• Bibiana Escobar, Pilar Taura, Nelson Barreneche, Joan Beltran, Jaume Balust, Graciela Martinez-Palli, Elizabeth Zavala, Angels Escorsell, and Juan Carlos Garcia-Valdecasas.
• Department of Anesthesiology, Hospital Clinic, University of Barcelona, Barcelona, Spain.
• Liver Transpl. 2009 Aug 1; 15 (8): 869-75.

AbstractFamilial 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.

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