Isolated Left Ventricular Apical Hypoplasia

Isolated left ventricular (LV) apical hypoplasia is a recently described congenital abnormality characterized by: (1) a truncated and spherical LV configuration with rightward bulging of the interventricular septum, (2) deficiency of the myocardium within the LV apex with adipose tissue infiltrating the apex, (3) origin of the papillary muscle in the flattened anterior apex, and (4) elongation of the right ventricle wrapping around the deficient LV apex. In this report, we demonstrate these characteristic features with cardiac magnetic resonance imaging and summarize the existing information on isolated LV apical hypoplasia.