Familial Hypertrophic Cardiomyopathy

Abstract

Hypertrophic Cardiomyopathy is the most common genetic cardiovascular disease. 70% of affected individuals demonstrate resting or provoked obstruction across the left ventricle (LV) outflow tract. Pathophysiology and clinical course are determined by the hypertrophy of LV. dynamic and mechanical left ventricular outflow tract occlusion, mitral regurgitation, diastolic dysfunction and myocardial ischemia, fibrosis, ventricular tachycardia and risk of sudden death. Patients are initially managed medically, however, significant heart failure symptoms or syncope in spite of optimal drug therapy and significant obstruction require invasive therapy. We report a case of familial hypertrophic cardiomyopathy treated successfully by surgical myectomy.