients (eg, >100 mm Hg), as well as all children <6 weeks of age, verapamil is potentially harmful.
|
C-LD
|
Recommendations for Invasive Treatment of Symptomatic Patients With Obstructive HCM
| Class I
|
Level of Evidence
|
| 1. In patients with obstructive HCM who remain severely symptomatic despite guideline-directed management and therapy, SRT in eligible patients,performed at experienced centers,† is recommended for relieving LVOTO.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In symptomatic patients with obstructive HCM who have associated cardiac disease requiring surgical treatment (eg, associated anomalous papillary muscle, markedly elongated anterior mitral leaflet, intrinsic mitral valve disease, multi-vessel coronary artery disease, valvular aortic stenosis), surgical myectomy, performed at experienced centers,† is recommended.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 3. In adult patients with obstructive HCM who remain severely symptomatic, despite guideline-directed management and therapy and in whom surgery is contraindicated or the risk is considered unacceptable because of serious co-morbidities or advanced age, alcohol septal ablation in eligible patients, performed at experienced centers,† is recommended.
|
C-LD
|
| Class IIb
|
Level of Evidence
|
| 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 mitral regurgitation.
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).
|
B-NR
|
| Class IIb
|
Level of Evidence
|
| 5. For severely symptomatic patients with obstructive HCM, SRT in eligible patients, performed at experienced centers, may be considered as an alternative to escalation of medical therapy after shared decision-making including risks and benefits of all treatment options.
|
C-LD
|
| Class III: Harm
|
Level of Evidence
|
| 6. For patients with HCM who are asymptomatic and have normal exercise capacity, SRT is not recommended.
|
C-LD
|
| Class III: Harm
|
Level of Evidence
|
| 7. For symptomatic patients with obstructive HCM in whom SRT is an option, mitral valve replacement should not be performed for the sole purpose of relief of LVOTO.
|
B-NR
|
Recommendations for Management of Patients With Non-obstructive HCM With Preserved EF
Recommendations for Management of Atrial Fibrillation
| Class I
|
Level of Evidence
|
| 1. In patients with HCM and clinical AF, anticoagulation is recommended with direct-acting oral anticoagulants as first-line option and vitamin K antagonists as second-line option, independent of CHA2DS2-VASc score.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In patients with HCM and subclinical AF detected by internal or external cardiac device or monitor of >24 hours’ duration for a given episode, anti-coagulation is recommended with direct-acting oral anticoagulants as first-line option and vitamin K antagonists as second-line option, independent of CHA2DS2-VASc score.
|
C-LD
|
| Class I
|
Level of Evidence
|
| 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 patient preferences and co-morbid conditions.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 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 direct-acting oral anticoagulants as first-line option and vitamin K antagonists as second-line option can be beneficial, taking into consideration duration of AF episodes, total AF burden, underlying risk factors, and bleeding risk.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 5. In patients with HCM and poorly tolerated AF, a rhythm control strategy with cardioversion or antiarrhythmic drugs can be beneficial with the choice of an agent according to AF symptom severity, patient preferences, and co-morbid conditions.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 6. In patients with HCM and symptomatic AF, as part of a AF rhythm control strategy, catheter ablation for AF can be effective when drug therapy is ineffective, contraindicated, or not the patient’s preference.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 7. In patients with HCM and AF who require surgical myectomy, concomitant surgical AF ablation procedure can be beneficial for AF rhythm control.
|
B-NR
|
Recommendations for the Management of Patients With HCM and Ventricular Arrhythmias
| Class I
|
Level of Evidence
|
| 1. In patients with HCM and recurrent poorly tolerated life-threatening ventricular tachyarrhythmias refractory to maximal anti-arrhythmic drug therapy and ablation, heart transplantation assessment is indicated in accordance with current listing criteria.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In adults with HCM and symptomatic ventricular arrhythmias or recurrent ICD shocks despite beta blocker use, anti-arrhythmic drug therapy listed is recommended, with the choice of agent guided by age, underlying co-morbidities, severity of disease, patient preferences, and balance between efficacy and safety.
|
| Amiodarone
C-LD
|
| Dofetilide
C-LD
|
| Mexiletine
C-LD
|
| Sotalol
C-LD
|
| Class I
|
Level of Evidence
|
| 3. In children with HCM and recurrent ventricular arrhythmias despite beta blocker use, anti-arrhythmic drug therapy is recommended, with the choice of agent guided by age, underlying co-morbidities, severity of disease, patient preferences, and balance of efficacy and safety.
|
C-LD
|
| Class I
|
Level of Evidence
|
| 4. In patients with HCM and pacing-capable ICDs, programming anti-tachycardia pacing is recommended to minimize risk of shocks.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 5. In patients with HCM and recurrent symptomatic sustained monomorphic ventricular tachycardia, or recurrent ICD shocks despite optimal device programming, and in whom anti-arrhythmic drug therapy is either ineffective, not tolerated, or not preferred, catheter ablation can be useful for reducing arrhythmia burden.
|
C-LD
|
Recommendations for Patients With HCM and Advanced HF
| Class I
|
Level of Evidence
|
| 1. In patients with HCM who develop systolic dysfunction with an LVEF <50%, guideline-directed therapy for HF with reduced EF is recommended.
