Four patients with supravalvular aortic stenosis are reported. In three of these patients a certain facial resemblance was striking, and these three patients were also mentally and physically retarded. The cases are very similar to the four cases recently reported in the literature. Supravalvular aortic stenosis in association with mental retardation and a certain facial appearance may constitute a previously unrecognized clinical syndrome. A systolic thrill and murmur most pronounced in the first right intercostal space and just below the clavicle, with an empty vascular pedicle on the roentgenogram, may be a clue to the clinical diagnosis of supravalvular aortic stenosis. Supravalvular aortic stenosis is best demonstrated by left ventriculography. A collateral circulation between the coronary arteries and the internal mammary artery has been shown in one patient.

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This article describes the case of a boy, 1 year and 5 months old, with generalized tortuosity and lengthening of all major arteries, including the coronary arteries and the pulmonary artery. The early death of the patient was attributed to coronary insufficiency and multiple severe peripheral pulmonary stenoses. The pathological changes were confined to the elastic arteries and the first part of the muscular arteries. The wall of the aorta was thickened and there was an increase of the elastic fibers. The same changes were present in the main pulmonary artery. In the large muscular arteries, the characteristic changes were thickening of the intima with hyperplasia of the elastic fibers and degenerative fragmentation of the internal elastic membrane. The walls of the coronary arteries were thickened and their lumina were narrow.

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A new therapeutic concept of enlarging the outflow tracts of both ventricles with a patch and inserting an aortic prosthesis has been developed for the treatment of tunnel subaortic stenosis. This operation has been applied clinically since June 1974 on several types of obstruction in the outflow tract of the left ventricle. Twenty-one operations have been performed on 20 patients under the age of 18 years, with an overall mortality of 24% and no late deaths. Seven patients developed complete right bundle branch block or left anterior hemiblock or both as a result of this operation; transient atrioventricular block and complete left bundle branch block occurred in one patient each. In no case, however, did rhythm disturbances contribute to death. In one patient, the septal incision injured a septal coronary artery, with fatal result. Fourteen patients had catheterization studies postoperatively. Although previous conventional surgery had been unsuccessful, aortoventriculoplasty (AoVPI) reduced the mean gradient across the left ventricular outflow tract significantly (p less than or equal to 0.01), from 94.7 +/- 25.5 mm Hg to 14.4 +/- 17.2 mm Hg, leaving the end-diastolic pressure practically unchanged. No significant defect remained in the patch-covered septal incision. Thus, we consider AoVPI to be the operation of choice for tunnel subaortic stenosis, for valvular aortic stenosis with a narrow annulus and in cases where an artificial aortic valve has become too small because of the patient's growth.