Mavacamten Treatment for Symptomatic Obstructive Hypertrophic Cardiomyopathy

Introduction to Mavacamten Therapy

The American College of Cardiology recommends mavacamten as a second-line therapy for adults with symptomatic NYHA class II-III obstructive hypertrophic cardiomyopathy who remain symptomatic despite first-line beta-blockers or nondihydropyridine calcium channel blockers 1, 2

The American College of Cardiology suggests initiating nonvasodilating beta-blockers, titrated to resting heart rate <60-65 bpm, as first-line therapy for symptomatic obstructive hypertrophic cardiomyopathy 1, 3
Alternative first-line therapy includes verapamil or diltiazem (up to 480 mg/day) if beta-blockers are ineffective, contraindicated, or poorly tolerated 1, 3
Mavacamten is recommended as second-line therapy for adult patients with persistent symptoms 1, 2, 4

The American College of Cardiology advises mandatory interruption of mavacamten if left ventricular ejection fraction (LVEF) <50% at any visit 1, 5
LVEF reduction risk is estimated to be 5.7% due to the drug alone, and up to 7-10% when considering other clinical conditions 1, 2
Pregnancy is an absolute contraindication for mavacamten due to teratogenic effects 6

Efficacy data demonstrates that 30-60% of patients achieve improvement in left ventricular outflow tract (LVOT) gradients, symptoms, and functional capacity 1, 2

The American College of Cardiology recommends that mavacamten should be initiated in adult patients with symptomatic NYHA class II-III obstructive hypertrophic cardiomyopathy who remain symptomatic despite maximally tolerated first-line therapy with beta-blockers or nondihydropyridine calcium channel blockers, with a treatment hierarchy that positions mavacamten as second-line therapy alongside disopyramide or septal reduction therapy 7
Mavacamten is absolutely contraindicated in pregnancy due to teratogenic effects, and pregnancy screening is mandatory in women of childbearing potential before initiation 7
The American College of Cardiology advises against premature initiation of mavacamten before adequate trial of first-line beta-blockers or calcium channel blockers, and emphasizes the importance of ensuring effective contraception in women of childbearing potential 7

The American College of Cardiology recommends that a negative pregnancy test is mandatory in women of childbearing potential before initiating mavacamten 7