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与梗阻性完全性肺静脉异位引流相关的右心房异构的混合姑息治疗。

Hybrid palliation for right atrial isomerism associated with obstructive total anomalous pulmonary venous drainage.

作者信息

Jhang Won-Kyoung, Chang Yong-Jin, Park Chun-Soo, Oh Yeon-Mi, Kim Young-Hwue, Yun Tae-Jin

机构信息

Division of Pediatric Cardiac Surgery, Asan Medical Center, College of Medicine, University of Ulsan, 388-1 Poongnap-dong, Songpa-gu, Seoul, Republic of Korea, 138-736.

出版信息

Interact Cardiovasc Thorac Surg. 2008 Apr;7(2):282-4. doi: 10.1510/icvts.2007.171017. Epub 2008 Jan 17.

Abstract

A twenty-four-day-old girl, who was prematurely born at 36 weeks of gestation, and weighed 2.2 kg, and diagnosed with right atrial isomerism, functionally single ventricle, bilateral superior vena cava (SVC) and obstructive supracardiac total anomalous pulmonary venous drainage (TAPVD) draining to the junction between the right SVC and the right atrium, underwent a hybrid procedure in the operating room, which consisted of pulmonary artery banding, ductus ligation and stenting of the draining vein of TAPVD. Obstruction at the drainage site of TAPVD was initially relieved after stenting, but, one month after the procedure, the distal end of the stent became stenotic and she received bilateral sutureless repair of TAPVD. At postoperative seven months, she underwent bidirectional cavopulmonary shunt uneventfully, and she has been followed-up for two months in a stable state without any problem in the pulmonary venous pathway.

摘要

一名24天大的女孩,孕36周早产,体重2.2千克,被诊断为右心房异构、功能性单心室、双侧上腔静脉及梗阻性心上型完全性肺静脉异位引流(TAPVD),引流至右SVC与右心房交界处,在手术室接受了杂交手术,包括肺动脉环扎、动脉导管结扎及TAPVD引流静脉支架置入。TAPVD引流部位的梗阻在支架置入后最初得到缓解,但术后1个月,支架远端出现狭窄,她接受了TAPVD双侧无缝合修补术。术后7个月,她顺利接受了双向腔肺分流术,且已随访2个月,状态稳定,肺静脉通路无任何问题。

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