The American Heart Association and the American College of Cardiology today released an updated guideline for managing patients with hypertrophic cardiomyopathy (HCM). The guideline encourages shared decision-making between the clinician and patient as essential when determining treatment course and updates recommendations for sudden cardiac death (SCD) risk assessment and HCM center referrals based on the latest evidence.

HCM is a disease that causes the heart muscle to become abnormally thick, which can make it harder for the heart to pump enough blood to the body. It’s estimated that 1 in every 500 people have HCM, but a large percentage of patients are undiagnosed. Many patients with HCM are asymptomatic, but those who do have symptoms suffer from fainting, chest pain, shortness of breath or heart palpitations.

The guideline updates the previous version, which was issued in 2011, to offer recommendations on the evaluation and management of patients with HCM and is geared to cardiovascular clinicians as well as non-cardiovascular clinicians.

Recommendations reflect recent evidence about diagnostic modalities such as electrocardiography, imaging and genetic testing; management of patients including medical therapies, septal reduction therapies and SCD risk assessment/prevention; and other considerations such as participation in activities/sports, occupation and pregnancy.

People with HCM may have additional medical conditions such as heart failure, atrial fibrillation and ventricular arrythmias, which means treatment decisions are not always clear cut. The updated guideline clarifies the varied treatments that may include medications such as a beta-blocker and/or a calcium channel blocker, a surgical procedure and/or device like an implantable cardioverter defibrillator (ICD). Different from the previous 2011 guideline, the updated guideline emphasizes shared decision-making in the management of HCM to personalize treatment options based on the patient’s goals and concerns.

“Shared decision-making, a dialogue between patients and their care team that includes full disclosure of all testing and treatment options, discussion of the risks and benefits of those options and, importantly, engagement of the patient to express their own goals, is particularly relevant in the management of conditions such as hypertrophic cardiomyopathy,” said Steve R. Ommen, MD, FACC, FAHA, professor of medicine at the Mayo Clinic College of Medicine and consultant in the department of cardiovascular medicine and chair of the writing committee for the guideline. “This updated guideline places emphasis on including the patient in the decision-making process rather than simply providing dogmatic lists of do’s and don'ts.”

While the guideline writers recognize that patients with HCM can be evaluated and treated by a cardiovascular care team, recommendations are made for patients with severe HCM, or those facing complex decisions, to be referred to multidisciplinary HCM centers to receive optimal care.

Guidance around participation in healthy physical activity has also been updated to make it clearer that recreational exercise is an option for patients with HCM. According to the updated guideline, participation in competitive sports may also now be considered in selected patients after a complete discussion between patients with HCM and their doctors about the potential risks.

“Increasingly, data affirm that the beneficial effects of exercise on general health can be extended to patients with HCM,” Ommen said. “Healthy recreational exercise (moderate intensity) has not been associated with increased risk of ventricular arrhythmia events in recent studies.”  

Furthering the personalized approach to care, the updated HCM Guideline also includes updated recommendations for assessing individual risk markers for SCD, which can help identify patients who may need an implantable cardioverter defibrillator (ICD), and counseling patients about the potential genetic transmission of HCM and screening options for family members.

The 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy will publish in the Journal of the American College of Cardiology and Circulation.

The guideline was written in collaboration with the Heart Failure Society of America, the American Society of Echocardiography, the Heart Rhythm Society, the Society of Cardiovascular Magnetic Resonance, the American Academy of Thoracic Surgery, and the Society for Cardiovascular Angiography and Interventions and was endorsed by the Pediatric & Congenital Electrophysiology Society.