肺动脉闭锁合并室间隔缺损和MAPCA手术理念探讨

The research of operation concept in pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals

目的探讨肺动脉闭锁（PA）合并室间隔缺损（VSD）有部分病例合并主肺动脉侧支（MAPCA）PA／VSD／MAPCA的手术理念。方法回顾2003年1月至2012年12月92例PA／VSD患儿（婴）情况。患儿（婴）年龄生后1天～5．2岁，平均（1．3±0．6）岁。接受姑息和根治手术，其中行一期根治术26例，分期姑息手术66例。姑息手术分两个阶段，分别为体肺分流、右心室流出道和肺动脉的管道连接、合并肺动脉狭窄的扩大成形以及MAPCA的结扎、填塞或单源化，最终17例已完成了二期和三期的根治手术。结果第一阶段手术死亡3例。第二阶段9例患儿达到了根治手术条件而完成了二期根治手术，死亡1例。第三阶段8例患儿完成了根治术，3例行姑息性根治术，死亡1例。结论PA／VSD／MAPCA的治疗方法可以早期建立主肺动脉的连接或右心室肺动脉的连接，来促使自身肺动脉的发育，单源化手术应在尽早进行，同时多余的MAPCA不断地得到阻断和结扎。采用分期的手术方法可以达到根治效果。

Objective Sum up the research of concept of surgical procedure of PA/VSD/MAPCA. Methods Between January 2003 and December 2012, 92 children were operated, aged 1 day to 5.2 years; median age （ 1.3 ± 0.6 ） years. There were 26 cases for one stage operation, 66 cases were for palliative operation which were divided into 3 stages. The 3 stages of palliative operation included system to pulmonary shunt, conduit from RVOT to MPA or transannular patch from RVOT to MPA, enlargemene of pulmonary artery and ligation of MAPcA. From all the patients, 15 cases had been finished the two staged operation. In the end, there were 17 cases who were completed two or three stage radical operation. Results There were 3 cases who died from the first period. There were 9 cases who were completed two stage operation, and 1 case died. There were 8 cases who were completed three stage operation, there was 1 cases who died in this period. Conclusion The surgical treatment for the PA/VSD/MAPCA could be that the connection between RVOT and pulmonary artery should be performed as early as possible which could provide the blood flow to pulmonary artery and promote the growth. Uniforcalization could also be set up as early as possible if it is needed. It could be ligated in different period. The PA/VSD/MAPCA could be repaired by stagesand have satisfied results.