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2020 AHA/ACC Guideline for the Diagnosis and Treatment Of Circulation AHA/ACC CLINICAL PRACTICE GUIDELINE 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines WRITING COMMITTEE MEMBERS* Developed in collaboration with and Steve R. Ommen, MD, FACC, FAHA, Chair† endorsed by the American Association Seema Mital, MD, FACC, FAHA, FRCPC, Vice Chair† for Thoracic Surgery, American Society of Echocardiography, Heart Failure Society Michael A. Burke, MD† of America, Heart Rhythm Society, Sharlene M. Day, MD† Society for Cardiovascular Angiography Anita Deswal, MD, MPH, FACC, FAHA‡§ and Interventions, and Society for Perry Elliott, MD, FRCP, FACC† Cardiovascular Magnetic Resonance. Lauren L. Evanovich, PhD† Endorsed by The Pediatric & Congenital Judy Hung, MD, FACC‖ Electrophysiology Society José A. Joglar, MD, FACC, FAHA† ACC/AHA Joint Committee Members, Paul Kantor, MBBCh, MSc, FRCPC† see page e608 Carey Kimmelstiel, MD, FACC, FSCAI† Downloaded from http://ahajournals.org by on February 26, 2021 Michelle Kittleson, MD, PhD, FACC† Mark S. Link, MD, FACC¶ Martin S. Maron, MD# Matthew W. Martinez, MD, FACC† Christina Y. Miyake, MD, MS† Hartzell V. Schaff, MD, FACC** Christopher Semsarian, MBBS, PhD, MPH, FAHA† Paul Sorajja, MD, FACC, FAHA, FSCAI†† Key Words: AHA Scientific Statements ◼ guidelines ◼ hypertrophic cardiomyopathy ◼ sarcomeric genes ◼ shared decision-making ◼ echocardiography ◼ cardiovascular magnetic resonance ◼ exercise stress testing ◼ left ventricular outflow tract obstruction ◼ systolic dysfunction ◼ diastolic dysfunction ◼ genetics ◼ family screening ◼ sudden cardiac death ◼ ventricular arrhythmias ◼ atrial fibrillation ◼ rhythm monitoring ◼ risk *Writing committee members are required to recuse themselves from voting on sections to which their specific stratification ◼ implantable cardioverter relationships with industry may apply; see Appendix 1 for detailed information. †ACC/AHA Representative. ‡ACC/ defibrillator ◼ septal reduction therapy AHA Joint Committee on Clinical Practice Guidelines Liaison. §HFSA Representative. ||ASE Representative. ¶HRS ◼ surgical myectomy ◼ septal alcohol Representative. #SCMR Representative. **AATS Representative. ††SCAI Representative. ablation ◼ physical activity ◼ pregnancy ◼ occupation The American Heart Association requests that this document be cited as follows: Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P, Evanovich LL, Hung J, Joglar JA, Kantor P, Kimmelstiel C, Kittleson M, Link MS, Maron © 2020 by the American Heart MS, Martinez MW, Miyake CY, Schaff HV, Semsarian C, Sorajja P. 2020 AHA/ACC guideline for the diagnosis and Association, Inc. and the American treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology/American College of Cardiology Foundation. Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2020;142:e558–e631. doi: 10.1161/ CIR.0000000000000937 https://www.ahajournals.org/journal/circ e558 December 22/29, 2020 Circulation. 2020;142:e558–e631. DOI: 10.1161/CIR.0000000000000937 Ommen et al 2020 AHA/ACC Guideline for Hypertrophic Cardiomyopathy TABLE OF CONTENTS 9.1. Sports and Activity ��������������������������������������� e602 STATEMENTS CLINICAL 9.2. Occupation . e604 AND GUIDELINES Top 10 Take-Home Messages–2020 AHA/ACC 9.3. Pregnancy ��������������������������������������������������� e605 Guideline for the Diagnosis and Treatment of Patients 9.4. Comorbidities . e606 With Hypertrophic Cardiomyopathy . e559 10. Unmet Needs . e607 Preamble ��������������������������������������������������������������������� e560 10.1. Limitations and Knowledge Gaps ����������������� e607 1. Introduction . e562 10.1.1. Clinical Trials ����������������������������������� e607 1.1. Methodology and Evidence Review . e562 10.1.2. Prevent or Attenuate Disease 1.2. Organization of the Writing Committee ����������� e562 Progression ������������������������������������� e607 1.3. Document Review and Approval . e563 10.1.3. Reduce Symptom Burden and 1.4. Scope of the Guideline . e563 Increase Functional Capacity, 1.5. Class of Recommendation and Level of Particularly in Nonobstructive HCM . e607 Evidence . e564 10.1.4. Risk Stratification . e607 1.6. Abbreviations . e564 10.1.5. Arrhythmia Management . e607 2. Definition, Etiology, Clinical Course, and Natural 10.1.6. Genetics ����������������������������������������� e608 History ��������������������������������������������������������������������� e564 10.1.7. Exercise and Sports Participation ����� e608 2.1. Prevalence ������������������������������������������������������� e564 References ������������������������������������������������������������������� e609 2.2. Nomenclature/Differential Diagnosis . e564 