Early Radiological Intervention in Budd-Chiari Syndrome: Clinical Outcomes From a Single-Center Study.

BACKGROUND/AIMS: Budd-Chiari Syndrome (BCS) is a vascular disease of the liver characterized by venous outflow obstruction most often due to a myeloproliferative neoplasm (MPN). Restoration of hepatic venous outflow and long-term anticoagulation remain the mainstay of treatment. We examined our experiences and treatment outcomes of BCS, including comprehensive testing for underlying MPN. METHODS: A retrospective study was conducted to assess overall and transplant-free survival in patients with primary BCS. All cases of primary BCS that presented to our institution over the past 3 decades were included. Details relating to their hematological work-up and radiological interventions were recorded, as well as their longer term outcomes, including the need for liver transplantation. RESULTS: Forty-three primary BCS patients were identified over a 28-year period, with a median follow-up time of 134 months. MPN was detected in 25 (62.5%) of cases who had comprehensive testing. All patients received primary anticoagulation. Seventeen (39.5%) patients underwent primary transjugular intrahepatic portosystemic shunt (TIPS). Twenty (46.5%) received anticoagulation alone as primary therapy, of whom 14 (70%) failed therapy and ultimately needed a rescue TIPS. Four (9.3%) patients underwent liver transplantation. Overall survival was 100% and 92.9% at 1 and 5 years, respectively, with transplant-free survival of 97.7% and 88.1% at 1 and 5 years, respectively. CONCLUSION: MPN (particularly JAK2V617F-positive) is an important underlying cause of primary BCS. Anticoagulation alone without radiological intervention can lead to disease progression. In this single-center cohort, early TIPS for rapid hepatic decompression appeared safe and was associated with excellent long-term outcomes.