A 30-year-old athlete is found to have asymmetric LV hypertrophy (maximum wall thickness 18 mm, septal-posterior ratio 1.6:1), LVOT gradient 50 mmHg at rest, systolic anterior motion of the mitral valve, and exertional syncope. Genetic testing confirms MYBPC3 mutation. Which is the most appropriate intervention for symptom control?

• A Nifedipine (dihydropyridine CCB) to reduce afterload and improve diastolic function
• B Digoxin to improve LV contractility and reduce LVOT gradient
• C Exercise training programme to achieve cardiac remodelling
• D Disopyramide plus beta-blocker if obstruction persists — or septal reduction therapy (myectomy/alcohol ablation) if refractory ✓

Explanation

Obstructive HCM (LVOT gradient ≥30 mmHg resting or ≥50 mmHg provoked) with symptoms is managed with negative inotropes: beta-blockers first line, then disopyramide added for persistent obstruction. Maven-HCM trial demonstrated mavacamten (myosin inhibitor) as a new option. Septal reduction therapy (surgical myectomy preferred; alcohol septal ablation in unsuitable surgical candidates) is offered for drug-refractory obstruction with severe symptoms. Vasodilators including dihydropyridine CCBs and digoxin are contraindicated as they worsen outflow obstruction. Competitive athletics is restricted.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.