Cardiac anomalies in Williams-Beuren syndrome

We have described some of the cardiological findings in 66 patients with Williams-Beuren syndrome and analysed the two dimensional cross sectional echocardiograms in 61 of them in comparison with normal controls. Supravalvar aortic narrowing was shown in all patients examined echocardiographically and may be a useful diagnostic sign. We documented a 7.8% incidence of systemic hypertension, a 15% clinical and echocardiographic incidence of mitral valve prolapse, and a 11.6% incidence of bicuspid aortic valve.