Abstract

ESTIGARRIBIA PASSARO, Jorge; VIDAL CORTINAS, Inés; BAEZ, Álvaro  and  LUIS VIDAL, Juan. Miocardiopatía hipertrófica: Aspectos conceptuales de la enfermedad y fundamentos del Registro Uruguayo de Miocardiopatía Hipertrófica. Rev.Urug.Cardiol. [online]. 2011, vol.26, n.1, pp.27-37. ISSN 1688-0420.

SUMMARY Hypertrophic cardiomyopathy is a primary cardiomyopathy, inherited as an autosomal dominant threat, with prevalence in the general adult population of 1 in 500, primarily characterized by parietal hypertrophy of the left ventricle, in the absence of a cardiac or systemic disease that could explain it. Despite not being uncommon, it is an underdiagnosed disease and its clinical management is little systematized in practice. Echocardiography has the key to the diagnosis, and magnetic resonance imaging has made recent contributions in the definition of its morphological varieties. It has a histological pattern of architectural disarray of myocardial fibers, which are hypertrophic and poorly aligned. Hypertrophy is often asymmetric and can generate a dynamic gradient by obstruction of the outflow tract of the left ventricle which involved an anomalous motion of the mitral valve (systolic anterior movement, SAM), which usually also determines a valvular insufficiency. The most common symptoms are breathlessness, chest pain, syncope and sudden death (SD). It is the first cause of SD in young people and athletes in competition, and a capital aspect of its clinical management is the stratification of the risk of this tragic complication to decide to implant an automatic cardioverter-defibrillator timely. Through Sociedad Uruguaya de Cardiología, a Registry of carriers of this disease has been implemented (RUMHI) which aims to improve the knowledge of the entity, obtain an approximation to the frequency and know its evolution, prognosis and treatment in Uruguay.

Keywords : CARDIOMIOPATHY, HYPERTROPHIC.

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