Complex congenital heart disease and pediatric liver transplantation: case reports and a brief review / 中华器官移植杂志

ABSTRACT

Objective 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.