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.




D., F., A., R., M., M., A., T., G.B., D. D., S., M., … L., G. (2016). Coronary artery involvement of williams syndrome in infants and surgical revascularization strategy. Annals of Thoracic Surgery, 101(1), 359–361. https://doi.org/10.1016/j.athoracsur.2015.03.064 LK  - http://lh.cineca.it/Ccube/openclink.do?sid=EMBASE&sid=EMBASE&issn=15526259&id=doi:10.1016%2Fj.athoracsur.2015.03.064&atitle=Coronary+artery+involvement+of+williams+syndrome+in+infants+and+surgical+revascularization+strategy&stitle=Ann.+Thorac.+Surg.&title=Annals+of+Thoracic+Surgery&volume=101&issue=1&spage=359&epage=361&aulast=Federici&aufirst=Duccio&auinit=D.&aufull=Federici+D.&coden=ATHSA&isbn=&pages=359-361&date=2016&auinit1=D&auinitm=

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