Associated Subvalvular and Supravalvular Aortic Stenosis with Aortic Valve Anomaly

Abstract

The patient, an asymptomatic 1 1%-year-old boy, was admitted to the hospital on March 3, 1965. A 2-year-old sibling had died following an operation for pulmonary valvular stenosis at another institution. The patient had been thought normal at birth, but a heart murmur had been found when he was 6 months old. A loud holosystolic murmur was widely heard over the precordium. A mild aortic diastolic murmur also was present. The blood pressure was 128/80 in the right arm and approximately the same in the left. The electrocardiogram showed evidence of marked left ventricular hypertrophy. On fluoroscopic and radiographic study there was evidence suggestive of left ventricular enlargement. The aorta seemed normal in size. Cardiac catheterization and angiographic study showed severe discrete subvalvular stenosis, mild supravalvular aortic stenosis, and a deformity of the aortic valve permitting mild regurgitation. In addition the inferior vena cava was observed to be completely obstructed cephalad to the point of reception of the renal veins. Large collateral vessels drained into the azygos system. Simultaneously measured pressures were 263/ 14 in the left ventricle and 107/70 in the aorta distal to the area of supravalvular constriction. The pressure in the small ventricular segment between the subvalvular diaphragm and the aortic annulus was 122124. An operation was performed on March 10 with the aid of total cardiopulmonary bypass. There was evidence of left ventricular hypertrophy. The aorta was moderately dilated above the area of supravalvular stenosis, as were the coronary sinuses below. The coronary arteries were not enlarged. When the aorta was opened, the preoperative findings were confirmed. There was a mild discrete supravalvular stenosis, the aortic diameter in this area being approximately 15 mm. (Figure). The left coronary aortic cusp was very small, with a presenting edge no longer than 8 mm. and a rather shallow sinus. The right coronary aortic cusp was larger, the noncoronary cusp still larger, and both had