title: Congenital supravalvular aortic stenosis and sudden death associated with anesthesia: what's the mystery?
abstract: 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. The biventricular hypertrophy that accompanies these lesions increases myocardial oxygen consumption and compromises oxygen delivery. In addition, these patients often have direct, multifactorial compromise of coronary blood flow. In this article, we review both the pathophysiology of congenital supravalvular aortic stenosis and the literature regarding sudden death in association with sedation and anesthesia. Recommendations as to preoperative assessment and management of these patients are made based on the best available evidence.
authors.affiliation: Division of Cardiac Anaesthesia, Department of Anesthesiology, Perioperative and Pain Medicine, Children's Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA.
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Burch
authors.forename: Francis X
authors.initials: FX
authors.lastname: McGowan
authors.forename: Barry D
authors.initials: BD
authors.lastname: Kussman
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Powell
authors.forename: James A
authors.initials: JA
authors.lastname: DiNardo
mesh_terms: D000758:Anesthesia / Q000009:adverse effects*; D021921:Aortic Stenosis, Supravalvular / Q000150:complications / Q000151:congenital* / Q000503:physiopathology; D003326:Coronary Circulation; D023921:Coronary Stenosis / Q000209:etiology; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D006801:Humans; D017202:Myocardial Ischemia / Q000209:etiology; D018980:Williams Syndrome / Q000503:physiopathology