室间隔完整型肺动脉闭锁手术治疗的个体化方案

Clinical research of individual operation scheme in pulmonary atresia with intact ventricular septum

目的总结室间隔完整型肺动脉闭锁（pulmonary atresia with intact ventficular septum，PA／IVS）的个体化手术方案。方法回顾2004年5月至2012年5月，共收治72例PA／IVS患儿（婴），生后1天～5．2岁，平均（1．3±0．6）岁；三尖瓣（TV）中到重度反流58例；均行急诊或亚急诊手术。首次就诊非体外循环下单纯行BT分流术（A组）16例；体外循环下行肺动脉瓣切开后跨瓣补片术加或不加BT分流术（B组）33例；镶嵌治疗方法，闭式肺动脉瓣口的疏通术加或不加BT分流术（C组）22例。其中有16例已完成二期手术。结果术后死亡10例（13．9％），其中行体外循环下行肺动脉流出道的疏通再加BT术者6例，镶嵌治疗者2例。术后随访，超声检查显示肺动脉瓣的跨瓣压差15～39mmHg（1mmHg=0．133kPa），平均（23±5）mmHg；三尖瓣反流明显改善，中度反流11例，轻到中度13例，其余均为轻度。术后随访2—5年，8例双心室修补，5例中度的三尖瓣反流加重到重度，右心室严重的发育不良者已行11／2心室修补，2例分别完成了单纯BDG和Fontan术。结论PA／IVS的治疗方法中体肺动脉分流术（MBTS）是常用的姑息手术方法，首次就诊镶嵌治疗方法优于传统的体外循环方法纠治，随访三尖瓣的z值和右心室发育情况决定双室修补、11／2心室或Fontan术。

Objective Pulmonary atresia with intact ventricular septum （PA/IVS） is a rare complicated congenital heart disease. The intracardiac structure is also complicated. We sum up the corporal surgical procedure of PA/IVS. Methods Between May 2004 and May 2010, we performed emergency or sub-emergency operation in 72 newborns, infants and children [ age, 1 day to 5.2 years ; mean age, （ 1.3 ± 0.6） years ] with favorable anatomy. The surgical procedures were divided intothree kinds： 1.16 cases were performed with Blalock-Tassuing （B-T） shunt operation for A group; 2.33 cases were performed with transannular patch from RVOT-MPA with or without B-T shunt operation for B group. 3. 22 cases were performed a hybridprocedure with or without B-T shunt operation for C group. From all the patients, 15 cases had been finished the two staged op- eration. Results 10 patients has been died in which 6 patients were in B group with B-T shunt. Only 2 patients were for thehybrid procedure. The ECHO showed the gradient across the pulmonary annular was 15 - 39 mm Hg [ （ 23 ± 5 ） mm Hg （ 1 mm Hg = 0. 133 kPa） ] after operation. The tricuspid insufficient had been siginificantly improved. There were 58 caseswere preoperatively serious tricuspid insufficient. There were 11 cases and 13 cases were respectively for middle and mild to middle tricuspid insufficient postoperatively. Follow-up with 2 - 5 years, there were radical operation for 8 cases, one and halfventrical operation for 5 cases and BDG and Fontan operation for 2 cases. Conclusions. Conclusion BT shunt in common procedure and using a hybrid approach is more safe and feasible than traditional procedure for the patients with PA/IVS. Z valeand right ventricular growth were importment for procedure in the future including radical ,one and half ventrical and Fon tan operation.