Abstract
Hypertrophic cardiomyopathy (HCM) is a disease characterized by four major aspects: left ventricular hypertrophy without apparent cause, familial nature, myocyte disarray and sudden cardiac death. Among young competitive athletes, sudden death is usually precipitated by hypertrophic cardiomyopathy. HCM is an autosomal dominant disease caused by mutations in 11 or more loci. In 70% of cases, mutations in cardiac myosin heavy chain gene have been identified. Dyspnea, syncope and angina are relatively common symptoms. Sudden death in certain groups of patients may occur. Clinical diagnosis is usually confirmed by echocardiography: it allows assessments of the wall thickness, the systolic function and the presence of left ventricular outflow tract obstruction. At present, cardiac MRI has been considered as the essential diagnostic tool of HCM. Medical therapy is generally effective in the treatment of HCM. If medical therapy fails, surgical techniques or chemical ablation of the interventricular septum must be developed. Implantable cardioverter defibrillator prevents sudden death.
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