Adds drug class to hypertrophic cardiomyopathy guidelines, recommends more exercise

Updates to national guidelines for the management of hypertrophic cardiomyopathy (HCM) approve the use of cardiac myosin inhibitors in certain cases, greenlight more intensive physical activity, and expand treatment options for younger patients. I did.

The American College of Cardiology (ACC) and the American Heart Association (AHA) have published this guideline. Journal of the American College of Cardiology and CirculationEach.

“Physical activity recommendations continue to evolve with research,” Steve R. Omen, M.D., director of the Mayo Hypertrophic Cardiomyopathy Clinic in Rochester, Minn., and chairman of the guidelines committee, said in a press release from both organizations. . “As part of a healthy lifestyle, people with HCM are now encouraged to engage in low to moderate intensity physical activity. We understand how vigorous physical activity makes sense for some people. With shared decision-making between clinicians and physicians, some patients may be able to return to competitive sports.”

The updated document added a clarification: “No universal restrictions on strenuous physical activity or competitive sports are indicated.” Class 2a recommendations state that active recreational activities are reasonable after annual inspections and shared decision-making with “experts.” Class 2b recommendations require that an expert with experience in managing athletes with HCM review the patient's case, perform a comprehensive annual evaluation, and balance potential benefits with risks. They point out that there is no problem in considering competitive sports after taking common decision-making into consideration.

“Difficult treatment decisions – when reasonable alternatives exist, when the strength of the recommendation is weak (e.g. decisions relying on recommendation class 2b), or when they are particularly sensitive (e.g. interpretation of genetic tests, primary prevention implants)” “In the case of HCM-specific invasive procedures, the involvement of specialized HCM centers may be of great benefit,” the guideline authors noted.

Regarding treatment, cardiac myosin inhibitors are a class 1 option for patients with symptomatic obstructive HCM for whom first-line pharmacotherapy (beta-blockers or non-dihydropyridine calcium channel blockers) does not provide adequate symptom relief. has been added, but this only applies. To adults. Guidelines warn that cardiac myosin inhibitors should be discontinued in patients with HCM who develop sustained systolic dysfunction with a left ventricular ejection fraction <50%.

However, the authors note that this new class is being monitored under the FDA's Risk Evaluation and Mitigation Strategies (REMS) program and “may result in additional steps and time for both clinicians and patients.” It pointed out. Clinicians require special training to prescribe drugs, and patients require regular check-ups. ”

Some guidelines have also been updated for younger patients.

It was recommended that valsartan be considered to slow adverse cardiac remodeling in patients with non-obstructive HCM due to pathogenic or likely pathogenic cardiac sarcomere gene mutations if under 45 years of age (class 2b).

The guidelines previously recommended shared decision-making to consider implantable cardioverter-defibrillators for patients with HCM who have one or more major risk factors for sudden cardiac death and are 16 years of age or older. However, that age criterion has been removed.

New recommendations for pediatric HCM patients include exercise stress testing to determine functional capacity and prognosis, regardless of symptom status, given that children may not be able to easily explain their symptoms. is recommended. “A new risk calculator specifically for children and adolescents has been validated and may help young patients and their families contextualize their estimated risk of sudden cardiac death,” the guideline authors wrote. writing.

Additionally, a contraindication warning has been added for Mabacamten (Camjos) during pregnancy due to its potential for teratogenicity.

In addition to the ACC and AHA, this guideline was developed and approved in collaboration with the American College of Sports Medicine, the Heart Rhythm Society, the Pediatric and Congenital Electrophysiology Society, and the Cardiovascular Magnetic Resonance Society.