Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy

Publication Date: December 1, 2020
Last Updated: March 14, 2022

Treatment

Shared Decision-Making

For patients with HCM or at risk for HCM, shared decisionmaking 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 as the opportunity for patients to express their goals and concerns. (I, B-NR)
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Multidisciplinary HCM Centers

In patients with HCM in whom septal reduction therapy (SRT) is indicated, the procedure should be performed at experienced centers (comprehensive or primary HCM centers) with demonstrated excellence in clinical outcomes for these procedures) (Table 1 and Table 2). (I, C-LD)
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In patients with HCM, consultation with or referral to a comprehensive or primary HCM center is reasonable to aid in complex disease-related management decisions (Table 1). (IIa, C-LD)
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Clinical Diagnosis

Diagnosis Initial Evaluation and Follow-up

In patients with suspected HCM, comprehensive physical examination and complete medical and 3-generation family history is recommended as part of the initial diagnostic assessment (Table 3 and Table 4). (I, B-NR)
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Echocardiography

In patients with suspected HCM, a TTE is recommended in the initial evaluation. (I, B-NR)
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In patients with HCM with no change in clinical status or events, repeat TTE is recommended every 1–2 years to assess the degree of myocardial hypertrophy, dynamic LVOTO, MR, and myocardial function (Figure 1). (I, B-NR)
Adults
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Children
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For patients with HCM who experience a change in clinical status or a new clinical event, repeat TTE is recommended. (I, B-NR)
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For patients with HCM and resting LVOT gradient <50 mm Hg, a TTE with provocative maneuvers is recommended. (I, B-NR)
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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. (I, B-NR)
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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. (I, B-NR)
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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. (I, B-NR)
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For patients with HCM who have undergone SRT, TTE within 3–6 months after the procedure is recommended to evaluate the procedural results. (I, B-NR)
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Screening: In first-degree relatives of patients with HCM, a TTE is recommended as part of initial family screening and periodic follow-up (Figure 1, Table 4). (I, B-NR)
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Screening: In individuals who are genotype-positive or phenotype-negative, serial echocardiography is recommended at periodic intervals depending on age (1–2 years in children and adolescents, 3–5 years in adults) and change in clinical status (Figure 1, Table 4). (I, B-NR)
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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 MR secondary to structural abnormalities of the mitral valve apparatus, or in the assessment of the feasibility of alcohol septal ablation. (IIa, C-LD)
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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 CMR are not readily available or contraindicated. (IIa, B-NR)
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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. (IIa, C-LD)
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CMR Imaging

For patients suspected to have HCM in whom echocardiography is inconclusive, CMR imaging is indicated for diagnostic clarification. (I, B-NR)
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For patients with LVH in whom there is a suspicion of alternative diagnoses, including infiltrative or storage disease as well as athlete’s heart, CMR imaging is useful (Figure 1). (I, B-NR)
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For patients with HCM who are not otherwise identified as high risk for 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 imaging is beneficial to assess for maximum LV wall thickness, ejection fraction (EF), LV apical aneurysm, and extent of myocardial fibrosis with late gadolinium enhancement (LGE). (I, B-NR)
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For patients with obstructive HCM in whom the anatomic mechanism of obstruction is inconclusive on echocardiography, CMR imaging is indicated in order to inform the selection and planning of SRT. (I, B-NR)
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For patients with HCM, repeat contrast-enhanced CMR imaging on a periodic basis (every 3–5 years) for the purpose of SCD risk stratification may be considered to evaluate changes in LGE and other morphologic changes, including EF, development of apical aneurysm, or LV wall thickness (Figure 1, Table 5). (IIb, C-EO)
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Cardiac Computed Tomography (CT)

In adult patients with suspected HCM, cardiac CT may be considered for diagnosis if the echocardiogram is not diagnostic and CMR imaging is unavailable. (IIb, C-LD)
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Heart Rhythm Assessment

In patients with HCM, a 12-lead ECG is recommended in the initial evaluation and as part of periodic follow-up (every 1–2 years) (Figure 1, Table 4). (I, B-NR)
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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 1–2 years) to identify patients who are at risk for SCD and guide management of arrhythmias (Figure 1). (I, B-NR)
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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. (I, B-NR)
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In first-degree relatives of patients with HCM, a 12-lead ECG is recommended as a component of the screening algorithm (Figure 1, Table 4). (I, B-NR)
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In patients with HCM who have additional risk factors for AF, such as left atrial dilatation, advanced age, and New York Heart Association (NYHA) class III to class IV 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 1–2 years) (Figure 1). (IIa, B-NR)
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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 1–2 years). (IIb, B-NR)
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Angiography and Invasive Hemodynamic Assessment

