改良侧通道Fontan手术在小儿复杂先天性心脏病中的应用

Application of Modified Lateral Tunnel Fontan Procedure on Complex Congenital Heart Disease in Children

目的总结改良侧通道Fontan手术纠治小儿危重复杂先天性心脏病的经验,探讨该手术方法的优越性。方法自1999年3月至2008年8月我院共对86例复杂型心内畸形患者施行心内板障侧通道Fontan手术,男47例,女39例;年龄1.9～11.5岁,平均年龄4.7岁;体重8.6～52.0kg,平均体重17.0kg。病种为无脾综合征33例,多脾综合征17例,三尖瓣闭锁(TA)11例,房室连接不一致的右心室双出口(DORV)11例,完全性大动脉错位(D-TGA)合并肺动脉狭窄8例,矫正性大动脉错位(cTGA)5例,Ebstein’畸形1例。术前分别行单侧双向上腔静脉肺动脉吻合术(BSCPA),双侧双向上腔静脉肺动脉吻合术和半-Fontan手术(hemi-Fontan opertiong);二次手术时间间隔0.7～7.8年(3.6±2.9年)。术中采用心内板障侧通道Fontan手术(LT组,47例)和改良心内板障侧通道Fontan手术(M-LT组,39例)方法连接下腔静脉的血引流入右肺动脉,部分完成二期改良Fontan手术。结果两组共死亡7例(9%),LT组死亡5例,M-LT组死亡2例,差异无统计学意义(χ2=0.865,P=0.448)。在分期改良Fontan手术中,M-LT组患者术前行BSCPA术明显多于LT组。术后仍有22例患者发生低心排血量综合征,其中肾功能受损导致无尿而行腹膜透析13例,透析2～5d后尿量恢复。术后LT组的心律失常患者明显多于M-LT组(χ2=8.763,P=0.003),置胸腔引流管时间LT组明显长于M-LT组(t=2.970,P=0.003)。门诊随访3个月～8年,无1例死亡。M-LT组随访33例(85%),LT组随访39例(83%),均未出现严重的并发症,患者活动能力明显改善。结论改良侧通道Fontan手术有一定的优越性,不失为一种提高手术成功率、减少术后并发症的改良方法。

Objective To summarize the treatment experiences of Modified lateral tunnel（LT） Fontan operation on complex congenital heart disease in children and investigate the advantages of this operation. Methods From March 1999 to August 2008,86 patients with cynosis complex congenital heart disease underwent LT Fontan operation in our hospital. There were 47 male and 39 female aged 1.9-11.5 years with a mean age of 4.7 years and weighed 8.6-52.0 kg with a mean weight of 17.0 kg. There were 33 cases with asplenia syndrome,17 cases with polysplenia syndrome,11 cases with tricuspid atresia（TA）,11 cases with double outlet right ventricle（DORV） of atrioventricular discordance,8 cases with complete transposition of great arteries（D -TGA） complicated with pulmonary stenosis,5 cases with corrected transposition of great arteries（cTGA） and 1 case with Ebstein＇s anomaly. Unilateral superior bidirectional superior cavopulmonary anastomosis（BSCPA）,bilateral bidirectional superior cavopulmonary anastomosis and hemi-Fontan opertion were done before operatipon. The time between two operations was 0.7-7.8 years（3.6±2.9 years）. LT Fontan operation（LT group,47cases） and Modified LT Fontan operation（M-LT group,39cases） were used in operation to drain blood from inferior vena cava to right pulmonary artery. Partly completed second-stage M-LT Fontan operation. Results There were 7 deaths in two groups（9%）,5 in LT group and 2 in M-LT group. There was no statistical significance（χ^2=0.865,P=0.448）. In stage-modified LT Fontan operation,there were significantly more cases who had BSCPA operation preoperatively in M-LT group than that in LT group. Twenty-two cases had low cardiac output syndrome after operation,13 cases underwent peritoneal dialysis because of renal dysfunction,and their urine volume recovered after 2-5 days＇ dialysis. There were significantly more cases who had arrhythmia in LT group than that in M-LT group（χ^2=8.763,P=0.003）. The time of chest drainage was longer in LT group than that in M-LT group（t=2.970,P=0.003）. The follow-up time was 3 months-8 years. No death was found. In M-LT group 33（85%） cases were followed up and in LT group 39（83%）cases were followed up. No severe complication was found. Patients＇ activity ability improved significantly. Conclusion The M-LT Fontan operation is an advanced operation to improve the success rate of operation and reduce postoperative complications.