2126 Circulation May 28, 2013 alternates with a ring of smooth muscle, forming a lamellar
unit. 20 The elastic lamellae allow an artery to respond to the increased hemodynamic …

Williams syndrome has been variably termed Williams–Beuren syndrome, idiopathic
hypercalcemia, and supravalvar aortic stenosis syndrome. It is estimated to occur in …

Braunwald's Heart Disease remains your indispensable source for definitive, state-of-the-art
answers on every aspect of contemporary cardiology. Edited by Drs. Robert O. Bonow …

The management of congenital heart disease has changed dramatically over the last 15
years and continues to evolve at a rapid pace. More than ever before contemporary practice …

Williams-Beuren syndrome (WBS) is a microdeletion disorder caused by heterozygous loss
of approximately 1.5-Mb pairs of DNA from chromosome 7. Patients with WBS have a …

The bestselling guide to the medical management of common genetic syndromes—now fully
revised and expanded A review in the American Journal of Medical Genetics heralded the …

IMPLICATIONS: Patients with congenital supravalvular aortic stenosis and associated
peripheral pulmonary artery stenoses are inherently at risk for development of myocardial …

Supravalvular aortic stenosis is a systemic elastin (ELN) arteriopathy that disproportionately
affects the supravalvular aorta. ELN arteriopathy may be present in a nonsyndromic …

The underlying cause of congenital supravalvular aortic stenosis (SVAS) has recently been
identified as a loss-of function mutation of the elastin gene on chromosome 7q11. 23 …

Aims: We investigated the incidence and outcome of cardiac malformations in 53 patients
with Williams syndrome. Methods and results: The mean age, and period of follow-up, were …