New HCM guideline endorses cardiac myosin inhibitors, encourages exercise
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Key takeaways:
- Cardiac myosin inhibitors are now recommended as therapy for certain patients with obstructive hypertrophic cardiomyopathy.
- Patients with hypertrophic cardiomyopathy are encouraged to exercise.
A new guideline on hypertrophic cardiomyopathy endorses the use of cardiac myosin inhibitors for certain patients with obstructive HCM and encourages exercise in all patients with HCM.
The guideline, developed by the American College of Cardiology and the American Heart Association in collaboration with the American Medical Society for Sports Medicine, the Heart Rhythm Society, the Pediatric & Congenital Electrophysiology Society and the Society for Cardiovascular Magnetic Resonance, was published in the Journal of the American College of Cardiology and Circulation.
“Incorporating the most recent data, this new guideline equips clinicians with the latest recommendations for the treatment of HCM,” Steve R. Ommen, MD, FACC, FAHA, medical director of the Mayo Hypertrophic Cardiomyopathy Clinic and chair of the guideline writing committee, said in a press release. “We’re seeing more evidence that patients with HCM can return to their normal daily lives with proper care and management.”
Cardiac myosin inhibitors
Cardiac myosin inhibitors now have a class I, level of evidence B (randomized) indication for use in adults with symptomatic obstructive HCM who have persistent symptoms due to left ventricular outflow tract obstruction despite use of beta-blockers (recommended as first-line therapy) or nondihydropyridine calcium channel blockers (recommended if beta-blockers are ineffective). Currently, the only cardiac myosin inhibitor approved by the FDA is mavacamten (Camzyos, Bristol Myers Squibb), but also in development is aficamten (Cytokinetics).
“Mavacamten was the first drug designed specifically to treat HCM’s underlying pathophysiology,” Ommen told Healio. “It’s important that we include that in the guidelines so that cardiologists who might not work at centers of excellence understand when to start considering it in the treatment of their patients.”
Also recommended as therapies beyond beta-blockers and calcium channel blockers for patients with symptomatic obstructive HCM who have persistent symptoms due to left ventricular outflow tract obstruction is disopyramide in combination with an atrioventricular nodal blocking agent and septal reduction therapy performed in an experienced center.
Benefits of exercise
The guideline authors wrote that there is now enough evidence to show that the health benefits of exercise also extend to patients with HCM, and that light, moderate and vigorous exercise have all not been associated with ventricular arrhythmias in short-term studies.
Should a patient with HCM wish to participate in rigorous exercise training for performance or competition, they should discuss risks and benefits with HCM experts, develop a training plan and be re-evaluated regularly, the authors wrote.
“Historically, there were data that suggested that HCM was dangerous in athletes,” Ommen told Healio. “Unfortunately, that evolved to exercise is dangerous, not just competitive athletics. With some new data that have come out over the past couple of years, we have increasing data that low- to moderate-intensity exercise, even vigorous exercise as defined by some parameters that are in the document, can be safe and healthy for patients with HCM. That allows us to have those patients live active, healthy lifestyles like the rest of us are supposed to. We did not extend that to say every patient with HCM can play competitive sports if they want to. That is an individual discussion. But we did make sure we wrote in the statement that the presence of HCM should not result in automatic disqualification from participation.”
The guideline also states that each patient should be assessed for risk for sudden cardiac death, that sudden cardiac death risk factors can be different for pediatric and adult patients, that patients with HCM and atrial fibrillation should be treated with oral anticoagulants regardless of CHA2DS2-VASc score and that exercise stress testing is helpful to determine exercise tolerance and latent exercise-provoked LV outflow tract obstruction.
While the guideline reflects the latest data on HCM, many questions remain unanswered, Ommen said.
“There are a lot of research gaps remaining in the treatment of symptomatic nonobstructive hypertrophic cardiomyopathy,” Ommen told Healio. “There is always a need for better risk prediction for sudden cardiac death and other adverse outcomes in HCM.”
References:
- Exercise, New Drug Class Recommended for Management of Hypertrophic Cardiomyopathy. https://newsroom.heart.org/news/exercise-new-drug-class-recommended-for-management-of-hypertrophic-cardiomyopathy. Published May 8, 2024. Accessed May 8, 2024.
- Ommen SR, et al. Circulation. 2024;doi:10.1161/10.1161/CIR.0000000000001250.
For more information:
Steve R. Ommen, MD, FACC, FAHA, can be reached at ommen.steve@mayo.edu; X (Twitter): @steveommen.
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