Biventricular isolated apical hypoplasia: First-time description of a new cardiac abnormality by multimodality imaging

Abstract

Left ventricular isolated hypoplasia is a seldom-described cardiac abnormality. Right ventricular hypoplasia is usually associated with congenital anomalies of the pulmonary or the tricuspid valve, whereas biventricular isolated apical hypoplasia has never been described. We report the case of a 48-year-old man with no history of known cardiac disease who was found to have a complex cardiac abnormality characterized by: 1) Deficiency of the myocardium within the biventricular apex with adipose tissue infiltration; 2) Truncated right ventricle because of an absent trabecular portion of the inflow tract; 3) Truncated and spherical left ventricular apex; 4) Origin of the mitral papillary muscle in the flattened anterior left ventricular apex. Multimodality imaging was performed to delineate the morphological and functional characteristics of this cardiomyopathy fully. To the best of our knowledge, this is the first description of a new cardiac abnormality characterized by the hypoplasia of the apical region of both ventricles in the absence of valvular or coronary artery disease.