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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 summarize and analyze the surgical treatment of 40 children with intact interventricular septum pulmonary atresia (PAA / IVS) and to evaluate their clinical efficacy and to explore appropriate surgical procedures. Methods: 40 cases of PAA / IVS children were divided into 3 groups according to the surgical treatment: ① “Hybridization” technique group (14 cases, group A), including “hybridization” Tissue was ligated in 7 cases (A1 subgroup, tricuspid Z value 0 ~ -1) and “hybridization” + PDA ligation + body shunt in 7 cases (A2 subgroup, tricuspid Z value 0 ~ -1) ; Biventricular surgery group (15 cases, group B), including right ventricular outflow tract cross-patch + PDA ligation in 11 cases (B1 subgroup, tricuspid Z value -0.5 ~ -2.0) and right ventricular outflow 4 patients (B2 subgroup, tricuspid Z value of 0.5 ~ -2.0); ③Sensorial surgery group (11 patients, group C), including one and a half ventricular correction Three patients (C1 subgroup, tricuspid Z score -2 ~ -3), bi-directional Green technique in 7 patients (C2 subgroup, tricuspid Z score -4 -5) and pulmonary shunt in 1 patient (C3 Subgroup, tricuspid Z-5). Results: Compared with group B, group A had younger age, lighter body weight, postoperative ventilator support time and hospital stay in ICU (all P <0.05). Among the 40 children, 5 were perioperatively, including 1 in group A, 4 in group B, and none in group C. The patients were followed up for 3 months to 5 years. Most children undergoing primary surgery gained weight, physical activity increased, and cyanosis disappeared. Conclusion: According to the Z value of tricuspid valve, the therapeutic effect of “hybridization” technique is better than that of traditional surgery for PAA / IVS with better right ventricular development.