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From the *Division of Cardiac Anaesthesia, Department of Anesthesiology, Perioperative and Pain Medicine, and
Department of Pediatrics, Childrens Hospital Boston and Harvard Medical School, Boston, Massachusetts.
Address correspondence and reprint requests to James A. DiNardo, MD, Department of Anesthesia, Childrens Hospital Boston, 300 Longwood Ave., Boston, MA 02115. Address e-mail to james.dinardo{at}childrens.harvard.edu.
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.
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