Escobar Bibiana, Taura Pilar, Barreneche Nelson, Beltran Joan, Balust Jaume, Martinez-Palli Graciela, Zavala Elizabeth, Escorsell Angels, Garcia-Valdecasas Juan Carlos
Department of Anesthesiology, Hospital Clinic, University of Barcelona, Barcelona, Spain.
Liver Transpl. 2009 Aug;15(8):869-75. doi: 10.1002/lt.21772.
Familial amyloidotic polyneuropathy (FAP) patients present adrenergic cardiac input blockade secondary to amyloid deposits and sympathetic neuropathy. Consequently, their capacity to compensate for hemodynamic changes is limited. To avoid hemodynamic disturbances in sequential liver transplants, a standard procedure with venovenous bypass or inferior vena cava preservation is contemplated. The aim of this study was to evaluate the impact of both techniques on the hemodynamic management and outcome of patients affected by FAP and scheduled for a domino liver transplantation program. We evaluated 36 FAP patients. Venovenous bypass was performed for 20 patients (the venovenous bypass group), whereas the vena cava preservation technique was used for the remaining 16 patients (the cava preservation group). The time that elapsed from FAP diagnosis to liver transplantation was 3.2 +/- 2.7 years. Peripheral neuropathy was present in all patients, autonomic dysfunction was present in 71%, and cardiac involvement was present in 69%. Renal and gastrointestinal manifestations were reported in 19% and 53% of patients, respectively. The 1-, 3-, and 5-year survival rates were 97%, 93%, and 93%, respectively. Intraoperative hemodynamic and cardiac disorders, need for vasoactive drugs, blood loss, and transfusion requirements were recorded. Postoperative outcome and cardiac and renal complications were also recorded. No significant differences in disease severity or demographic characteristics were observed. Among all the variables studied, only the total ischemia time and time in surgery were significantly longer in the venovenous bypass group patients (P < or = 0.05). During the postoperative period, the incidence of minor cardiovascular events, incidence of acute renal dysfunction, and outcomes were similar in the 2 groups. In conclusion, either preservation of the vena cava or the standard technique with venovenous bypass can be safely used in FAP patients during liver transplantation. Liver Transpl 15:869-875, 2009. (c) 2009 AASLD.
家族性淀粉样多神经病（FAP）患者存在继发于淀粉样蛋白沉积和交感神经病变的肾上腺素能心脏传入阻滞。因此，他们代偿血流动力学变化的能力有限。为避免在序贯肝移植中出现血流动力学紊乱，考虑采用静脉-静脉转流或保留腔静脉的标准术式。本研究的目的是评估这两种技术对计划进行多米诺肝移植的FAP患者血流动力学管理及预后的影响。我们评估了36例FAP患者。20例患者采用静脉-静脉转流（静脉-静脉转流组），其余16例患者采用保留腔静脉技术（腔静脉保留组）。从FAP诊断到肝移植的时间为3.2±2.7年。所有患者均存在周围神经病变，71%存在自主神经功能障碍，69%存在心脏受累。分别有19%和53%的患者报告有肾脏和胃肠道表现。1年、3年和5年生存率分别为97%、93%和93%。记录术中血流动力学和心脏紊乱情况、血管活性药物的使用需求、失血量及输血需求。还记录术后结局及心脏和肾脏并发症。未观察到疾病严重程度或人口统计学特征的显著差异。在所有研究变量中，仅静脉-静脉转流组患者的总缺血时间和手术时间显著更长（P≤0.05）。术后期间，两组的轻微心血管事件发生率、急性肾功能障碍发生率及结局相似。总之，在肝移植期间，保留腔静脉或采用静脉-静脉转流的标准技术均可安全用于FAP患者。《肝脏移植》15:869 - 875，2009年。（c）2009年美国肝脏病研究协会。
