Coronary Artery Involvement of Williams Syndrome in Infants and Surgical Revascularization Strategy.

Abstract

Williams syndrome (WS) is a genetic disorder due to deficiency of elastin gene expression. It is characterized by typical somatic abnormalities and a wide range of cardiovascular malformations. Coronary artery involvement is a frequent finding of the syndrome, particularly in those patients with severe supravalvular aortic stenosis. We present the case of an 11-month-old infant affected by WS who developed severe coronary artery disease 2 months after the surgical repair of supravalvular aortic stenosis. The clinical picture and successful surgical revascularization strategy is also described.

• Authors: Duccio Federici, Arianna Ranghetti, Maurizio Merlo, Amedeo Terzi, Giovanni Battista Di Dedda, Simona Marcora, Chiara Marrone, Matteo Ciuffreda, Francesco Seddio, Lorenzo Galletti
• Journal: The Annals of thoracic surgery (Ann Thorac Surg) Vol. 101 Issue 1 Pg. 359-61 (Jan 2016) ISSN: 1552-6259 [Electronic] Netherlands
• PMID: 26694280 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Topics

• Coronary Artery Bypass (methods)
• Coronary Stenosis (diagnosis, etiology, surgery)
• Coronary Vessels (surgery)
• Echocardiography
• Electrocardiography
• Humans
• Infant
• Male
• Tomography, X-Ray Computed
• Williams Syndrome (complications, surgery)