Appendix 1. Author Relationships With Industry and 2.3. Definition, Clinical Diagnosis, and Phenotype ��� e565 Other Entities (Relevant) ��������������������������� e626 2.4. Etiology ����������������������������������������������������������� e565 Appendix 2. Reviewer Relationships With Industry and 2.5. Natural History/Clinical Course ������������������������� e566 Other Entities (Comprehensive) ����������������� e628 3. Pathophysiology . e566 3.1. LVOT Obstruction . e566 3.2. Diastolic Dysfunction ��������������������������������������� e567 3.3. Mitral Regurgitation . e567 TOP 10 TAKE-HOME MESSAGES– 3.4. Myocardial Ischemia . e567 2020 AHA/ACC GUIDELINE FOR 3.5. Autonomic Dysfunction ����������������������������������� e567 THE DIAGNOSIS AND TREATMENT 4. Shared Decision-Making . e568 5. Multidisciplinary HCM Centers ��������������������������������� e569 OF PATIENTS WITH HYPERTROPHIC 6. Diagnosis, Initial Evaluation, and Follow-up . e570 CARDIOMYOPATHY 6.1. Clinical Diagnosis ��������������������������������������������� e570 Downloaded from http://ahajournals.org by on February 26, 2021 1. Shared decision-making, a dialogue between 6.2. Echocardiography . e571 6.3. Cardiovascular Magnetic Resonance Imaging ��� e574 patients and their care team that includes full dis- 6.4. Cardiac Computed Tomography . e576 closure of all testing and treatment options, dis- 6.5. Heart Rhythm Assessment ������������������������������� e576 cussion of the risks and benefits of those options 6.6. Angiography and Invasive Hemodynamic and, importantly, engagement of the patient to Assessment ����������������������������������������������������� e577 express their own goals, is particularly relevant in 6.7. Exercise Stress Testing . e578 the management of conditions such as hypertro- 6.8. Genetics and Family Screening ������������������������� e579 phic cardiomyopathy (HCM). 6.9. Genotype-Positive, Phenotype-Negative ����������� e583 2. Although the primary cardiology team can ini- 7. SCD Risk Assessment and Prevention ����������������������� e584 tiate evaluation, treatment, and longitudinal 7.1. SCD Risk Assessment ��������������������������������������� e584 care, referral to multidisciplinary HCM centers 7.2. Patient Selection for ICD Placement ����������������� e586 with graduated levels of expertise can be impor- 7.3. Device Selection Considerations ����������������������� e588 8. Management of HCM ��������������������������������������������� e590 tant to optimizing care for patients with HCM. 8.1. Management of Symptomatic Patients With Challenging treatment decisions—where reason- Obstructive HCM ��������������������������������������������� e590 able alternatives exist, where the strength of rec- 8.1.1. Pharmacologic Management of ommendation is weak (eg, any Class 2b decision) Symptomatic Patients With Obstructive or is particularly nuanced, and for invasive pro- HCM ����������������������������������������������������e590 cedures that are specific to patients with HCM— 8.1.2. Invasive Treatment of Symptomatic represent crucial opportunities to refer patients to Patients With Obstructive HCM ������������� e591 these HCM centers. 8.2. Management of Patients With Nonobstructive 3. Counseling patients with HCM regarding the HCM With Preserved EF ����������������������������������� e593 potential for genetic transmission of HCM is 8.3. Management of Patients With HCM and one of the cornerstones of care. Screening first- Atrial Fibrillation . e595 8.4. Management of Patients With HCM and degree family members of patients with HCM, Ventricular Arrhythmias ����������������������������������� e597 using either genetic testing or an imaging/elec- 8.5. Management of Patients With HCM and trocardiographic surveillance protocol, can begin Advanced HF ��������������������������������������������������� e599 at any age and can be influenced by specifics of 9. Lifestyle Considerations for Patients With HCM ������� e602 the patient/family history and family preference. Circulation. 2020;142:e558–e631. DOI: 10.1161/CIR.0000000000000937 December 22/29, 2020 e559 Ommen et al 2020 AHA/ACC Guideline for Hypertrophic Cardiomyopathy As screening recommendations for family mem- option independent of the CHA2DS2VASc score. bers hinge on the pathogenicity of any detected As rapid atrial fibrillation is often poorly toler- variants, the reported pathogenicity should be ated in patients with HCM, maintenance of sinus reconfirmed every 2 to 3 years. rhythm and rate control are key pursuits in suc- 4. Optimal care for patients with HCM requires cardiac cessful treatment. imaging to confirm the diagnosis, characterize the 9. Heart failure symptoms in patients with
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