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

Federici, Duccio; Ranghetti, Arianna; Merlo, Maurizio; Terzi, Amedeo; Di Dedda, Giovanni Battista; Marcora, Simona; Marrone, Chiara; Ciuffreda, Matteo et al. · Ann Thorac Surg · 2016

case_report · Level V

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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.

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