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威廉姆斯综合征与先天性单心室的新生儿心脏手术

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle.

作者信息

Katt Taylor E, Spicer Robert L, Yetman Anji T, Ibrahimiye Ali N, Hammel James M, Robinson Jeffrey A

机构信息

College of Medicine, University of Nebraska Medical Center, Omaha, Nebraska.

Dr. C.C. and Mabel L. Criss Heart Center, Children's Hospital and Medical Center, Omaha, Nebraska.

出版信息

JACC Case Rep. 2020 Aug 12;2(11):1716-1719. doi: 10.1016/j.jaccas.2020.05.098. eCollection 2020 Sep.

Abstract

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. ().

摘要

威廉姆斯综合征(WS)是一种与主动脉瓣上狭窄和分支肺动脉狭窄相关的动脉病变紊乱。我们描述了一名患有WS的婴儿出现右心室双出口合并二尖瓣闭锁及主动脉弓发育不全的情况。该病例显示了管理患有复杂心脏缺陷的WS患者的困难。据我们所知,这是首次报道的患有WS的患者出现单心室生理情况。()

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dc49/8312043/4e1052ef9b4c/fx1.jpg

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