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The AHA and ACC 2024 Clinical HCM management guidelines include revised and new recommendations regarding ICD placement, atrial fibrillation, and more.
Shared decision-making for patients of all ages with hypertrophic cardiomyopathy (HCM)—not only those who are at least 16 years of age—is among the revised, evidence-based 2024 HCM management guidelines issued by the American Heart Association and the American College of Cardiology Joint Committee on Clinical Practice Guidelines.
“Shared decision-making is essential to provide the best clinical care. This involves thoughtful dialogue among patients, families, and their care team in which health care professionals present all available testing and treatment options; discuss the risks, benefits, and applicability of those options to the individual patient; and ensure the patient expresses their personal preferences and goals to develop their treatment plan,” the authors wrote in Journal of the American College of Cardiology and Circulation. “Although the primary cardiology team can initiate evaluation, treatment, and longitudinal care, referral to multidisciplinary HCM centers with appropriate expertise can be important to optimizing care for patients with HCM.”
To update the 2020 guidelines, Steve R. Ommen, MD, and the writing committee reviewed human studies, reviews, and other evidence in the standard medical databases. They developed the 2024 guidelines in collaboration with and endorsed by the American Medical Society for Sports Medicine, the Heart Rhythm Society, the Pediatric & Congenital Electrophysiology Society, and the Society for Cardiovascular Magnetic Resonance.
The committee’s revised and new guidelines include:
Revised Guidelines:
- Heart Rhythm Assessment
- 2020: In patients with HCM who have additional risk factors for AF, including left atrial dilatation, advanced age, and New York Heart Association functional class III to class IV HF, and who are eligible for anticoagulation, extended ambulatory monitoring is reasonable to screen for AF during initial evaluation and periodic follow-up.
- 2024: In patients with HCM who are at high risk for developing AF based on risk factors, as determined by a validated risk score, and who are eligible for anticoagulation, extended ambulatory monitoring is recommended to screen for AF during initial evaluation and annual follow-up.
- Patient Selection for ICD Placement
- 2020: For patients 16 years of age and older with HCM and with one or more major sudden cardiac death (SCD) risk factors, discussing the estimated 5-year sudden death risk and mortality rates can be useful during shared decision-making for implantable cardioverter defibrillator (ICD) placement.
- 2024: For patients with HCM with one or more major SCD risk factor, discussing the estimated 5-year sudden death risk and mortality rates can be useful during shared decision-making for ICD placement.
- Pharmacological Management of Symptomatic Patients with Obstructive HCM
- 2020: For patients with obstructive HCM with persistent severe symptoms attributable to left ventricular outflow tract obstruction despite beta blockers or nondihydropyridine calcium channel blockers, either adding disopyramide in combination with one of the other drugs, or septal reduction therapy performed at experienced centers, is recommended.
- 2024: For patients with obstructive HCM with persistent symptoms attributable to left ventricular outflow tract obstruction despite beta blockers or nondihydropyridine calcium channel blockers, adding a myosin inhibitor (in adult patients only) or disopyramide (combined with an atrioventricular nodal blocking agent), or septal reduction therapy performed at experienced centers, is recommended.
- Recreational Physical Activity and Competitive Sports
- 2020: For patients with HCM, participating 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 informs that the risk for sudden death and ICD shocks may increase, and with the understanding that eligibility decisions for competitive sports participation often involve third parties such as team physicians, consultants, and other institutional leadership.
- 2024: For patients with HCM, participating in vigorous recreational activities is reasonable after an annual comprehensive evaluation and shared decision-making with an expert professional who balances potential risks and benefits. For patients with HCM who are capable of high-level physical performance, competitive sports may be considered after review by an expert provider with experience managing athletes with HCM who performs annual comprehensive evaluations and shared decision-making that balances potential risks and benefits.
New Recommendations:
- Exercise Stress Testing: In pediatric patients with HCM, regardless of symptoms, exercise stress testing is recommended to provide information about functional capacity and prognosis.
- Management of Patients With Nonobstructive HCM With Preserved Ejection Fraction: For patients 45 years of age or younger with nonobstructive HCM due to a pathogenic or likely pathogenic cardiac sarcomere genetic variant and a mild phenotype, valsartan may help slow adverse cardiac remodeling.
- Management of Patients With HCM and Advanced Heart Failure: In patients with HCM who develop persistent systolic dysfunction (left ventricular ejection fraction <50%), cardiac myosin inhibitors should be discontinued.
- Recreational Physical Activity and Competitive Sports: For most patients with HCM, complete restriction from vigorous physical activity or competitive sports is not indicated.
- Pregnancy in Patients With HCM: In pregnant women, mavacamten use is contraindicated due to potential teratogenicity.
“Clinical practice guidelines provide recommendations applicable to patients with or at risk of developing cardiovascular disease. The focus is on medical practice in the United States, but these guidelines are relevant to patients throughout the world. Although guidelines may be used to inform regulatory or payer decisions, the intent is to improve quality of care and align with patients’ interests. Guidelines are intended to define practices meeting the needs of patients in most, but not all, circumstances and should not replace clinical judgment,” the authors advised.