Total Anomalous Pulmonary Venous Return Complicated by Progressive Pulmonary Venous Stenosis after Total Repair: Report of One Case

全肺靜脈回流異常於手術矯正後出現漸進性肺靜脈狹窄：一病例報告

張正成(Jeng-Sheng Chang) ； 賴永章(Yung-Chang Lai) ； 李秉純(Ping-Chun Li) ； 陳志榮(Chi-Long Chen)

《Acta Paediatrica Taiwanica》 43卷5期 (2002/10) Pp. 276-280

https://doi.org/10.7097/APT.200210.0276

摘要

一名三個月大的女嬰因罹患阻塞性心下型全靜脈流異常(TAPVR,infracardiac type)於本院接受緊急開心手術矯正。一周後，心臓超音波檢查顯示四條肺靜脉都可順利地回流至左心房，但是後續的心臓超音波追踪檢查却發現肺靜脉産生漸進性的阻塞。病人在50天大時因嚴重肺充血死亡。病理解剖發現左心房與原來肺靜脉的會合靜脉(PV confluence)之間的接合很通暢，但是各條肺靜脉都有內皮細胞的增生與管經的阻塞，而且阻塞的範圍往各肺靜脉的上游延伸上去。文獻上記錄，心下型全肺靜脈異常的病人於開心手術後有很高比率會發生漸進性肺靜脈狹窄的情形。特別是肺靜脉管經本來就較小的病人更易發生。我們因此建議：對此類病人應在第一次開心手術矯正時就應盡量分離出每一條肺靜脉，幷利用心包膜片以製造一個較大的接合口，而且應避免使用連續縫合法。如果，術後仍不幸發生這種漸進性肺靜脉狹窄的幷發症，就只好采用一種“無縫線新心房”的術式來修補。

關鍵字

全肺靜脈回流異常 ； 肺靜脈狹窄

並列摘要

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.

並列關鍵字

Total anomalous pulmonary venous return ； pulmonary venous stenosis