Isolated left ventricular apical hypoplasia, rare inborn cardiomyopathy

The review of data of literature concerning the description of clinical cases of isolated left ventricular apical hypoplasia (LVAH) in 42 patients are presented. left ventricular apical hypoplasia is characterized by 1) a spherical left ventricular with a cut-off tip and a swelling of the interventricular septum to the right in combination with systolic dysfunction of left ventricular; 2) replacement of the myocardium of the upper limb with fat tissue; 3) localization of papillary muscles on the flattened apex of the left ventricular; 4) elongated right ventricle (RV), flowing around the top of the upper left vertex. The course of this condition may be asymptomatic, or manifest as a clinic of biventricular heart failure with high pulmonary hypertension and heart rhythm disturbances.