title: [Hypertrophic cardiomyopathy: spontaneous course].
abstract: Hypertrophic cardiomyopathy is a relatively rare (prevalence approximately 0.2%), primary myocardial disorder with an autosomal pattern of inheritance, characterized by mostly asymmetric left ventricular hypertrophy with myocyte and myofibrillar disarray. To date, about 34 mutations of the beta-cardiac myosin heavy chain gene have been described and shown to have prognostic implications. The disease has an annual mortality rate of 3%, related to both sudden cardiac death and progressive systolic dysfunction. Not only diastolic but also progressive systolic dysfunction with cavity dilatation occurs in a minority of patients with severe hypertrophy during the long-term course. Sudden death often occurs in young, asymptomatic or mildly symptomatic patients. The degree of hypertrophy and the presence of a pressure gradient are of little prognostic significance. Nonsustained ventricular tachycardia is associated with a poor prognosis in the presence of a history of syncope.
mesh_terms: D000293:Adolescent; D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000235:genetics / Q000401:mortality / Q000503:physiopathology*; D002648:Child; D002883:Chromosomes, Human, Pair 14; D003645:Death, Sudden; D006439:Hemodynamics; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000503:physiopathology; D018995:Myosin Heavy Chains / Q000235:genetics; D017354:Point Mutation; D011379:Prognosis; D017180:Tachycardia, Ventricular / Q000503:physiopathology
chemical_list: D018995:Myosin Heavy Chains
vernacular_title: Hypertrophe Kardiomyopathie: Spontanverlauf.