上海交通大学学报（医学版）

上海交通大学学报（医学版）

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伴有左侧肺动脉发育不良的法洛四联症外科治疗策略

高波涛，郑景浩，祝忠群，陈会文，张海波，徐志伟   

  1. 上海交通大学 医学院附属上海儿童医学中心心胸外科， 上海 200127
  • 出版日期:2015-06-28 发布日期:2015-07-30
  • 作者简介:高波涛（1979—）， 男， 主治医师， 硕士; 电子信箱: botaogao_79.0217@126.com。

Surgical strategies for treatment of tetralogy of Fallot with left pulmonary artery hypoplasia

GAO Bo-tao, ZHENG Jing-hao, ZHU Zhong-qun, CHEN Hui-wen, ZHANG Hai-bo, XU Zhi-wei   

  1. Department of Cardiothoracia Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China
  • Online:2015-06-28 Published:2015-07-30

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摘要：

目的 探讨伴有左侧肺动脉发育不良的法洛四联症（TOF）外科治疗策略。方法 回顾性分析17例伴有左侧肺动脉发育不良的TOF患儿，其中男性12例，女性5例，年龄2个月～3岁，体质量4～13 kg。术中根据患儿肺动脉情况行TOF根治或姑息手术。术后定期复查胸片、心电图、彩色多普勒超声心动图和CT，观察患儿是否有残余分流、残余梗阻以及心功能不全。结果 17例伴有左侧肺动脉发育不良的TOF患儿中，8例在左肺动脉补片扩大成形，同时行TOF根治术，其中7例存活，6例接受随访5～29（14±10）个月，2例随访中发现左肺动脉开口小，余5例无并发症出现，1例由于合并肺静脉狭窄且无法处理，术后出现严重低心排出量综合征而死亡；另9例患儿中，2例分别因术中探查发现冠状动脉畸形以及三尖瓣发育差改行单心室手术，其余7例行TOF姑息术，其中5例同时行左肺动脉补片扩大成形， 7例行TOF姑息手术者6例存活，5例术后随访6～18（17±13）个月，随访中发现4例患儿左肺动脉发育仍较差，1例术后由于心功能不全、多脏器衰竭放弃治疗后死亡。结论 伴有左侧肺动脉发育不良的TOF外科治疗应在左肺动脉补片扩大成形的基础上尽可能施行根治术，若术中预估术后可能存在左肺动脉残余梗阻，可行室间隔缺损补片打洞。

关键词: 法洛四联症, 肺动脉指数, 发育不良

Abstract:

Objective To investigate the surgical strategies for the treatment of tetralogy of Fallot (TOF) with left pulmonary artery hypoplasia. Methods A total of 17 TOF pediatric patients with left pulmonary artery hypoplasia were retrospectively analyzed. Among them, there were 12 males and 5 females with ages of 2 months-3 years and body weights of 4-13 kg. Curative repair or palliative repair was conducted according to the development of pulmonary artery. Regular postoperative examinations were performed, including X-ray, electrocardiogram, color Doppler echocardiography, and CT, to observe possible residual shunts, residual obstruction, and cardiac dysfunction. Results Among 17 TOF pediatric patients with the left pulmonary artery hypoplasia, 8 of them underwent patch cavoplasty of left pulmonary artery and curative repair and 7 patients survived. The 6 survived patients were followed up for 5-29 (14±10) months and among them, openings of left pulmonary artery of 2 patients were small and no complications were found among other 5 patients. One patient could not be treated due to pulmonary vein hypoplasia and died of severe low cardiac output syndrome. Among another 9 patients, 2 of them underwent single ventricular repair because of coronary artery abnormality and tricuspid valve hypoplasia, respectively, and 7 of them underwent palliative repair. Among 7 patients undergoing palliative repair, 5 of them underwent patch cavoplasty of left pulmonary artery; 6 of them survived, 5 of them were followed up for 6-18 (17±13) months and among them, there were 4 patients with left pulmonary hypoplasia and 1 patient died of cardiac dysfunction and multiple organ function failure. Conclusion TOF with left pulmonary artery hypoplasia should undergo the curative repair as far as possible based on patch cavoplasty of left pulmonary artery.If it is possible to develop the residual stenosis of left pulmonary artery after repair, the surgeon can punch a hole in the ventricular septal defect patch.

Key words: tetralogy of Fallot, pulmonary artery index, hypoplasia