摘要目的 探讨合并复杂先天性心脏病(CHD)的终末期肝病儿童的肝移植手术治疗策略.方法 回顾性分析2例合并复杂CHD的胆道闭锁儿童肝移植的临床资料,并结合现有相关文献资料,讨论合并复杂CHD的终末期肝病儿童的不同手术方案对预后的影响.结果 例1为7个月龄男性患儿,胆道闭锁合并无顶冠状静脉窦、永存左上腔静脉、卵圆孔未闭、周围肺动脉狭窄.该患儿未行先天性心脏病矫正术,先行原位肝移植术,肝移植手术耗时6 h 35 min,术后发生心功能不全、低氧血症,继发肺炎于术后12 d死亡.例2为7个月龄男性患儿,胆道闭锁合并室间隔缺损、卵圆孔未闭、动脉导管未闭及肺动脉瓣狭窄.手术方案采用先行先天性心脏病矫正术后行原位肝移植的序贯式联合手术,总手术耗时16 h 15min,术后60 h拔除气管插管,术后6d转出重症监护室,心肺功能稳定.该患儿术后早期出现肝动脉闭塞,继发缺血性胆道并发症,于术后40 d行二次肝移植,手术顺利,术后移植肝功能恢复良好.结论 合并复杂CHD的终末期肝病儿童的肝移植围手术期风险高,应根据心脏病及肝病的严重程度制定个体化的手术治疗方案.

abstractsObjective To discuss the surgical strategy for children with complex congenital heart disease (CHD) and end-stage liver disease (ESLD).Methods We reported two eases of pediatric liver transplantation in patients with complex CHD and ESLD.Medical data including operation procedure,ICU management and outcomes were reviewed retrospectively.Also we reviewed the literature on the topic of clinical outcomes resulted from different surgery options.Results The first case was a seven-month-old male patient with biliary atresia and complex CHD (unroofed coronary sinus syndrome,persistent left superior vena cava,patent foramen ovale,and peripheral pulmonary stenosis).Liver transplantation was successfully performed without corrective heart surgery.The operation time was 6 h and 35 min.The patient suffered acute cardiac dysfunction and significant hypoxemia after extubation,then pneumonia developed,and eventually the patient died on post-operative day 12.The second case was a seven-month-old male patient with biliary atresia and complex CHD (ventricular septal defect,patent foramen ovale,patent ductus arteriosus,pulmonary stenosis).Liver transplantation was performed on the same day following total correction of cardiac defects by open-heart surgery.The operation time was 16 h and 15 min.The patient was extubated after 60 h ventilation,and was transferred to ward from ICU on post-operative day 6 with stable cardiopulmonary function.However,hepatic artery occlusion occurred on early postoperative stage,and consequently the patient received the second liver transplantation for ischemic biliary complication on post-operative day 40.The second liver transplantation procedure was uneventful.The liver graft recovered smoothly with stable hemodynamics.Conclusion Children with complex CHD undergoing liver transplantation are at an increased perioperative risk.The surgical strategy for each patient must be tailored individually according to specific cardiovascular status and limited hepatic reserve.