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完全性肺静脉异位引流在完全修复术后合并进行性肺静脉狭窄:1例报告

Total anomalous pulmonary venous return complicated by progressive pulmonary venous stenosis after total repair: report of one case.

作者信息

Chang Jeng-Sheng, Lai Yung-Chang, Li Ping-Chun, Chen Chi-Long

机构信息

Division of Pediatric Cardiology, Department of Pediatrics, China Medical College Hospital, No. 2, Yuh-Der Road, Taichung, Taiwan.

出版信息

Acta Paediatr Taiwan. 2002 Sep-Oct;43(5):276-80.

Abstract

Total repair of an infracardiac type total anomalous pulmonary venous return was performed on a 3-day-old female newborn. Echocardiogram study showed a smooth connection between the pulmonary veins and the left atrium one week after the operation, however, later echocardiograms showed a progressive obstruction of the pulmonary veins. The patient died at 50 days of age due to lung congestion. The autopsy revealed severe intima hypertrophy and upstream obstruction in every pulmonary vein, while the anastomosis between the left atrium and the pulmonary venous confluence remained well patent. In view of the high rate of progressive pulmonary venous stenosis after total repair in patients with infracardiac type total anomalous pulmonary venous return, it is advised that the operator should try to mobilize every pulmonary vein, make larger areas of anastomosis with pericardial patch augmentation and avoid using continuous suture. When the progressive pulmonary venous stenosis occurred during the follow-up period, a 'sutureless neoatrium' procedure can be used to resolve the obstructions.

摘要

对一名3日龄女性新生儿进行了心内型完全性肺静脉异位引流的完全修复手术。术后一周超声心动图检查显示肺静脉与左心房之间连接通畅,但随后的超声心动图显示肺静脉逐渐出现梗阻。该患者于50日龄时因肺充血死亡。尸检发现每条肺静脉均有严重的内膜肥厚和上游梗阻,而左心房与肺静脉汇合处的吻合口仍保持通畅。鉴于心内型完全性肺静脉异位引流患者在完全修复后发生进行性肺静脉狭窄的发生率较高,建议术者应尽量游离每条肺静脉,用心包补片扩大吻合面积并避免使用连续缝合。当随访期间出现进行性肺静脉狭窄时,可采用“无缝合新心房”手术来解除梗阻。

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