Management of Budd-Chiari syndrome (BCS) should be undertaken using a stepwise approach including anticoagulation, angioplasty/stent/thrombectomy/thrombolysis, TIPS and orthotopic liver transplantation, at experienced centres.
Long-term anticoagulation should be given to all patients with primary BCS.
Because of the increased risk of heparin-induced thrombocytopenia, the use of unfractionated heparin is generally not recommended and may only be reserved for special situations (e.g., glomerular filtration rate <30 ml/min, pending invasive procedures).
Stenoses that are amenable to percutaneous angioplasty/stenting (short length stenoses) should be actively looked for and treated accordingly.
TIPS insertion should be attempted by operators with specific experience in BCS when angioplasty/stenting/thrombectomy/thrombolysis is not feasible, and when the patient does not improve on medical therapy including anticoagulants.
Consider improvement as a combination of several of the following outcomes: decreasing rate of ascites formation, decreasing serum bilirubin, serum creatinine and INR when elevated (or increasing factor V in patients receiving vitamin K antagonists).
The BCS-TIPS prognostic index score can be used to predict outcome in patients in whom TIPS insertion is being considered.
Liver transplantation should be considered in patients with uncontrolled clinical manifestation despite a stepwise approach, or in patients with a high BCS-TIPS prognostic index score (>7) before TIPS placement.
In patients with BCS presenting as acute liver failure, urgent liver transplantation should be considered. Emergency TIPS should be performed, if possible, independently of listing for liver transplantation.
Corrigendum to ‘Baveno VII – Renewing consensus in portal hypertension’ [J Hepatol (2022) 959-974] Journal of Hepatology, Vol. 77, Issue 2