Primary Budd-Chiari syndrome occasionally follows a fulminant course, presenting as acute liver failure. This is a distinct and urgent clinical scenario that requires a management strategy fundamentally different from non-fulminant presentations.
Primary Budd-Chiari syndrome with acute liver failure (fulminant presentation). Patients presenting this way represent a special population: liver transplantation must be considered from the start of management, not as a later escalation.
The approach involves emergency procedural intervention carried out in parallel with immediate transplant listing — the complete protocol specifies how these two strategies are coordinated and the conditions under which each applies.
DOI: 10.1016/j.jhep.2025.08.001
This applies to all patients except those who present with ALF, in whom liver transplantation (LT) should be considered from the start.
In patients with acute liver failure, emergency TIPS should be attempted, while in parallel listing the patient for LT, although transplantation may not always be needed.
View source ↗