婴儿威廉姆斯综合征的冠状动脉受累情况及外科血运重建策略
Coronary Artery Involvement of Williams Syndrome in Infants and Surgical Revascularization Strategy.
作者信息
Federici Duccio, Ranghetti Arianna, Merlo Maurizio, Terzi Amedeo, Di Dedda Giovanni Battista, Marcora Simona, Marrone Chiara, Ciuffreda Matteo, Seddio Francesco, Galletti Lorenzo
机构信息
Cardiac Surgery Unit, "Papa Giovanni XXIII" Hospital, Bergamo, Italy.
Cardiovascular Department, "Papa Giovanni XXIII" Hospital, Bergamo, Italy.
出版信息
Ann Thorac Surg. 2016 Jan;101(1):359-61. doi: 10.1016/j.athoracsur.2015.03.064.
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.
威廉姆斯综合征（WS）是一种由于弹性蛋白基因表达缺陷导致的遗传性疾病。其特征为典型的躯体异常和多种心血管畸形。冠状动脉受累是该综合征的常见表现，尤其是在那些患有严重主动脉瓣上狭窄的患者中。我们报告一例11个月大患威廉姆斯综合征的婴儿，其在主动脉瓣上狭窄手术修复2个月后出现严重冠状动脉疾病。文中还描述了临床表现及成功的手术血运重建策略。
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