Cardiomyopathy 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy
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2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy |
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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]
| 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
| Class I | Level of Evidence |
| 1. 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. | C-LD |
| Class IIa | Level of Evidence |
| 2. 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. | C-LD |
Recommendation for Diagnosis, Initial Evaluation, and Follow-up
| Class I | Level of Evidence |
| 1. 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. | B-NR |
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 |
References
- ↑ Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P; et al. (2020). "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". Circulation. 142 (25): e533–e557. doi:10.1161/CIR.0000000000000938. PMID 33215938 Check
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