婴儿完全性肺静脉异位连接的外科治疗

Surgical therapy of infants with total anomalous pulmonary venous connection

目的 回顾性总结婴儿完全性肺静脉异位连接外科治疗的策略和效果.方法 2007年1月至2012年4月共收治婴儿各型完全性肺静脉异位连接41例,均在全麻中低温体外循环下实施矫治术.心上型采用心上吻合27例,心内吻合3例,其中自体心包扩大吻合口11例;心内型7例为连接至冠状静脉窦,彻底剪除肺静脉开口前方的房间隔组织和冠状静脉窦顶壁,以人工材料再造房隔的同时扩大左房,1例为连接至右房顶,扩大房缺后心内补片纠治;心下型2例在心外右房后方纵切肺静脉共干后纵行吻合.结果 39例存活,临床症状消失,体格发育明显改善,无心功能异常表现.早期死亡2例,死亡率为4.8％.1例为肺动脉高压危象心跳骤停,1例为左心发育不良低心排.39例全部门诊随访,除1例二次手术外,无近中期吻合口狭窄,随访无死亡.结论 完全性肺静脉异位连接作为一个在正常双心室心脏中发生的孤立性畸形时,婴儿期是积极手术的理想时机,手术效果满意,预后好.

Objective To retrospectively summarize the strategies and effects of surgical therapy on infants with total anomalous pulmonary venous connection （TAPVC）.Methods From January 2007 to April 2012,41 cases of infants with TAPVC were chosen.They were performed with surgical repairing with general anesthesia by hypothermic cardiopulmonary bypass.Twenty seven cases were treated by supracardiac anastomosis and 3 cases were treated by intracardiac anastomosis,among which 11 cases were treated by autologous pericardical expand anastomosis.As for intracardiac type,7 cases were cured by means of connecting pulmonary vein to coronary sinus,and then by cutting arterial septum and coronary sinus upper wall in the mouth of pulmonary vein,and finally using artificial materials to reconstruct interatrial septum to expand the left artrium; 1 case by linking pulmonary vein to right atrium roof,then the patients were remedied by expanding the interatrial septum defect and giving intracardiac patch.In terms of infracardiac type,2 cases were treated by cutting the pulmonary vein behind the right atrium in lengthways,and connecting the pulmonary vein to the left artrium by longitudinal anastomosis.Results Thirty-nine cases survived the operations,of whom the clinical symptoms disappeared and their physical growth improved obviously without cardiac dysfunction.Only 2 cases died in the early period （mortality rate 4.8％）,1 of which died from sudden cardiac arrest as the result of pulmonary arterial hypertension crisis and 1 from low cardiac output as the result of left heart maldevelopment.The outpatient follow-up found no death case,but one case was operated twice,with no short and medium-term anastomosis stricture.Conclusions TAPVC,as an independant abnormality located in a normal heart,can be cured in babyhood with ideal effects and perfect prognosis.