Living Donor Liver Transplant in Patients With Budd-Chiari Syndrome: A Single-Center Experience at Our University Hospital.

Abstract

Budd-Chiari syndrome is an infrequent, but potentially fatal, hepatic condition with the clinical manifestation of obstructed venous drainage. This may lead to progressive hepatic congestion, portal hypertension, and, ultimately, liver failure. If medical, interventional, and surgical approaches are not effective, liver transplant offers a rescue modality. The primary objective of this study was to report the perioperative and, above all, the vascular challenges associated with living donor liver transplant in patients with Budd-Chiari syndrome. We retrospectively reviewed demographic and clinical characteristics of 6 patients with Budd-Chiari syndrome who underwent living donor liver transplant at our transplant center from April 2004 to July 2020. We also evaluated all data regarding perioperative course, surgical outcome, and the postoperative follow-up period. All patients displayed advanced liver disease with a Child-Pugh score C. The mean calculated Model for End-Stage Liver Disease score was 32. The causes of Budd-Chiari syndrome were factor V Leiden thrombophilia in 1 patient, myeloproliferative disorder in 3 patients, antiphospholipid antibody syndrome in 1 patient, and a protein C deficiency in 1 patient. The mean age of patients was 40 years. One of the 6 patients was female. All patients had living donor liver transplant from immediate kin according to Jordanian allocation rules. The mean graft-to-recipient weight ratio was 0.9, and the median follow-up period was 89 months. Cumulative 1-, 3-, and 5-year-survival rates were 84%, 67%, and 67%, respectively. Good survival rates are achievable with living donor liver transplant for patients with advanced Budd-Chiari syndrome, particularly by means of posterior cavoplasty for enlargement of the cava orifice. Therefore, in countries with insufficient deceased donor programs, such as Jordan, living donor liver transplant may be a lifesaving therapeutic possibility.