- 🔬Pathophysiology
- Autosomal-dominant sarcomere mutation → asymmetric septal hypertrophy → LV outflow obstruction (HOCM) + diastolic dysfunction.
- 🩺Signs & Symptoms
- Dyspnea, angina, syncope; harsh systolic murmur that ↑ with ↓preload (Valsalva, standing). May present as sudden cardiac death in a young athlete.
- 🎯Gold Standard Diagnosis
- Echocardiography (septal hypertrophy, systolic anterior motion of mitral valve, outflow gradient). ECG: LVH, deep septal Q waves.
- 💊First-line Treatment
- Beta-blocker (or non-DHP CCB); avoid dehydration, pure vasodilators, high-dose diuretics. If refractory: disopyramide/mavacamten, then myectomy/ablation; ICD if high SCD risk.