Imaging studies demonstrate supravalvar AS in a patient with Williams syndrome. Left image: A transesophageal echocardiogram shows tubular narrowing and thickening of the aortic walls above the sinuses of Valsalva (arrow). Right image: Aortic root angiography shows a typical tubular deformity (arrow).
AS: aortic stenosis; LA: left atrium; LV: left ventricle.