Echocardiographic features of supracristal ventricular septal defect with prolapsed aortic valve leaflet

Anatomically diagnostic echocardiographic features of a supracristal ventricular septal defect with prolapsed right coronary aortic leaflet are described in four children aged 2 to 10 years. Both single crystal M mode as well as 80 ° phased array sector scan techniques were used. The echographic features in the M mode scan from the aorta to the left ventricle in three of four patients included (1) the position of the ventricular septal defect as a clear space between the interrupted septal echoes below the aortic root, and (2) the prolapsed right coronary aortic leaflet as anomalous linear echoes in the right ventricular outflow tract. Angiographic, intraoperative and echocardiographic contrast studies were used to establish the diagnosis. On sector scanning using the long axis view, the supracristal ventricular septal defect was recognized as a clear space between the top of the ventricular septum and the anterior segment of the aortic root in three of four patients. The right coronary aortic leaflet was seen to prolapse into the right ventricular outflow tract through this defect, and its motion could be clearly followed during systole and diastole. It is concluded that echocardiography provides anatomic diagnosis of this lesion. Furthermore, the severity and progression of this lesion can be assessed by quantitation of the left ventricular size and performance.