For patients with HCM who are candidates for SRT and for whom there is uncertainty regarding the presence or severity of LVOTO on noninvasive imaging studies, invasive hemodynamic assessment with cardiac catheterization is recommended. (I, B-NR)
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In patients with HCM with symptoms or evidence of myocardial ischemia, coronary angiography (CT or invasive) is recommended. (I, B-NR)
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In patients with HCM who are at risk of coronary atherosclerosis, coronary angiography (CT or invasive) is recommended before surgical myectomy. (I, B-NR)
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Exercise Stress Testing

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 the detection and quantification of dynamic LVOTO. (I, B-NR)
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In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite guideline-directed management and therapy [GDMT]), cardiopulmonary exercise stress testing should be performed to quantify the degree of functional limitation and aid in selection of patients for heart transplantation or mechanical circulatory support. (I, B-NR)
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In patients with HCM, exercise stress testing is reasonable to determine functional capacity and to provide prognostic information as part of initial evaluation. (IIa, B-NR)
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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. (IIa, C-LD)
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In patients with obstructive HCM who are being considered for SRT and in whom functional capacity or symptom status is uncertain, exercise stress testing may be reasonable (Figure 1). (IIb, C-EO)
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In patients with HCM in whom functional capacity or symptom status is uncertain, exercise stress testing may be considered every 2–3 years (Figure 1). (IIb, C-EO)
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Genetics and Family Screening

In patients with HCM, evaluation of familial inheritance, including a 3-generation family history, is recommended as part of the initial assessment. (I, B-NR)
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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). (I, B-NR)
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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. (I, B-NR)
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In patients with HCM who choose to undergo genetic testing, pre- and post-test 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. (I, B-NR)
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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 HCMa. (I, B-NR)
Strong evidence a HCM genes include, at the time of this publication: MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, and ACTC1.
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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. (I, B-NR)
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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. (I, B-NR)
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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 variant reclassification, which may impact diagnosis and cascade genetic testing in family members (Figure 1 and Figure 2). (I, B-NR)
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In affected families with HCM, preconception and prenatal reproductive and genetic counseling should be offered. (I, B-NR)
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In patients with HCM, the usefulness of genetic testing in the assessment of risk of SCD is uncertain. (IIb, B-NR)
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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. (IIb, B-NR)
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For patients with HCM who have undergone genetic testing and were found to have no pathogenic variants (i.e., harbor only benign/likely benign variants), cascade genetic testing of the family is NOT useful. (III - No Benefit, B-NR)
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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. (III - No Benefit, B-NR)
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Strong evidence aHCM genes include, at the time of this publication: MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, and ACTC1.

Individuals Who Are Genotype-Positive, Phenotype-Negative

In individuals who are genotype-positive, phenotype-negative for HCM, serial clinical assessment, electrocardiography, and cardiac imaging is recommended at periodic intervals depending on age (every 1–2 years in children and adolescents, and every 3–5 years in adults) and change in clinical status (Figure 1 and Figure 2, Table 4). (I, B-NR)
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In individuals who are genotype-positive, phenotype-negative for HCM, participation in competitive athletics of any intensity is reasonable. (I, C-LD)
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In individuals who are genotype-positive, phenotype-negative for HCM, ICD is NOT recommended for primary prevention. (III - No Benefit, B-NR)
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SCD Risk Assessment

In patients with HCM, a comprehensive, systematic noninvasive SCD risk assessment at initial evaluation and every 1–2 years thereafter is recommended and should include evaluation of these risk factors (Figure 1 and Figure 3, Table 5):
  • Personal history of cardiac arrest or sustained ventricular arrhythmias
  • Personal history of syncope suspected by clinical history to be arrhythmic
  • Family history in close relative of premature HCM-related sudden death, cardiac arrest, or sustained ventricular arrhythmias
  • Maximal LV wall thickness, EF, LV apical aneurysm
  • NSVT episodes on continuous ambulatory electrocardiographic monitoring
(I, B-NR)
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For patients with HCM who are not otherwise identified as high risk for SCD, or in whom a decision to proceed with ICD implantation 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 LGE (Table 5). (I, B-NR)
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For patients who are ≥16 years of age with HCM, it is reasonable to obtain echocardiography-derived LA diameter and maximal LVOT gradient to aid in calculating an estimated 5-year sudden death risk that may be useful during shared decision-making for ICD placement (Table 5). (IIa, B-NR)
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ICD Placement in High-risk Patients With HCM

