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Mavacamten Mechanism and Monitoring — EECC MCQ

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ModerateCardiomyopathyMavacamten Mechanism and MonitoringEECC

A 48-year-old man with HCM (MWT 28 mm, resting LVOT gradient 10 mmHg, provocable gradient 75 mmHg) and NYHA II symptoms on bisoprolol 10 mg is considered for mavacamten. What is the mechanism of mavacamten and key monitoring requirement?

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Correct answer: A — Mavacamten is a cardiac myosin inhibitor that reduces actin-myosin cross-bridge formation; LVEF must be monitored regularly as excessive reduction can occur

Mavacamten is a first-in-class cardiac myosin inhibitor that reduces the number of actin-myosin cross-bridges in the sarcomere, thereby decreasing excessive contractility and LVOT obstruction in obstructive HCM. The EXPLORER-HCM and VALOR-HCM trials demonstrated significant reduction in LVOT gradients and improvement in symptoms and exercise capacity. The 2023 ESC Cardiomyopathy Guidelines include mavacamten as a Class IIa recommendation for symptomatic obstructive HCM refractory to beta-blocker/verapamil/disopyramide. Critical monitoring: LVEF must be assessed at baseline and every 12 weeks, as mavacamten can cause excessive LVEF reduction (<50%); the drug should be withheld if LVEF drops below 50%.

Reference: ESC (2023): Guidelines on Cardiomyopathies; EXPLORER-HCM Trial