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风向袋综合征:大动脉转位矫正型先天性心脏病中膜周部室间隔瘤导致的肺下梗阻

The windsock syndrome: subpulmonic obstruction by membranous ventricular septal aneurysm in congenitally corrected transposition of great arteries.

作者信息

Razzouk Louai, Applebaum Robert M, Okamura Charles, Saric Muhamed

机构信息

Leon H. Charney Division of Cardiology, New York University Langone Medical Center, New York, New York.

出版信息

Echocardiography. 2013 Sep;30(8):E243-8. doi: 10.1111/echo.12279. Epub 2013 Jul 1.

Abstract

Anomalies of the membranous portion of the interventricular septum include perimembranous ventricular septal defect and/or membranous septal aneurysm (MSA). In congenitally corrected transposition of the great arteries (L-TGA in sinus solitus), the combination of ventricular inversion and arterial transposition creates a unique anatomic substrate that fosters subpulmonic left ventricular outflow tract obstruction by an MSA. The combination of an L-TGA with subpulmonic obstruction by an MSA is referred to as the windsock syndrome. We report a case of windsock syndrome in a 25-year-old man which is to our knowledge the first three-dimensional echocardiographic description of this congenital entity.

摘要

室间隔膜部异常包括膜周部室间隔缺损和/或膜部间隔瘤(MSA)。在先天性矫正型大动脉转位(窦性心律下的L-TGA)中,心室反位和动脉转位的组合形成了一种独特的解剖结构,易导致由MSA引起的肺下左心室流出道梗阻。L-TGA与由MSA引起的肺下梗阻相结合被称为风袋综合征。我们报告了一例25岁男性的风袋综合征病例,据我们所知,这是该先天性疾病的首次三维超声心动图描述。

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