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. (I, C-EO)
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For patients with HCM, and previous documented cardiac arrest or sustained VT, ICD placement is recommended (Figure 3, Table 5). (I, B-NR)
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For adult patients with HCM with ≥1major risk factors for SCD, it is reasonable to offer an ICD. These major risk factors include (Figure 3, Table 5):
  • Sudden death judged definitively or likely attributable to HCM in ≥1 first-degree or close relatives who are ≤50 years of age;
  • Massive LVH ≥30 mm in any LV segment;
  • ≥1 recent episodes of syncope suspected by clinical history to be arrhythmic (i.e., unlikely to be of neurocardiogenic [vaso-vagal] etiology, or related to LVOTO);
  • LV apical aneurysm, independent of size;
  • LV systolic dysfunction (EF<50%).
(IIa, B-NR)
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For children with HCM who have ≥1 conventional risk factors, including unexplained syncope, massive LVH, NSVT, or family history of early HCM-related SCD, ICD placement is reasonable after considering the relatively high complication rates of long-term ICD implantation in younger patients) (Figure 3, Table 5). (IIa, B-NR)
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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 (Figure 3, Table 5). (IIa, B-NR)
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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 LGE by contrast-enhanced CMR imaging or NSVT present on ambulatory monitoring (Figure 3, Table 5). (IIb, B-NR)
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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 LGE on contrast-enhanced CMR imaging and systolic dysfunction in risk stratification (Figure 3, Table 5). (IIb, C-LD)
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In patients with HCM without risk factors, ICD placement should NOT be performed. (III - Harm, B-NR)
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In patients with HCM, ICD placement for the sole purpose of participation in competitive athletics should NOT be performed. (III - Harm, B-NR)
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Selection of ICD Device Type

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 consideration patient preferences, lifestyle, and expected potential need for pacing for bradycardia or VT termination. (I, B-NR)
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In patients with HCM who are receiving an ICD, single-coil ICD leads are recommended in preference to dual-coil leads. (I, B-NR)
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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). (IIa, B-NR)
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In selected adult patients with nonobstructive 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 (CRT) for symptom reduction is reasonable. (IIa, C-LD)
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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. (IIb, C-LD)
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Pharmacologic Management of Patients With Obstructive HCM

In patients with obstructive HCMa and symptoms attributable to LVOTO, nonvasodilating beta-blockers, titrated to effectiveness or maximally tolerated doses, are recommended. (I, B-NR)
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Verapamil (I, B-NR)
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Diltiazem (I, C-LD)
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For patients with obstructive HCMa 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 SRTb performed at experienced centers, is recommended. (I, B-NR)
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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. (I, C-LD)
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For patients with obstructive HCM and persistent dyspnea with clinical evidence of volume overload and high left-sided filling pressures despite other HCMGDMT, cautious use of low-dose oral diuretics may be considered. (IIb, C-EO)
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For patients with obstructive HCM, discontinuation of vasodilators (e.g., 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. (IIb, C-EO)
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For patients with obstructive HCM and severe dyspnea at rest, hypotension, very high resting gradients (e.g., >100 mm Hg), as well as all children <6 weeks of age, verapamil is potentially harmful. (III - Harm, C-LD)
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Symptoms include effort-related dyspnea or chest pain and occasionally other exertional symptoms (e.g., syncope, near syncope) that are attributed to aLVOTO and interfere with everyday activity or quality of life.
Comprehensive or primary bHCM centers with demonstrated excellence in clinical outcomes for these procedures (Table 1 and Table 2).

