Abstract
THE ANESTHESIA-RELATED deaths of 2 patients with Williams syndrome and supravalvular aortic stenosis (SVAS) are reported. There are 3 reports in the anesthesia literature of favorable outcomes of anesthetic management of patients with Williams syndrome.1-3 Other reports in the pediatric literature documented severe coronary artery disease, myocardial infarction,4,5 and anesthesia-related cardiac arrest and mortality6,7 in children with Williams syndrome. A review of the surgical literature described a significant incidence of coronary artery involvement in patients with SVAS.8 None of these reports described the anesthetic management of these patients. This case report highlights the potential risks of providing sedation or general anesthesia for patients with Williams syndrome, SVAS, and coronary artery disease.
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