Williams–Beuren syndrome, a multisystem disorder caused by the deletion of a
chromosome region of 1.5 million to 1.8 million base pairs containing 26 to 28 genes, is a …

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 …

Aims Understanding the anatomy of the aortic root is particularly relevant in the current era
of evolving management strategies including percutaneous and transcatheter therapeutic …

Congenital coronary artery anomalies, both in isolation and associated with other forms of
congenital heart disease, have been recognized as important lesions with significant …

Elastin is synthesized and secreted by vascular smooth muscle cells and is the major
extracellular matrix component deposited in the arterial wall. When last reviewed by this …

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

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

Current or recent low vitamin D status (or proxy measures such as dietary intake or ambient
ultraviolet radiation) is linked to several chronic diseases, including osteoporosis, cancers …

The population of neonates and children with congenital heart defects presents about a 30%
prevalence of associated genetic syndrome or additional extracardiac anomalies and may …

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 …