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[特纳综合征框架下右心室发育不全及因左右心房分流导致的发绀。经球囊导管封堵试验后手术闭合房间隔连接]

[Hypoplasia of the right ventricle and cyanosis due to left-right atrial shunt within the framework of Turner's syndrome. Surgical closing of the interatrial connection after occlusion testing with balloon catheterization].

作者信息

Landau J F, Durand M, Vincent P, Guilmet D, Delamare J

出版信息

Arch Mal Coeur Vaiss. 1983 Feb;76(2):233-7.

PMID:6407431
Abstract

The authors describe the unusual cardiac malformation in a young girl with Turner's syndrome: right ventricular hypoplasia and atrial septal defect, resulting in central cyanosis. A trial of temporary occlusion of the atrial septal defect at cardiac catheterisation before surgical closure suppressed the right-to-left shunt and the cyanosis without significantly changing the hemodynamic parameters. The authors review the types of cardiovascular abnormalities associated with Turner's syndrome and emphasize the value of a temporary occlusion test of defects in certain cyanotic cardiac malformations with atrial right-to-left shunts; it may lead to a relatively simple surgical procedure, as in the case described.

摘要

作者描述了一名患有特纳综合征的年轻女孩身上不寻常的心脏畸形

右心室发育不全和房间隔缺损,导致中心性发绀。在手术闭合之前,通过心导管插入术对房间隔缺损进行临时封堵试验,抑制了右向左分流和发绀,且未显著改变血流动力学参数。作者回顾了与特纳综合征相关的心血管异常类型,并强调了对某些伴有右向左分流的青紫型心脏畸形进行缺损临时封堵试验的价值;这可能会带来相对简单的手术过程,就像所描述的病例一样。

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