Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review
Andrew J. Matisoff, Laura Olivieri, Jamie M. Schwartz & Nina Deutsch
Since the first description in 1961, several case reports have documented an increased incidence of anesthesia-related cardiac arrest in patients with Williams–Beuren syndrome, commonly known as Williams syndrome (WS). Widespread arteriopathy secondary to an elastin gene defect results in various cardiac defects, including supravalvar aortic stenosis (SVAS) and coronary artery anomalies, which can increase the risk of myocardial ischemia.