Supravalvular aortic stenosis. Repair by extended aortoplasty.

Enlargement of the aorta with a diamond-shaped patch of the noncoronary sinus of Valsalva may not be sufficient in severe cases of supravalvular aortic stenosis. This traditional reconstruction is asymmetric, and, if the fibrous supravalvular ring is thick and rigid, the aorta may not open wide with patch angioplasty, so that aortic obstruction may remain. Also, because aortic valve function may not be perfect after asymmetric reconstruction, there may be aortic valve incompetence or obstruction of coronary ostia by the valve cusps. A new reconstructive operation was designed and used in eight patients. All survived and are asymptomatic. The aortoplasty was extended so that the supravalvular ring was incised at two points in the noncoronary and in the right coronary sinuses of Valsalva. The area of stenosis was opened wide, and the cusps of the aortic valve were lengthened, which provided better approximation and function. A tubular Dacron prosthesis, tailored to reconstruct the aorta, provided a wide aortic cross-sectional area. This technique of extended aortoplasty for symmetric reconstruction of the aorta should provide more predictable relief of aortic obstruction and improved function of the aortic valve.

Pulmonary stenosis in infants and young children.

Twenty-four patients less than 3 years old underwent operation for pulmonary stenosis. Pulmonary dysplasia was diagnosed preoperatively in only 4 patients; in 20 patients the lesion was categorized simply as pulmonary stenosis. At operation, more severe valve deformities were often present in patients less than 2 years of age. Preoperative evaluation did not reveal the extent of the deformity in 7 additional patients. The deformities included not only valvular dysplasia (thickened redundant valve cusps) but also supravalvular and annular abnormalities. Relief of obstruction was obtained only when all components of the obstructive abnormality were relieved. Patch angioplasty of the right ventricular outflow tract was necessary in 13 patients with complex morphology. Valvotomy was effective only for pulmonary stenosis due to pure commissural fusion. A spectrum of the morphology of pulmonary stenosis is recognized, with more complex lesions than simple commissural fusion identified in younger children. The more complex lesions may require more extensive operations (outflow tract patch) to completely relieve the obstructive pathological condition in the outflow tract.