小儿复杂先天性心脏病侧通道和外管道Fontan术比较

Complex congenital heart disease in children： a comparison between lateral tunnel andextracardiac condurit Fontan procedure

目的总结侧通道和外管道Fontan术纠治小儿危重复杂型先天性心脏病（先心病）的经验。方法2000年1月至2006年12月128例复杂型心内畸形病儿行改良Fontan术治疗。男76例，女52例。年龄1．9-16．5岁，平均（6．2±3．8）岁；体重8—62妇，平均（19．0±5．5）妇。病种主要有单心室（sV）59例；右室双出口（DORY）25例等。一期Fontan术87例；二期Fontan术41例。两次手术间隔0．8～7．3年，平均（3．9±2．8）年。首次手术为单侧Glenn术（12例）、双侧双向Glenn术（8例）等。行心内侧通道（LT）的Fontan组（LT组）89例，行外管道（ECC）的Fontan组（ECC组）39例。术前均行二维多普勒超声，11例加做核磁共振，30例行心导管和心血管造影。结果死亡17例（13．3％）。LT组病儿年龄明显低于ECC组。32例发生低心输出量综合征，其中11例肾功能受损导致无尿而行腹膜透析术（2～5d后尿量恢复）。LT组术后心律紊乱的发生率明显高于ECC组。门诊随访3～24个月，均未出现严重并发症，无死亡病例。结论LT组术后心律紊乱的发生率明显高于ECC组，ECC的Fontan术具有可避免主动脉阻断和心肌缺血、缩短CPB时间和保持更为流线型的下腔静脉回流血的优点，开窗术的远期优越性未能明确。

Objective To compare between lateral tunnel and extracardiac conduit Foutan procedure in complex congenital heart disease in children. Methods 128 patients with complex congenital heart disease received the modified Fontan procedure. They aged from 1.9 to 16.5 years and weighted from 8 to 62 kg. The main abnomudities included single ventricle （SV） in 59 eases and double outlet fight ventricle in 25. 87 patients were treated with one stage Fontan procedure and 41 patients with two stages Fontan procedure. The time between the two operations was 0.8 - 7.3 years [（3.9 ± 2.8） years]. The first operations were Glenn in 12 eases, double-Glenn in 8 and other preeedures in 21. 89 patients were treated by lateral tunnel Fontan procedure （LT） and 39 patients by extracardiac conduit Foutan procedure （ECC）. They were evaluated by ECHO, MRI for 11 eases and catheterization for 30 cases before operation. Results The raortality was 13.3% （17/28）. The age at operation in the LT group was yonger than in the ECC group. Early postoperative outcome was low cardiac output in 32 patients, renal dysfunction in 11. The postoperative nnhythmias rate in the LT group was highet than in the ECC group. The time of follow-up was from 3 months to 2 years and the results were satisfying. Conclusion The postoperative arrhythmias rate in the LT group was higher than in the ECC group. The ECC Fontan procedure may avoid the aortic cross-clamping, minimse the eardiiopulmonary bypass and keep direct flow from inferior vena eava to superior vena cava. The superiority of fenestration is still uncertain.