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• W2410040363 abstract "Fourteen patients underwent operative repair of supravalvular aortic stenosis (SVAS) in this hospital during the past 17 years. There were seven female and seven male subjects. The patients ranged in age from 5 to 23 years at the time of the operation. Only two of the patients were asymptomatic. Twelve described exercise intolerance. Four of these 12 also reported syncope or angina. In all patients, a loud systolic murmur was heard over the right second intercostal space. In one patient, a systolic ejection click and a murmur of aortic regurgitation were noted. In another, a murmur of aortic regurgitation was heard. In three patients, blood pressure was higher on the right arm than the left by at least 20 mm. Hg. Mild cardiac enlargement was noted on the thoracic roentgenogram of eight patients, but in only one of them was pulmonary vascularity increased. Electrocardiograms revealed left ventricular hypertrophy in each patient, with T wave inversion in the left precordial leads in seven patients. There were two anatomic forms of SVAS; hourglass (nine cases), and tubular (five cases). The pressure gradient across the supravalvular area ranged from 45 to 125 mm. Hg with a mean gradient of 85 mm. Hg. In 10 patients, abnormalities of the coronary arteries or aortic valve or both were noted at the time of operation. In four patients, aortic valve commissurotomy, dilatation of the coronary ostia, excision of hypertrophic intima, and/or saphenous vein bypass were performed, in addition to repair of SVAS. In those nine patients with associated stenotic lesion of the aortic arch and descending aorta, and of those six patients with peripheral pulmonary vascular stenosis, no surgical repair was attempted. There were four deaths, two early and two late. All deaths occurred in patients with tubular hypoplasia. The survivors are asymptomatic. We have made the following conclusions: (I) Repair of SVAS secondary to tubular hypoplasia is less successful than repair of stenosis secondary to the discrete, hourglass type deformity; (2) the correction of associated aortic stenosis or reflux or both should be deferred until the results of the SVAS repair have been determined: and (3) ostial obstruction of the coronary arteries should be corrected." @default.
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• W2410040363 date "1978-01-01" @default.
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• W2410040363 title "The surgical management of vascular abnormalities associated with supravalvular aortic stenosis" @default.
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• W2410040363 doi "https://doi.org/10.1016/s0022-5223(19)41165-3" @default.
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