This protocol is for adult patients with primary Budd-Chiari syndrome in the non-fulminant setting — specifically, the absence of acute liver failure. For this population, a stepwise management strategy is recommended, beginning with anticoagulation and treatment of underlying conditions, with further escalation options available for non-responsive patients.
The first-line priority is prompt initiation of therapeutic anticoagulation, to be continued indefinitely unless a major contraindication exists, together with addressing underlying conditions. The full regimen — including agent selection, sequencing, and monitoring targets — is detailed in the structured protocol.
The protocol targets a specific therapeutic anticoagulation range. The defined INR goal and monitoring approach are set out in the full protocol.
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 universal first step is to initiate anticoagulation as soon as possible and continue this indefinitely unless there is a major contraindication.
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