Supravalvar Aortic Stenosis

Christina Foster, RDCS (AE, PE)

Supravalvar aortic stenosis involves obstruction above the aortic valve, most commonly at the sino-tubular junction.  This type of stenosis is characterized by a discreet narrowing of the aortic lumen. In addition, there could be narrowing of the ascending aorta and/or aortic arch. Supravalvar aortic stenosis is more commonly a progressive disease that becomes more severe with age and therefore needs continual cardiology follow-up. It is commonly seen in patients with Williams Syndrome. Williams Syndrome is an autosomal dominant genetic disorder associated with vascular abnormalities that include thickening of the vascular media layer in medium and large sized arteries.

Echocardiography in Pediatric and Congenital Heart Disease, Wyman Lai
Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome,