背景:越来越多的中心在成人和儿童先天性心脏病患者中进行心脏和肝脏联合移植。
目的:本研究的主要目的是描述单中心队列的围手术期管理,识别挑战和潜在的解决方案。
方法:我们对2006年至2022年在斯坦福儿童医院接受心脏和肝脏联合移植的所有患者进行了回顾性回顾。术前信息包括心脏诊断,血流动力学,和肝脏疾病的严重程度。术中数据包括手术长度,体外循环时间,和输血的血液制品。术后数据包括在重症监护病房输血的血液制品,拔管时间,重症监护病房住院时间,生存结局和30天不良事件.
结果:2006年至2022年,18例患者在斯坦福儿童医院接受了整体心脏和肝脏联合移植,大多数15例(83%)因Fontan相关肝病导致Fontan循环衰竭而移植。手术操作时间中位数为13.4[11.5,14.5]h,体外循环时间为4.3[3.9,5.8]h。体外循环后手术室输注的总血制品中位数为89.4[63.9,127.0]mL/kg。9例患者(50%)在体外循环期间有血管停搏液。15例(83%)患者在体外循环后使用活化的凝血酶原复合物浓缩物,30天血栓栓塞率为22%。拔管的中位时间为4.0[2.8,6.5]天,中位重症监护病房住院时间20.0[7.8,48.3]天,中位住院时间54.0[30.5,68.3]天.肾脏替代治疗的发生率为11%;然而,出院时没有人需要肾脏替代治疗.30天内的神经系统事件为17%,30天和1年生存率为89%。
结论:围手术期的挑战包括围手术期大出血,不稳定的血液动力学,和终末器官损伤,包括急性肾损伤和神经系统事件。通过仔细的多学科计划,完整的心脏和肝脏联合移植的成功结果是可能的。通信,患者选择,和综合围手术期管理。
BACKGROUND: An increasing number of centers are undertaking combined heart and liver transplantation in adult and pediatric patients with congenital heart disease.
OBJECTIVE: The primary aim of this study was to describe the perioperative management of a single center cohort, identifying challenges and potential solutions.
METHODS: We conducted a retrospective review of all patients undergoing combined heart and liver transplantation at Stanford Children\'s Hospital from 2006 to 2022. Preoperative information included cardiac diagnosis, hemodynamics, and severity of liver disease. Intraoperative data included length of surgery, cardiopulmonary bypass time, and blood products transfused. Postoperative data included blood products transfused in the intensive care unit, time to extubation, length of intensive care unit stay, survival outcomes and 30-day adverse events.
RESULTS: Eighteen patients underwent en bloc combined heart and liver transplantation at Stanford Children\'s Hospital from 2006 to 2022, and the majority 15 (83%) were transplanted for failing Fontan circulation with Fontan Associated Liver Disease. Median surgical procedure time was 13.4 [11.5, 14.5] h with a cardiopulmonary bypass time of 4.3 [3.9, 5.8] h. Median total blood products transfused in the operating room post cardiopulmonary bypass was 89.4 [63.9, 127.0] mLs/kg. Nine patients (50%) had vasoplegia during cardiopulmonary bypass. Activated prothrombin complex concentrates were used post cardiopulmonary bypass in 15 (83%) patients with a 30-day thromboembolism rate of 22%. Median time to extubation was 4.0 [2.8, 6.5] days, median intensive care unit length of stay 20.0 [7.8, 48.3] days and median hospital length of stay 54.0 [30.5, 68.3] days. Incidence of renal replacement therapy was 11%; however, none required renal replacement therapy by the time of hospital discharge. Neurological events within 30 days were 17% and the 30 day and 1 year survival was 89%.
CONCLUSIONS: Perioperative challenges include major perioperative bleeding, unstable hemodynamics, and end organ injury including acute kidney injury and neurological events. Successful outcomes for en bloc combined heart and liver transplantation are possible with careful multidisciplinary planning, communication, patient selection, and integrated peri-operative management.