Dextrocardia com situs inversus associada à miocardiopatia não compactada

Abstract

Dextrocardia is a rare condition usually diagnosed incidentally and associated with other congenital anomalies. It is characterized by the position of the heart in the right hemithorax, with its base-apex axis directed to the bottom right. Its incidence associated with situs inversus, in the general population, is 1:10.0001 . Non-compaction cardiomyopathy (non-compaction CMP), in turn, has an incidence ranging from 0.014 to 1.3%, may occur alone or associated with other congenital defects and results from failure in the process of compaction of myocardial fibers, resulting in the persistence of trabeculations and deep recesses that communicate with the ventricular cavity. A rare association of these two diseases is described. Cases similar to the one reported have not been found.