What is the treatment for Budd Chiari syndrome?

Treatment of Budd-Chiari Syndrome

The treatment of Budd-Chiari syndrome requires a sequential stepwise approach, starting with anticoagulation, followed by angioplasty/stenting for eligible patients, then transjugular intrahepatic portosystemic shunt (TIPS) if medical treatment fails, and finally liver transplantation for non-responders. 1

Initial Management: Anticoagulation

• Immediate anticoagulation therapy should be initiated with low molecular weight heparin (LMWH) for 5-7 days, followed by oral vitamin K antagonists (VKA) with a target INR of 2-3 2, 1
• LMWH can be discontinued once the INR is within target range for two consecutive measurements 2
• Anticoagulation should be continued indefinitely, especially in patients with underlying myeloproliferative disorders 1
• Recent data shows bleeding complications have decreased from 50% to 17% with better management of anticoagulation during procedures and appropriate prophylaxis for portal hypertension-related bleeding 2

Emerging Alternative: Direct Oral Anticoagulants (DOACs)

• Recent evidence (2023) suggests DOACs may be effective and safe for long-term anticoagulation in BCS patients 3
• In a study of 22 patients treated with DOACs, 63.6% achieved or maintained complete response according to EASL criteria 3
• However, this remains off-label use and requires confirmation by larger prospective studies 3

Concurrent Management

• Treatment of the underlying prothrombotic cause (especially myeloproliferative disorders) should be initiated concomitantly with anticoagulation 2, 1
• Early treatment of underlying myeloproliferative disorders has shown benefits in retrospective analyses 2

Second-Line Treatment: Angioplasty and Stenting

• Indicated for patients with short and singular stenosis of hepatic veins or inferior vena cava (IVC) 1
• Most effective in patients with recent and incomplete thrombosis 2
• Stent placement reduces the rate of re-stenosis 1
• However, this approach is definitive treatment for less than 10% of Western BCS patients 2
• Caution: misplacement of stents may compromise subsequent TIPS or liver transplantation 2

Third-Line Treatment: TIPS

• Indicated when medical treatment and angioplasty fail 1
• Particularly useful in cases of fulminant BCS 1
• Has largely replaced surgical shunting as the invasive procedure of choice 4
• Clinical failure criteria for medical therapy include:
  • Persistent ascites despite diuretic therapy
  • Factor V level below 40% of normal
  • Elevated conjugated bilirubin (>15 μmol/L)
  • Portal hypertension-related bleeding
  • Recurrent bacterial infections
  • BMI <20 kg/m² 2
• Post-TIPS monitoring:
  • Early Doppler ultrasound followed by examinations every 6 months to detect thrombosis or TIPS dysfunction 1
  • Monitor for development of hypervascular hepatic nodules (occurs in ~40% of patients) 1

Fourth-Line Treatment: Liver Transplantation

• Indicated for patients with:
  • Failed TIPS
  • Fulminant hepatic insufficiency due to BCS
  • Advanced cirrhosis with deteriorating liver function 1
• Evaluation for transplantation should be initiated as soon as TIPS is indicated 1
• Best results are obtained in patients with thrombosis limited to the hepatic veins 1
• Five-year survival rates of at least 75% can be achieved with transplantation 5

Special Considerations

• Pregnancy: LMWH is the anticoagulant of choice as VKAs are contraindicated due to risk of fetal hemorrhage and teratogenicity 1
• Thrombolysis: Limited experience but may be beneficial in patients with recent and incomplete thrombosis when combined with angioplasty or stenting; however, complications can be fatal 2
• Surgical shunts: Mesocaval shunts with PTFE stent or autologous jugular vein interposition are options but have been largely replaced by TIPS 2, 4

Prognostic Factors

• Extent of thrombosis
• Underlying cause
• Presence of concomitant portal thrombosis (associated with worse prognosis) 1

Monitoring and Follow-up

• Regular clinical and imaging follow-up is crucial
• Doppler ultrasound every 6 months after TIPS
• Monitor for bleeding complications while on anticoagulation
• Assess for development of hypervascular hepatic nodules 1

With appropriate management following this stepwise approach, contemporary treatment achieves good survival rates of 87% at 1 year and 82% at 2 years 4.