|
C-LD
|
| Class I
|
Level of Evidence
|
| 2. In patients with HCM and systolic dysfunction, diagnostic testing to assess for concomitant causes of systolic dysfunction (such as coronary artery disease) is recommended.
|
C-LD
|
| Class I
|
Level of Evidence
|
| 3. In patients with non-obstructive HCM and advanced HF (NYHA functional class III to class IV despite guideline-directed therapy) cardio-pulmonary exercise test should be performed to quantify the degree of functional limitation and aid in selection of patients for heart transplantation or mechanical circulatory support.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 4. In patients with non-obstructive HCM and advanced HF (NYHA class III to class IV despite guideline-directed therapy) or with life-threatening ventricular arrhythmias refractory to maximal guideline-directed therapy, assessment for heart transplantation in accordance with current listing criteria is recommended.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 5. For patients with HCM who develop systolic dysfunction (LVEF <50%), it is reasonable to discontinue previously indicated negative inotropic agents (specifically, verapamil, diltiazem, or disopyramide).
|
C-EO
|
| Class IIa
|
Level of Evidence
|
| 6. In patients with non-obstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT) who are candidates for heart transplantation, continuous-flow LV assist device therapy is reasonable as a bridge to heart transplantation.
|
B-NR
|
| Class IIa
|
Level of Evidence
|
| 7. In patients with HCM and LVEF <50%, ICD placement can be beneficial.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 8. In patients with HCM and LVEF <50%, NYHA functional class II to class IV symptoms despite guideline-direct therapy, and LBBB, CRT can be beneficial to improve symptoms.
|
C-LD
|
Recommendations for Sports and Activity
| Class I
|
Level of Evidence
|
| 1. 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.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. For athletes with HCM, a comprehensive evaluation and shared discussion of potential risks of sports participation by an expert provider is recommended..
|
C-EO
|
| Class IIa
|
Level of Evidence
|
| 3. For most patients with HCM, participation in low-intensity competitive sports is reasonable.
|
C-EO
|
| Class IIa
|
Level of Evidence
|
| 4. In individuals who are genotype-positive, phenotype-negative for HCM, participation in competitive athletics of any intensity is reasonable.
|
C-LD
|
| Class IIb
|
Level of Evidence
|
| 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 understanding that eligibility decisions for competitive sports participation often involve third parties (eg, team physicians, consultants, and other institutional leadership) acting on behalf of the schools or teams.
|
C-LD
|
| Class III: Harm
|
Level of Evidence
|
| 6. In patients with HCM, ICD placement for the sole purpose of participation in competitive athletics should not be performed.
|
B-NR
|
Recommendations for Occupation in Patients With HCM
| Class IIa
|
Level of Evidence
|
| 1.For patients with HCM, it is reasonable to follow Federal Motor Carrier Safety Administration cardiovascular disease guidelines that permit driving commercial motor vehicles, if they do not have an ICD or any major risk factors for SCD and are following a guideline-directed management plan.
|
C-EO
|
| Class IIa
|
Level of Evidence
|
| 2. For pilot aircrew with a diagnosis of HCM, it is reasonable to follow Federal Aviation Administration guidelines that permit consideration of multicrew flying duties, provided they are asymptomatic, are deemed low risk for SCD, and can complete a maximal treadmill stress test at 85% peak heart rate.
|
C-EO
|
| Class IIb
|
Level of Evidence
|
| 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 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.
|
C-EO
|
Recommendations for Pregnancy in Patients With HCM
| Class I
|
Level of Evidence
|
| 1.For pregnant women with HCM and AF or other indications for anticoagulation, low-molecular-weight heparin or vitamin K antagonists (at maximum therapeutic dose of <5 mg daily) are recommended for stroke prevention.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 2. In pregnant women with HCM, selected beta blockers should be administered for symptoms related to outflow tract obstruction or arrhythmias, with monitoring of fetal growth.
|
C-LD
|
| Class I
|
Level of Evidence
|
| 3. In most pregnant women with HCM, vaginal delivery is recommended as the first-choice delivery option.
|
C-LD
|
| Class I
|
Level of Evidence
|
| 4. In affected families with HCM, pre-conceptional and prenatal reproductive and genetic counseling should be offered.
|
B-NR
|
| Class I
|
Level of Evidence
|
| 5. For pregnant women with HCM, care should be coordinated between their cardiologist and an obstetrician. For patients with HCM who are deemed high risk, consultation is advised with an expert in maternal-fetal medicine.
|
C-EO
|
| Class IIa
|
Level of Evidence
|
| 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 shared discussion regarding potential maternal and fetal risks, and initiation of guideline-directed therapy.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 7. In pregnant women with HCM, cardioversion for new or recurrent AF, particularly if symptomatic, is reasonable.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 8. In pregnant women with HCM, general or epidural anesthesia is reasonable, with precautions to avoid hypotension.
|
C-LD
|
| Class IIa
|
Level of Evidence
|
| 9. In pregnant women with HCM, it is reasonable to perform serial echocardiography, particularly during the second or third trimester when hemodynamic load is highest, or if clinical symptoms develop.
|
C-EO
|
| Class IIb
|
Level of Evidence
|
| 10. In pregnant women with HCM, fetal echocardiography may be considered for diagnosis of fetal HCM in the context of prenatal counseling.
|
C-EO
|
Recommendations for Patients With Comorbidities
References