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膜周部室间隔瘤:内脏异位相关的心室间隔对位不良伴主动脉二尖瓣不连续(右心室双出口)导致右心室流出道梗阻的罕见原因。

Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy.

作者信息

Bijulal Sasidharan, Sivasankaran Sivasubramanian, Sanjay Ganapathy, Tharakan Jaganmohan

机构信息

Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.

出版信息

Pediatr Cardiol. 2009 Feb;30(2):200-2. doi: 10.1007/s00246-008-9299-8. Epub 2008 Aug 15.

DOI:10.1007/s00246-008-9299-8
PMID:18704548
Abstract

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.

摘要

在膜周部室间隔缺损的自然病程中,间隔动脉瘤的形成通常会使缺损在血流动力学上的意义减小。本报告描述了一例患有前位对位不良型室间隔缺损合并主动脉二尖瓣连续性中断(右心室双出口)的患者,其膜周间隔动脉瘤导致严重的右心室流出道梗阻。除了间隔动脉瘤造成的动态梗阻外,该患者并无肺动脉狭窄。在该患者中,间隔动脉瘤产生了有利和不利的血流动力学效应。室间隔缺损尺寸减小产生了有利影响,而右心室流出道梗阻则导致了右心室高压和肥厚的不利情况。与前位对位不良的间隔相关的、已受损的右心室流出道存在大的间隔动脉瘤,导致了严重梗阻。

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