a type of restrictive cardiomyopathy or obliterative cardiomyopathy occurring in equatorial Africa and less often in other tropical and subtropical regions of the world. It is characterized by severe endocardial disposition of fibrous tissue in the apical and subvalvular regions of either or both ventricles. It causes restriction of blood flow into the ventricles causing restrictive physiology. Fibrosis of the atrioventricular valves causes regurgitation. Pericardial effusion is frequently present.Chest X-ray demonstrates pulmonary venous hypertension and a variable degree of cardiac enlargement. In left-sided involvement the left atrium is enlarged while in the right-sided form the right atrium is enlarged. A characteristic feature is intracardiac calcification usually near the cardiac apex. Echocardiography reveals obliteration of the apex of either or both ventricles, strongly echogenic endocardial or subendocardial regions, and atrioventricular valvular regurgitation. Ventriculography shows obliteration of the apical region of one or both ventricles and filling defects caused by the frequently associated ventricular thrombi. MRI has also depicted the obliteration of the apical region(s), thickening of the wall in the inlet regions of the ventricles and alteration of signal intensity of the subendocardial deposition compared with normal myocardium.
