Multiple stenotic arteriopathy in a 72-year-old female with Williams syndrome

摘要

In 1961 and 1962, Williams et al and Beuren et al reported a combination of cardiovascular lesions including supravalvular aortic stenosis (SVAS) and pulmonary artery stenosis (PAS) as features of mental and growth retardation in infants or children (Williams—Beuren syndrome (WBS) or Williams syndrome (WS)).~(1 2) Adult WS has subsequently become well known, but there is still little information on the long-term course. More than half of adults with WS are reported to be obese and 63% over the age of 18 years are hypertensive. Sudden death related to coronary sclerosis with ventricular outflow obstruction has been reported in children3 but not in adults.