Repair of sinus of Valsalva aneurysm associated with aortoventricular discontinuity

The case of patient with unruptured sinus of Valsalva aneurysm with absence of the intervalvular lamina fibrosa is presented. The association of Valsalva aneurysm and aortic subvalvular defects has been explained as a deficiency of elastic tissue at the aortic root or maldevelopment of the fibrous trigone. Valvular aortic insufficiency with histological evidence of myxoid changes required prosthetic replacement. Myxomatous changes in the fibrous trigone and aortic annulus can explain this unusual, complex anomaly. Accurate preoperative diagnosis may require special views and simultaneous left and right injections at cineangiography. Techniques of repair that have been successful are reviewed, and the essential features of adequate correction are outlined.