Aortic Dissection and Supravalvular Aortic Stenosis With Williams Syndrome Complicated by Infection

Williams syndrome (WS) is a contiguous gene syndrome involving the connective tissue, central nervous system, and cardiovascular disorders, such as supravalvular aortic stenosis (SVAS), pulmonary artery stenosis, and coronary artery anomaly, that carry a risk of sudden death. Although aortic dissection with SVAS in WS is extremely rare, it could be 1 of the triggers of sudden cardiac death. In this case, a patient with WS underwent ascending aorta replacement for aortic dissection that was potentially influenced by infective endocarditis and SVAS repaired by modified Brom aortoplasty.