What are the current guidelines for the management of Budd-Chiari syndrome, including anticoagulation therapy?

Management of Budd-Chiari Syndrome (BCS) involves anticoagulation therapy as a key component to prevent thrombotic progression and manage venous outflow obstruction.

Although the provided NICE guideline NG158 primarily addresses venous thromboembolic diseases such as deep vein thrombosis (DVT) and pulmonary embolism (PE), the principles of anticoagulation apply to BCS, which is a hepatic venous outflow obstruction often caused by thrombosis.

Anticoagulation Therapy: Initiate anticoagulation promptly once BCS is diagnosed, unless contraindicated, to prevent further thrombosis and improve hepatic venous drainage. Low molecular weight heparin (LMWH) or direct oral anticoagulants (DOACs) such as apixaban or rivaroxaban are preferred options, considering patient-specific factors including renal function, bleeding risk, and comorbidities.

For patients with active cancer or other hypercoagulable states, anticoagulation should be tailored accordingly, with LMWH or DOACs considered based on individual risk profiles and drug interactions.

Duration of Therapy: Anticoagulation is generally recommended for at least 3 to 6 months, with longer-term or indefinite anticoagulation considered in cases of persistent prothrombotic risk or recurrent thrombosis.

Additional Management: In cases where anticoagulation is contraindicated or ineffective, mechanical interventions such as angioplasty, stenting, or transjugular intrahepatic portosystemic shunt (TIPS) may be considered, although these are beyond the scope of the provided guideline.

Regular monitoring of anticoagulation efficacy and safety, including baseline and follow-up blood tests (full blood count, renal and hepatic function, coagulation profile), is essential.

Shared decision-making with the patient regarding anticoagulation options and adherence support is recommended to optimize outcomes.

Note: Specific NICE guidance on Budd-Chiari syndrome is limited; thus, management is extrapolated from general venous thromboembolism guidelines (NG158) and clinical practice.

References: The recommendations on anticoagulation treatment for venous thromboembolism, including choice of agents, duration, and monitoring, are detailed in NICE guideline NG158 ¹.