Invasive Treatment of Symptomatic Patients With Obstructive HCM

In patients with obstructive HCM who remain severely symptomatic despite GDMT, SRTa in eligible patients, performed at experienced centers, is recommended for relieving bLVOTO (Table 1 and Table 2). (I, B-NR)
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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 mitral valve disease, multivessel CADb, valvular aortic stenosis), surgical myectomy, performed at experienced centers, is recommended (Table 1 and Table 2). (I, B-NR)
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In adult patients with obstructive HCM who remain severely symptomatic despite GDMTa and in whom surgery is contraindicated or the risk is considered unacceptable because of serious comorbidities or advanced age, alcohol septal ablation in eligible patients, performed at experienced centers,b is recommended (Table 1 and Table 2). (I, C-LD)
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In patients with obstructive HCM, earlier (NYHA class II) surgical myectomy performed at comprehensive HCM centers (Table 1 and Table 2) may be reasonable in the presence of additional clinical factors, including:
  • Severe and progressive pulmonary hypertension thought to be attributable to LVOTO or associated MR.
  • Left atrial enlargement with ≥1 episodes of symptomatic AF.
  • Poor functional capacity attributable to LVOTO as documented on treadmill exercise testing.
  • Children and young adults with very high resting LVOT gradients (>100 mmHg).
(IIb, B-NR)
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For severely symptomatic patients with obstructive HCM, SRTa in eligible patients, performed at experienced centersb (Table 1 and Table 2), may be considered as an alternative to escalation of medical therapy after shared decision-making including risks and benefits of all treatment options. (IIb, C-LD)
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For patients with HCM who are asymptomatic and have normal exercise capacity, SRT is NOT recommended. (III - Harm, C-LD)
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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. (III - Harm, B-NR)
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a General eligibility criteria for septal reduction therapy:
  • 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.
  • 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.
  • Anatomic: Targeted anterior septal thickness sufficient to perform the procedure safely and effectively in the judgment of the individual operator.
Comprehensive or primary bHCM centers with demonstrated excellence in clinical outcomes for these procedures (Table 1 and Table 2).

Management of Patients With Nonobstructive HCM With Preserved EF

In patients with nonobstructive HCM with preserved EF and symptoms of exertional angina or dyspnea, beta-blockers or non-dihydropyridine calcium channel blockers are recommended. (I, C-LD)
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In patients with nonobstructive HCM with preserved EF, it is reasonable to add oral diuretics when exertional dyspnea persists despite the use of beta-blockers or non-dihydropyridine calcium channel blockers. (IIa, C-EO)
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In patients with nonobstructive HCM with preserved EF, the usefulness of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers in the treatment of symptoms (angina and dyspnea) is not well established. (IIb, C-LD)
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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 (LV2 end-diastolic volume <50 mL/m and LV2 stroke volume <30 mL/m), apical myectomy by experienced surgeons at comprehensive centers may be considered to reduce symptoms. (IIb, C-LD)
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In asymptomatic patients with nonobstructive HCM, the benefit of beta-blockers or calcium channel blockers is not well established. (IIb, C-EO)
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Management of Patients With HCM and Atrial Fibrillation

In patients with HCM and clinical AF, anticoagulation is recommended with direct-acting oral anticoagulants (DOAC2) as first-line option and vitamin K antagonists as second-line option, independent of CHADS2-VASc score. (I, B-NR)
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In patients with HCM and subclinical AF detected by internal or external cardiac device or monitor of >24 hours duration for a given episode, anticoagulation is recommended with DOAC2 as first-line option and vitamin K antagonists as second-line option, independent of CHADS2-VASc score. (I, C-LD)
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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 comorbid conditions. (I, C-LD)
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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 second-line option can be beneficial, taking into consideration duration of AF episodes, total AF burden, underlying risk factors, and bleeding risk. (IIa, C-LD)
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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 comorbid conditions. (IIa, B-NR)
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In patients with HCM and symptomatic AF, as part of an AF rhythm control strategy, catheter ablation for AF can be effective when drug therapy is ineffective, contraindicated, or not the patient’s preference. (IIa, B-NR)
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In patients with HCM and AF who require surgical myectomy, concomitant surgical AF ablation procedure can be beneficial for AF rhythm control. (IIa, B-NR)
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Management of Patients With HCM and Ventricular Arrhythmias

In patients with HCM and recurrent poorly tolerated life-threatening ventricular tachyarrhythmias refractory to maximal antiarrhythmic drug therapy and ablation, heart transplantation assessment is indicated in accordance with current listing criteria. (I, B-NR)
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In adults with HCM and symptomatic ventricular arrhythmias or recurrent ICD shocks despite beta-blocker use, antiarrhythmic drug therapy listed is recommended, with the choice of agent guided by age, underlying comorbidities, severity of disease, patient preferences, and balance between efficacy and safety. (I, B-NR)

Amiodarone
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Dofetilide (I, C-LD)
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Mexiletine

(I, C-LD)
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Sotalol (I, C-LD)
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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 by age, underlying comorbidities, severity of disease, patient preferences, and balance of efficacy and safety. (I, C-LD)
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In patients with HCM and pacing-capable ICDs, programming anti-tachycardia pacing is recommended to minimize risk of shocks. (I, C-LD)
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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 by age, underlying comorbidities, severity of disease, patient preferences, and balance of efficacy and safety. (I, C-LD)
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Management of Patients With HCM and Advanced HF

