Treatment of Primary Budd-Chiari Syndrome (Non-Fulminant) in Adult Patients

This protocol addresses primary Budd-Chiari syndrome in adults presenting without acute liver failure — the non-fulminant form — where a stepwise escalation of interventions applies.

Clinical scenario

Primary, non-fulminant Budd-Chiari syndrome in an adult patient. The absence of acute liver failure defines the management pathway: a structured stepwise strategy is followed, advancing through consecutive interventions when the patient does not respond to each prior step.

Treatment approach (partial overview)

When medical management alone is insufficient and percutaneous balloon angioplasty is not feasible or has not achieved an adequate response, a transjugular intrahepatic portosystemic shunt (TIPS) is the recommended next step. The full protocol specifies the technical approach and the criteria for stent selection.

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References

DOI: 10.1016/j.jhep.2025.08.001

In patients with primary, non-fulminant, Budd-Chiari syndrome, a stepwise management strategy should be followed consisting of anticoagulation and treatment of underlying conditions, followed consecutively by percutaneous angioplasty, TIPS, and LT in non-responsive patients.
In cases where medical management alone is insufficient to alleviate symptoms and percutaneous transluminal balloon angioplasty is not an option or has failed, TIPS is the recommended next step.
In this setting, portosystemic shunting, in particular TIPS, is the next step.

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