• Genevieve E Staudt and Susan S Eagle.
• Division of Pediatric Cardiac Anesthesiology, Vanderbilt University Medical Center, Nashville, TN. Electronic address: genevieve.e.staudt@vumc.org.
• J. Cardiothorac. Vasc. Anesth. 2021 Jan 1; 35 (1): 176-186.

AbstractWilliams syndrome (WS) is a relatively rare congenital disorder which manifests across multiple organ systems with a wide spectrum of severity. Cardiovascular anomalies are the most common and concerning manifestations of WS, with supravalvar aortic stenosis present in up to 70% of patients with WS. Although a relatively rare disease, these patients frequently require sedation or anesthesia for a variety of medical procedures. The risk of sudden death in this population is 25 to 100 times that of the general population, with many documented deaths associated with sedation or anesthesia. This increased risk coupled with a disproportionately frequent need for anesthetic care renders it prudent for the anesthesiologist to have a firm understanding of the manifestations of WS. In the following review, the authors discuss pertinent clinical characteristics of WS along with particular anesthetic considerations for the anesthesiologist caring for patients with WS presenting for non-cardiac surgery.Copyright © 2020 Elsevier Inc. All rights reserved.

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