Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy is a myocardial disease characterized by myocardial hypertrophy, disorganization of cardiac myocytes, and fibrosis. Twenty-five percent of patients have a dynamic left ventricular outflow tract gradient caused by the combined effects of rapid ventricular ejection, a narrowed outflow tract, and systolic anterior motion of the mitral valve. Most cases are caused by mutations in genes that encode cardiac sarcomeric proteins. Patients present at all ages with chest pain, dyspnea, palpitations, and syncope. The most important complications of the disease are sudden cardiac death, heart failure, and thromboembolism. The principal aims of management are the alleviation of symptoms and the prevention of sudden death. In patients with substantial left ventricular outflow tract obstruction, interventions that reduce the magnitude of the outflow tract gradient (disopyramide, verapamil, β-blockade, alcohol ablation of the interventricular septum, dual-chamber pacing, and surgery) often improve symptoms. Therapeutic options in patients without left ventricular outflow tract obstruction are more limited. Clinical risk stratification is used to estimate the risk of sudden death and to target effective prophylactic treatment with an implantable cardioverter defibrillator.