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Anesth Analg 2008; 107:1848-1854
© 2008 International Anesthesia Research Society
doi: 10.1213/ane.0b013e3181875a4d
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PEDIATRIC ANESTHESIOLOGY

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{dagger}, and James A. DiNardo, MD*

From the *Division of Cardiac Anaesthesia, Department of Anesthesiology, Perioperative and Pain Medicine, and {dagger}Department of Pediatrics, Children’s Hospital Boston and Harvard Medical School, Boston, Massachusetts.

Address correspondence and reprint requests to James A. DiNardo, MD, Department of Anesthesia, Children’s 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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Lippincott, Williams & Wilkins Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins with the assistance of Stanford University Libraries' HighWire Press®. Copyright 2006 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999 HighWire Press
Copyright © 2008 by the International Anesthesia Research Society.