In patients with HCM who develop systolic dysfunction with an LVEF <50%, guideline-directed therapy for HF with reduced EF is recommended. (I, C-LD)
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In patients with HCM and systolic dysfunction, diagnostic testing to assess for concomitant causes of systolic dysfunction (such as CAD) is recommended. (I, C-LD)
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In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite guideline-directed therapy), CPET should be performed to quantify the degree of functional limitation and aid in selection of patients for heart transplantation or mechanical circulatory support. (I, B-NR)
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In patients with nonobstructive 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. (I, B-NR)
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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). (IIa, C-EO)
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In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT) who are candidates for heart transplantation, continuous-flow LVAD therapy is reasonable as a bridge to heart transplantation. (IIa, B-NR)
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In patients with HCM and LVEF <50%, ICD placement can be beneficial. (IIa, C-LD)
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In patients with HCM and LVEF <50%, NYHA functional class II to class IV symptoms despite guideline-directed therapy, and LBBB, CRT can be beneficial to improve symptoms. (IIa, C-LD)
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Sports and Activity

For most patients with HCMa, 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. (I, B-NR)
a 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.
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For athletes with HCM, a comprehensive evaluation and shared discussion of potential risks of sports participation by an expert provider is recommended. (I, C-EO)
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For most patients with HCM, participation in low-intensity competitive sports is reasonable. (IIa, C-EO)
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In individuals who are genotype-positive, phenotype-negative for HCM, participation in competitive athletics of any intensity is reasonable. (IIa, C-LD)
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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 (e.g., team physicians, consultants, and other institutional leadership) acting on behalf of the schools or teams. (IIb, C-LD)
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In patients with HCM, ICD placement for the sole purpose of participation in competitive athletics should NOT be performed. (III - Harm, B-NR)
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Occupation in Patients With HCM

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. (IIa, C-EO)
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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. (IIa, C-EO)
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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. (IIb, C-EO)
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Pregnancy

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. (I, B-NR)
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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. (I, C-LD)
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In most pregnant women with HCM, vaginal delivery is recommended as the first-choice delivery option. (I, C-LD)
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In affected families with HCM, preconceptional and prenatal reproductive and genetic counseling should be offered. (I, B-NR)
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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. (I, C-EO)
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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. (IIa, C-LD)
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In pregnant women with HCM, cardioversion for new or recurrent AF, particularly if symptomatic, is reasonable. (IIa, C-LD)
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In pregnant women with HCM, general or epidural anesthesia is reasonable, with precautions to avoid hypotension. (IIa, C-LD)
573
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. (IIa, C-EO)
573
In pregnant women with HCM, fetal echocardiography may be considered for diagnosis of fetal HCM in the context of prenatal counseling. (IIb, C-EO)
573

Comorbidities

In patients with HCM, adherence to the guidelines on the prevention of atherosclerotic cardiovascular disease is recommended to reduce risk of cardiovascular events. (I, C-EO)
573
In patients with HCM who are overweight or obese, counseling and comprehensive lifestyle interventions are recommended for achieving and maintaining weight loss and possibly lowering the risk of developing LVOTO, HF, and AF. (I, B-NR)
573
In patients with HCM and hypertension, lifestyle modifications and medical therapy for hypertension are recommended, with preference for beta-blockers and non-dihydropyridine calcium channel blockers in patients with obstructive HCM. (I, C-LD)
573
In patients with HCM, assessment for symptoms of sleep-disordered breathing is recommended and, if present, referral to a sleep medicine specialist for evaluation and treatment. (I, C-LD)
573

Recommendation Grading

Overview

Title

Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy

Authoring Organizations

Publication Month/Year

December 1, 2020

Last Updated Month/Year

February 13, 2024

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Inclusion Criteria

Male, Female, Adult, Child

Health Care Settings

Ambulatory, Long term care

Intended Users

Nurse, nurse practitioner, physician, physician assistant, social worker

Scope

Diagnosis, Treatment, Management

Diseases/Conditions (MeSH)

D002312 - Cardiomyopathy, Hypertrophic

Keywords

ischemia, atrial fibrillation, exercise, echocardiography, sudden cardiac death, coronary, Hypertophic Cardiomyopathy, systole, stroke volume

Supplemental Methodology Resources

Data Supplement

Methodology

Number of Source Documents
711
Literature Search Start Date
January 1, 2010
Literature Search End Date
April 30, 2020