Aorto-left ventricular tunnel with origin in the left sinus of Valsalva: a rare cause of congenital aortic insufficiency

Aortico-left-ventricular tunnel is a rare congenital cardiac lesion that often results in severe aortic insufficiency in infancy. Normally, the defect appears immediately above the right coronary sinus of Valsalva and consists of a tunnel-like connection between the aortic root and the left ventricle. The differential diagnosis to coronary artery fistulae and ruptured aneurysms of the sinus of Valsalva is often difficult. We describe a patient with an aortico-left-ventricular tunnel originating from the left aortic sinus. Similar findings have been described in only three case reports. Additionally, in our case there was a stenosis in the midportion of the tunnel which was connected to an intraseptal aneurysm communicating with the left ventricular chamber via a "septal" defect. The infant underwent successful surgical correction of this malformation at 1 year and 5 months of age.