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

Primary Non-Fulminant Budd-Chiari Syndrome in Adults: What to Do After Initial Anticoagulation

Clinical scenario

This protocol addresses adults with primary Budd-Chiari syndrome presenting in a non-fulminant form — acute liver failure is absent. Evidence-based management follows a stepwise strategy, with escalating interventional steps applied when earlier treatment does not achieve the intended goal.

When initial treatment has not achieved target

The first line of management is therapeutic anticoagulation — initiated promptly and continued indefinitely unless contraindicated — combined with treatment of underlying conditions. When this approach does not achieve the target anticoagulation intensity (an INR of 2.0–3.0), or the patient does not respond adequately, the protocol moves to the next step.

Next step — partial overview

At this stage, patients are evaluated for specific anatomical abnormalities in the relevant venous structures. Where present, a percutaneous interventional approach is the indicated next step. A catheter-based adjunctive technique may be used alongside to support vessel patency.

The complete protocol — including precise indications, procedural sequence, and full management algorithm — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

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.

The next step in management is to actively look for short-segment stenoses in the IVC or hepatic veins that could be amenable to percutaneous transluminal balloon angioplasty and/or stenting, regardless of the response to anticoagulation.

In patients with short-segment stenoses in the inferior vena cava or hepatic veins, percutaneous transluminal balloon angioplasty should be considered.

Catheter delivered thrombolysis can be useful as adjunctive therapy to improve initial recanalisation or treat immediate rethrombosis during the percutaneous transluminal balloon angioplasty procedure.

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