Congenital Supravalvular Aortic Stenosis and Sudden Death Associated with Anesthesia: What's the Mystery?

Thomas M. Burch, MD Francis X. McGowan, Jr., MD, Barry D. Kussman, MBBCh, Andrew J. Powell, MD, James A. DiNardo, MD. 2008

Patients with congenital supravalvular aortic stenosis and associated peripheral pulmonary artery stenoses, the majority of whom have Williams-Beuren syndrome, are inherently at risk for development of myocardial ischemia. This is particularly true in the setting of procedural sedation and anesthesia.