Abstract
Noncompaction of the ventricular myocardium is a genetic cardiomyopathy due to an alteration in endomyocardial morphogenesis. When present, it is commonly associated with other congenital cardiac diseases, whereas it is rare as an isolated finding without other associated cardiac structural alterations. Since its first report in 1990, the noncompaction of the ventricular myocardium has been widely studied in order to establish its genetic character, natural history, diagnostic methods, hemodynamic repercussions and prognosis. This study aims to report the case of an isolated noncompaction of the left ventricular myocardium with autopsy.
Highlights
Noncompaction of the ventricular myocardium is a genetic cardiomyopathy due to an alteration in endomyocardial morphogenesis. It is commonly associated with other congenital cardiac diseases, whereas it is rare as an isolated finding without other associated cardiac structural alterations
Since its first report in 1990, the noncompaction of the ventricular myocardium has been widely studied in order to establish its genetic character, natural history, diagnostic methods, hemodynamic repercussions and prognosis
This study aims to report the case of an isolated noncompaction of the left ventricular myocardium with autopsy
Summary
O miocárdio não compactado de ventrículo esquerdo é cardiomiopatia genética em decorrência de uma anormalidade da morfogênese endomiocárdica. Desde seu primeiro relato em 1990, a não compactação do miocárdio vem sendo amplamente estudada a fim de se estabelecerem caráter genético, história natural, métodos de diagnóstico, repercussões hemodinâmicas e prognóstico. O objetivo deste estudo foi relatar um caso de miocárdio não compactado isolado de ventrículo esquerdo com estudo de necrópsia. Noncompaction of the ventricular myocardium is a genetic cardiomyopathy due to an alteration in endomyocardial morphogenesis. When present, it is commonly associated with other congenital cardiac diseases, whereas it is rare as an isolated finding without other associated cardiac structural alterations. This study aims to report the case of an isolated noncompaction of the left ventricular myocardium with autopsy
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