Cardiomyopathy 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

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

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
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.	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