SUPRAVALVAR AORTIC STENOSIS

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Abstract
The roentgen experience with 27 patients with supravalvar aortic stenosis (SVAS) who had both cardiac catheterization and cinecardioangiography is presented. Gross and microscopic pathologic features of SVAS in previously reported cases are reviewed. Observations at the time of surgery in 11 cases of this series are detailed. A classification of SVAS and postvalvar pulmonary artery stenosis (PPAS) is suggested based on the cardioangiographic findings in these 27 cases. One form of SVAS (Type II), heretofore not emphasized, is a complex anomaly with mild to moderate supravalvar narrowing just above shallow sinuses of Valsalva, small aortic valve annulus, and thick semirigid valve cusps with unfused commissures. Conditions with which SVAS is associated are briefly discussed. These conditions include: the syndrome of SVAS, mental retardation and peculiar facies, idiopathic infantile hypercalcemia, familial SVAS with normal intellect, sporadic SVAS with normal intellect, and Marfan's syndrome. The basic clinical and hemodynamic characteristics of SVAS are described and a table is presented summarizing plain film, clinical, cardioangiographic and cardiac catheterization data in every case in this series.