A 38-year-old woman presents with syncope during exercise. Echocardiography reveals asymmetric septal hypertrophy of 24 mm, systolic anterior motion of the mitral valve, and a resting LVOT gradient of 50 mmHg. She has a family history of sudden cardiac death at age 35 in her brother. What is the most appropriate first-line symptomatic management?
- A Mavacamten (cardiac myosin inhibitor)
- B Alcohol septal ablation
- C Beta-blocker (metoprolol or propranolol) ✓
- D Surgical myectomy (Morrow procedure)
Explanation
Beta-blockers are the first-line pharmacological therapy for obstructive HCM, reducing LVOT gradient, improving diastolic filling, and relieving exertional symptoms. Non-dihydropyridine calcium channel blockers (verapamil) are an alternative when beta-blockers are not tolerated. Mavacamten is a novel cardiac myosin inhibitor approved for symptomatic obstructive HCM in adults (EXPLORER-HCM trial) that reduces LVOT gradient and improves symptoms, indicated when beta-blockers or calcium channel blockers fail. Septal reduction therapies (surgical myectomy preferred; alcohol ablation as alternative) are for patients with severe refractory obstruction. Additionally, this patient needs ICD evaluation given her family history.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.