全腔静脉-肺动脉连接术Ⅰ期或分期生理矫治复杂先天性心脏病

Single or 2-stage total cavo-pulmonary connection to correct the complex congenital heart diseases？ - Single center experience and lessons from 88 cases

目的 总结全腔静脉-肺动脉连接术（TCPC）生理矫治复杂先天性心脏病的疗效,探讨选择Ⅰ期或分期TCPC手术的策略.方法 2003年12月至2009年11月,为88例患儿施行了TCPC术.男58例,女30例.低风险病例进行Ⅰ期TCPC术,高风险病例进行分期TCPC术.Ⅰ期手术组41例,平均年龄（8.4±4.5）岁;分期手术组47例,平均年龄（9.0±4.2）岁.Ⅰ期TCPC组中27例采用外管道,14例肺动脉直接下拉与下腔静脉吻合.Ⅱ期TCPC组中42例采用外管道,3例采用心房内通道,2例肺动脉直接与下腔静脉吻合.结果 早期死亡6例,病死率6.8%.Ⅰ期TCPC组死亡5例（4例死于重度低心排征,1例死于呼吸衰竭）,病死率12.2%;Ⅱ期TCPC组死亡1例（死于开胸时大出血）,病死率2.1%,二组差异无统计学意义（P=0.054）.二组的体外循环时间、辅助通气时间、胸管停留时间、住监护室时间、术后住院大数差异无统计学意义.65例随访4个月到6.5年.1例于术后8个月死于严重的房室瓣反流、心力衰竭;1例术后3个月出现感染性心内膜炎,死于多器官衰竭.结论 Ⅰ期和分期TCPC都能达到满意的手术效果.分期TCPC的效果似较Ⅰ期TCPC好.对存在两个或两个以上危险因素者,应选择分期手术.低风险患儿也应尽可能进行分期TCPC手术.

Objective To summarize the experience of single or 2-stage total cavo-pulmonary connection （TCPC） to correct the complex congenital heart diseases in children. Methods From December 2003 to November 2009, 88 patients underwent TCPC at this center. Among them, 58 were male and 30 were female. Surgical risks were assessed before surgery. The 41 patients with low surgical risks underwent single stage TCPC, and the other 47 high risk patients were performed 2-stage TCPC. Among the single stage TCPC group,extracardiac conduits were performed on 27 patients,and direct anastomosis between pulmonary trunk and inferior vena cava on the other 14 patients. Among the patients performed 2-stage TCPC, extracardiac conduits were performed on 42 patients, intracardiac conduit on 3, and direct anastomosis between pulmonary trunk and inferior vena cava on the rest 2 patients. Results Six patients died after surgery with overall mortality of 6. 8%,including 5 with single stage TCPC patients （mortality, 12. 2%） and 1 with 2-stage TCPC patient （mortality,2. 1 %）. Among the five deaths with single stage TCPC,4 died for severe low cardiac output syndrome, and the other 4 died for respiratory failure. The 1 death with 2-stage TCPC died for severe bleeding when opening the chest during the operation. No significant differences of mechanical ventilating time, duration of chest tube, ICU duration, and post operative hospital stay were noted between the 2 groups. The 65 survived patients were followed up from 4. 0 months to 6. 5 years. One patient died for sever atrialventricular valve regurgitation and heart failure 8 months later after the surgery. One patient died for multiple organs failure caused by infective endocarditis. Conclusions This study suggests the short-term outcomes of 2-stage TCPC are better than the single stage TCPC. The 2-stage TCPC is recommended to all the natients especiallv to the patients with 2 or more risk factors.