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先天性矫正性大动脉转位中的动态流出道梗阻。

Dynamic outflow tract obstruction in congenitally corrected transposition of the great arteries.

机构信息

Heart & Vascular Institute, Department of Cardiovascular Medicine, Cleveland Clinic, Desk J1-5, Cleveland, OH 44195, USA.

出版信息

Int J Cardiovasc Imaging. 2010 Aug;26(6):617-9. doi: 10.1007/s10554-010-9588-1. Epub 2010 Feb 27.

Abstract

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease, constituting 0.5% of all congenital heart defects. The incidence of left ventricle (non-systemic ventricle) outflow tract obstruction ranges between 44 and 57%. Herein, we present the case of a 45 year old woman with CCTGA with progressively worsening dyspnea who had been referred for surgical correction of severe systemic ventricle (morphologic right ventricle) atrio-ventricular valve (tricuspid valve) regurgitation. Cardiac magnetic resonance imaging (CMR) and transesophageal imaging (TEE) demonstrated severe systemic ventricle (morphologic right ventricle) contractile dysfunction, as well as dynamic non-systemic ventricle (morphologic left ventricle) outflow tract obstruction due to systolic anterior motion (SAM) of the non-systemic ventricle (morphologic left ventricle) atrio-ventricular valve (mitral valve) with a large membranous ventricular septal aneurysm that protrudes into the outflow tract of the non-systemic ventricle (morphologic left ventricle). Ultimately, our patient was felt to be too high-risk for surgical correction and a course of medical therapy has been pursued.

摘要

先天性矫正性大动脉转位(CCTGA)是一种罕见的先天性心脏病,占所有先天性心脏病的 0.5%。左心室(非系统性心室)流出道梗阻的发生率在 44%至 57%之间。在此,我们报告了 1 例 45 岁女性 CCTGA 病例,该患者进行性呼吸困难,因严重系统性心室(形态右心室)三尖瓣反流而行手术矫正。心脏磁共振成像(CMR)和经食管成像(TEE)显示严重的系统性心室(形态右心室)收缩功能障碍,以及由于非系统性心室(形态左心室)二尖瓣收缩期前向运动(SAM)导致的动态非系统性心室(形态左心室)流出道梗阻,伴有大的膜性室间隔瘤突入非系统性心室(形态左心室)流出道。最终,我们认为该患者手术矫正风险过高,因此采用了药物治疗。

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