Budd-Chiari syndrome
ICD-10 I82.0 · ICD-11 DB98.5

Treatment of Primary Budd-Chiari Syndrome in Adults Without Acute Liver Failure

This page addresses the management of primary, non-fulminant Budd-Chiari syndrome in adult patients — specifically the presentation in which acute liver failure is absent.

Clinical scenario: Adult patients with primary Budd-Chiari syndrome in its non-fulminant form. The absence of acute liver failure is a defining feature of this presentation. A stepwise management strategy is recommended, with escalation for patients who do not respond to earlier steps in the pathway.

Management approach

For patients in this scenario who do not respond to prior medical or interventional therapies, a definitive surgical intervention is part of the evidence-based pathway — the complete protocol specifies the criteria and conditions under which it applies.

Instant Access to Structured Evidence-Based Regimens

References

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 patients with liver disease not responding to medical or interventional therapy or in those with HCC, LT should be considered.

DOI: 10.1016/j.jhep.2025.08.001

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