Surgical treatment of supravalvular aortic stenosis: Report of 16 cases and review of literature

Abstract

Summary The surgical experience with 16 patients with congenital supravalvular aortic stenosis has been reviewed, as was the literature. Fourteen patients had localized stenosis. In this group of cases, there were no deaths, and 13 patients had a good result. Two patients had diffuse supravalvular aortic stenosis. One died after attempted repair; and, in the case of the other, the gradient was not diminished after repair. Perhaps operation should be avoided when the supravalvular stenosis is diffuse. Pathologically, two basic forms of supravalvular aortic stenosis occur (localized and diffuse), thickening of the intima and hypertrophy of the media are evident in the stenotic region, and abnormalities of the aortic cusps are present in at least one third of the cases. Clinically, angina and syncope are the commonest symptoms and the systolic blood pressure is often higher in the right arm than in the left. Surgically, partial resection of the stenotic ridge and application of a patch graft is generally the best technique. We would like to thank Drs. Owings W. Kincaid and George D. Davis, of the Section of Roentgenology, for their help. Also, we would like to thank Dr. Regina Gluck of Old Westbury, New York, for supplying us with the postoperative catheterization data for Case 9.