Double outlet right ventricle with left ventricular outflow tract obstruction due to small ventricular septal defect

This is a report of the seventh and eighth known cases of double outlet right ventricle with left ventricular outflow tract stenosis due to a small ventricular septal defect. Case 1 is the second patient with this uncommon anomaly who has undergone successful surgical correction by enlargement of the ventricular septal defect and reconstruction of the outflow tract from the left ventricle to the aorta by means of a teflon tunnel. Case 2 was studied at autopsy and illustrates well the anatomic features of this malformation. It is suggested that this type with left ventricular outflow tract stenosis be incorporated into current classifications of double outlet right ventricle.