What is the treatment for Budd Chiari syndrome?

Treatment of Budd-Chiari Syndrome

Treatment for Budd-Chiari syndrome follows a sequential algorithm, beginning with immediate anticoagulation, followed by angioplasty if feasible, then transjugular intrahepatic portosystemic shunt (TIPS) if medical treatment fails, and finally liver transplantation for non-responders. 1

Initial Management

1. Immediate Anticoagulation

   • Start low molecular weight heparin for 5-7 days
   • Transition to oral vitamin K antagonists (target INR 2-3) 1
   • Direct oral anticoagulants (DOACs) may be considered as an alternative, though this remains off-label 2, 3
   • Anticoagulation should not be delayed once diagnosis is established

2. Management of Portal Hypertension Complications

   • Treat ascites and varices according to standard protocols 1
   • Monitor for hemorrhagic complications (reported in 17-50% of patients) 1

3. Mandatory Etiological Investigation

   • Myeloproliferative disorders (most common cause)
   • Hereditary thrombophilias (Factor V Leiden)
   • Acquired thrombophilias
   • Paroxysmal nocturnal hemoglobinuria
   • Autoimmune disorders
   • Local factors (tumors) 1
   • Treatment of underlying cause is fundamental for long-term management

Interventional Procedures

1. Angioplasty with Stenting

   • Indicated for patients with short and singular stenosis of hepatic veins or IVC
   • Stent placement reduces the rate of re-stenosis 1
   • More likely to be successful with a short history of thrombosis 4

2. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

   • Indicated when medical treatment and angioplasty fail 1
   • Especially important in cases of fulminant Budd-Chiari syndrome 1
   • May obviate the need for liver transplantation in severe acute liver insufficiency 5
   • Should be performed in expert centers with specialized teams 5
   • Pre-TIPS MRI with hepatospecific contrast agents is essential to detect liver nodules 5, 1
   • Post-TIPS Doppler ultrasound should be performed early and then every 6 months 1

3. Liver Transplantation

   • Indicated for patients with:
     • Failed TIPS
     • Fulminant hepatic insufficiency due to Budd-Chiari
     • Advanced cirrhosis with deteriorating liver function 1
   • Evaluation for transplantation should be initiated as soon as TIPS is indicated
   • Best results obtained in patients with thrombosis limited to the hepatic veins 1
   • Avoid delaying referral to a transplant center for patients with fulminant hepatic failure 1

Long-term Management

1. Anticoagulation

   • Long-term anticoagulation is recommended, especially for patients with underlying myeloproliferative disorders 1
   • The European Association for the Study of the Liver recommends indefinite anticoagulation 1
   • Anticoagulation should continue after TIPS placement 5
   • Consider long-term anticoagulation after transplantation, even in patients without identifiable coagulation disorders 6

2. Follow-up

   • Regular clinical and imaging follow-up after TIPS
   • Doppler ultrasound every 6 months to detect TIPS dysfunction 1
   • Monitor for development of hypervascular hepatic nodules (occurs in approximately 40% of patients) 1
   • Lifelong tracking of hepatic function is indicated as progressive hepatic damage may develop despite patent surgical shunts or TIPS 6

Special Considerations

1. Pregnancy

   • Low-molecular-weight heparin is the anticoagulant of choice in pregnant patients
   • Vitamin K antagonists are contraindicated due to risk of fetal hemorrhage and teratogenicity 1

2. Direct Oral Anticoagulants (DOACs)

   • Recent evidence suggests DOACs may be effective and safe for long-term anticoagulation in BCS 2, 3
   • In a multicenter study, 63.6% of patients achieved or maintained complete response on DOACs 2
   • DOACs appear to be at least equally effective to LMWH/VKA for anticoagulation in BCS 3
   • However, larger prospective studies are needed for confirmation

3. Thrombolysis

   • Limited role in management
   • More likely to be successful when the thrombolytic agent is locally infused and combined with a successful radiological procedure 4
   • Useful in adjunctive management when infused locally into recently thrombosed veins 4

Pitfalls and Caveats

• Medical therapy alone is rarely sufficient for long-term management 6
• TIPS and vascular stents have limited expected utility and likelihood of stent occlusion/revisions 6
• Progressive hepatic damage may develop even with patent surgical shunts or TIPS 6
• Transplantation should be considered only after excluding underlying malignancy 1
• Approximately 40% of patients develop hypervascular hepatic nodules, requiring regular follow-up 1
• Prognostic factors include extent of thrombosis, underlying cause, and presence of concomitant portal thrombosis (worse prognosis) 1