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Early Radiological Intervention in Budd-Chiari Syndrome: Clinical Outcomes From a Single-Center Study
Tai Ermongkonchai1,2, Alysia Bastas1,2, Katrina Tan1,2
1Liver Transplant Unit Victoria, Austin Health, Heidelberg, Victoria, Australia, austin.org.au.
International Journal of Hepatology
|August 13, 2026
Summary
Budd-Chiari Syndrome (BCS) is often caused by myeloproliferative neoplasms (MPN). Early transjugular intrahepatic portosystemic shunt (TIPS) for liver decompression offers excellent outcomes, while anticoagulation alone may lead to disease progression.
Area of Science:
- Hepatology
- Vascular Medicine
- Hematology
Background:
- Budd-Chiari Syndrome (BCS) is a liver vascular disease characterized by venous outflow obstruction.
- Myeloproliferative neoplasms (MPN) are a frequent underlying cause of BCS.
- Effective treatment focuses on restoring hepatic venous outflow and long-term anticoagulation.
Purpose of the Study:
- To evaluate treatment outcomes for primary BCS.
- To assess the incidence of underlying MPN in BCS patients.
- To analyze the efficacy of different therapeutic interventions.
Main Methods:
- Retrospective analysis of 43 primary BCS patients over 28 years.
- Inclusion of hematological work-up and radiological intervention data.
- Assessment of overall and transplant-free survival.
Main Results:
- MPN was detected in 62.5% of patients.
- Overall survival was 100% at 1 year and 92.9% at 5 years.
- Transjugular intrahepatic portosystemic shunt (TIPS) was associated with excellent long-term outcomes.
Conclusions:
- MPN, especially JAK2V617F-positive, is a key cause of primary BCS.
- Anticoagulation alone can lead to disease progression.
- Early TIPS for hepatic decompression is safe and effective for BCS management.