Orthotopic Liver Transplantation for Budd-Chiari Syndrome: Observations from a 30-Year Liver Transplant Program

Abstract

Background and objectives Budd-Chiari syndrome (BCS) refers to a complete thrombotic obstruction of the venous hepatic outflow tract due to various etiologies and constitutes a rare indication for ortothopic liver transplantation (LT). Few studies investigated long-term outcomes after LT for BCS. The aim of this study was to examine potential risk factors for late mortality and to evaluate long-term outcomes after LT for BCS. Materials and methods: 46 patients received an LT for BCS between 1989 and 2019 at the transplant center of the Charité-Universitätsmedizin Berlin. We analyzed potential effects of disease etiology, vascular events, rejection, and immunosuppression on long-term survival after transplantation using Kaplan-Meier curves and Cox logistic regression. Results: Of the 46 patients, 70% were female and 30% were male. Median age at the time of transplantation was 36 years. A total of 41 vascular events, including 26 thrombotic and 17 hemorrhagic incidents, occurred. The 1 year, the 5 year, the 10 year, and the 20 year survival rates were 87%, 83%, 76%, and 60%, respectively. By comparison, survival rates of the liver transplant cohort across all other indications at our center were slightly inferior with 85%, 75%, 65%, and 46%, respectively. In the study population, patients with myeloproliferative disorders showed worse outcomes compared to patients with other causes of BCS. Conclusion: Liver transplantation for BCS showed excellent results, even superior to those for other indications. Vascular events (i.e., thrombotic or hemorrhagic complications) did not have any prognostic value for overall mortality. Patients with myeloproliferative disorders seem to have a disadvantage in survival.

What is the optimal management of thromboprophylaxis after liver transplantation regarding prevention of bleeding, hepatic artery, or portal vein thrombosis? A systematic review of the literature and expert panel recommendations

“…Furthermore, patient survival in the non‐physiological bypass group was significantly lower than the control group whereas the rate of hypercoagulable conditions was higher among recipients with the non‐physiological bypass 12 . Liver recipients with etiologies such as Budd‐Chiari Syndrome and underlying hypercoagulable states have a documented high‐rate of post‐transplant thrombotic complications thus prophylaxis with vitamin K antagonists post‐transplant has been a standard of care 42 …”

“… 12 Liver recipients with etiologies such as Budd‐Chiari Syndrome and underlying hypercoagulable states have a documented high‐rate of post‐transplant thrombotic complications thus prophylaxis with vitamin K antagonists post‐transplant has been a standard of care. 42 …”

Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature

“…Liver transplantation may be recommended as a last resort or in fulminant situations, with promising and favorable outcomes. Ibach et al [ 89 ] analyzed 46 cases of BCS of whom 22 suffered from MPNs and reported that individuals with BCS who were subjected to liver transplantation experienced a median survival of approximately 24 years. Mortality rates were higher in patients with BCS and MPNs (RR=3.44, P = 0.05), however, two patients diagnosed with these blood cancers died due to secondary acute myeloid leukemia and extramedullary hematopoiesis in the spleen with consequent organ rupture, events which can occur during disease evolution irrespective of the use of liver transplantation in these cases.…”