Familial supravalvular aortic stenosis: a report of two brothers

We studied two brothers in a family with isolated congenital supravalvular aortic stenosis without Williams syndrome. Clinical, echocardiographic, hemodynamic and angiographic features of our cases were described. A severe long, narrowed supravalvular aortic stenosis involving the ascending aorta with proximal narrowing of the aortic branches and mild aortic insufficiency were found. To the best of our knowledge, this is the first report of this combination of lesions in the Orient.