婴幼儿复杂先天性心脏病的外科治疗

目的总结婴幼儿复杂先天性心脏病（CCHD）的临床特点及外科治疗结果,探讨手术适应证,提高手术疗效。方法1999年11月至2008年6月,武汉亚洲心脏病医院共施行婴幼儿CCHD323例,其中男202例（62.5%）,女121例（37.5%）;年龄4d～36个月,平均年龄18.4个月;体重4～15kg,平均体重9.9kg。病种为法洛四联症（TOF）218例,右心室双出口（DORV）41例,完全性肺静脉异位引流（TAPVD）12例,完全性心内膜垫缺损（TECD）8例,主动脉弓缩窄（CoA）15例,主肺间隔缺损（A-P Window）合并主动脉弓离断（IAA）、动脉导管未闭（PDA）2例,永存动脉干（PTA）2例,单心室（SV）9例,Ebstein畸形2例,肺动脉闭锁（PA）10例,大动脉转位（TGA）3例,矫正性大动脉转位（cTGA）1例。在低温体外循环下行I期矫治297例,姑息性手术26例。结果体外循环时间89±34min,主动脉阻断时间48±39min。全部患者均通过电话或书信获得随访,随访时间1～72个月,术后死亡8例（2.5%）,其中早期死亡7例（1个月内）,死亡原因为：手术时间长、术中不能停体外循环2例,不能脱离呼吸机3例,误吸致心脏骤停、复苏后多器官功能衰竭（MODS）1例,中央分流术后持续低氧、心脏骤停1例;中期死亡1例,死亡原因为喉炎并发肺部感染。生存315例（97.5%）,发生并发症95例（29.4%）,均经对症治疗出院;出院后心功能改善为Ⅰ～Ⅱ级,呼吸道感染减少,体重明显增加。结论婴幼儿CCHD应早发现、早诊断、早手术,手术治疗结果满意。具体术式应根据不同的畸形特点选择最适合患者的方法,强调手术的适应证及畸形的满意矫治,注重分期手术。

Surgical Treatment of Complex Congenital Heart Disease in Infants

Objective To summarize the clinical features and results of surgical treatment of complex congenital heart disease（CCHD） in infants,investigate the operative indications and improve the operative effect. Methods From November 1999 to June 2008,323 infants with CCHD were operated in Wuhan Asia Heart Hospital. There were 202（62.5%） male and 121（37.5%） female aged from 4 days to 36 months. The average age was 18.4 months. The range of weight was 4-15 kg,and the average weight was 9.9 kg. There were 218 cases with tetralogy of fallot（TOF）,41 with double outlet right ventricle（DORV）,12 with total anomalous pulmonary venous drainage（TAPVD）,8 with complete endocardial cushion defect（TECD）,15 with coarctation of aorta（CoA）,2 with aorta-pulmonary window（A-P Window） associated with interrupted aortic arch（IAA） and patent ductus arteriosus （PDA）,2 with persistent truncus arteriosus （PTA）,9 with single ventricle（SV）,2 with Ebstein＇s anomaly,10 with pulmonary atresia（PA）,3 with transposition of great arteries（TGA）and 1 with corrected transposition of great arteries（cTGA）. Two hundred and ninety-seven patients underwent I stage correction,26 underwent palliative operation. All the corrective operations were performed under hypothermic cardiopulmonary bypass（CPB）. Results The cardiopulmonary bypass（CPB） time and aortic cross clamping time were 89±34 min and 48±39 min,respectively. All the patients were followed up by telephone or mail. The follow-up time was 1-72 months. Eight patients（2.5%） died after operation,7 of them died in the early period of operation（within 1 month）. Two patients died of long operation time and CPB-dependence,3 died of ventilator-dependence,1 died of cardiac arrest caused by aspiration following multiple organ dysfunction syndrome（MODS）after resuscitation,and 1 died of continuous hypoxia and cardiac arrest after central shunt operation. There was 1 medium-term death,which was caused by laryngitis complicated with pu