室间隔完整的肺动脉闭锁外科治疗

Surgical treatment of the pulmonary artery atresia with intact ventricular septum

目的:总结分析40例室间隔完整的肺动脉闭锁(PAA/IVS)患儿的外科治疗,评价其临床疗效,并探讨适当的术式。方法:将40例PAA/IVS患儿根据手术治疗情况分为3组:①"杂交"技术组(14例,A组),包括"杂交"技术+动脉导管未闭(PDA)结扎7例(A1亚组,三尖瓣Z值0～-1)和"杂交"技术+PDA结扎+体肺分流术7例(A2亚组,三尖瓣Z值0～-1);②双心室手术矫治组(15例,B组),包括右室流出道跨环补片+PDA结扎术11例(B1亚组,三尖瓣Z值-0.5～-2.0)和右室流出道跨环补片+PDA结扎+体肺分流术4例(B2亚组,三尖瓣Z值0.5～-2.0);③减状手术组(11例,C组),包括1个半心室矫治术3例(C1亚组,三尖瓣Z值-2～-3)、双向格林术7例(C2亚组,三尖瓣Z值-4～-5)及体肺分流术1例(C3亚组,三尖瓣Z值-5)。结果:与B组比较,A组患儿年龄小,体重轻,术后呼吸机辅助时间和ICU住院时间明显缩短(均P<0.05)。40例患儿中,围手术期死亡5例,其中A组死亡1例,B组死亡4例,C组无一例死亡。术后随访3个月～5年,多数经历一期手术的患儿体重增加,体力活动增大,紫绀消失。结论:根据三尖瓣Z值,对于右室发育较好的PAA/IVS,采用"杂交"技术的治疗效果明显优于传统外科手术。

Objective：To evaluate the clinical results of hybrid and classic surgical procedure for the management of pulmonary artery atresia with intact ventricular septum（PAA/IVS） in children.Method：Forty children with PAA/IVS were divided into three groups according to the surgical procedure： group A1（n=7,Z score 0——1） were received hybrid procedure with PDA ligation,and group A2（n=7,Z score 0——1） were received hybrid procedure and BT shunt with PDA ligation;group B1（n=11,Z score-0.5——2.0） were received ROVT reconstruction with PDA ligation,and group B2（n=4,Z score 0.5——2.0） were received ROVT reconstruction with PDA ligation and BT shunt;group C1（n=3,Z score-2——3） were received one and a half ventricular repair;group C2（n=7,Z score-4——5） were received bi-directional Glenn bypass operation,and group C3（n=1,Z score-5） were received BT shunt.Result：Five babies（5/40） died（1 from group A,and 4 from group B）.The body weight,age,respiratory support time and postoperative hospital stay of babies were less in group A than group B（P0.05）.Conclusion：In carefully selected babies with PAA/IVS according to the tricuspid valve diameter,right ventricular size and coronary anatomy,hybrid procedure is feasible and may represent a new alternative to classic surgery operation.