Surgical Correction of Congenital Supravalvular Aortic Stenosis

Supravalvular aortic stenosis is a rare congenital cardiac anomaly involving stenosis of the sinotubular junction that can extend to the ascending aorta and the aortic arch. It is an elastin arteriopathy that is classically associated with Williams syndrome. Many surgical techniques have been developed over time, most commonly involving pericardial patches to relieve the stenotic area. The 1-patch repair techniques (McGoon and Doty repairs) have low mortality but do not restore the normal morphology of the aortic root. Herein we describe the extended 3-patch technique that can symmetrically relieve supravalvular aortic stenosis.