What is the treatment for Budd Chiari syndrome?

Treatment of Budd-Chiari Syndrome

The treatment of Budd-Chiari syndrome follows a stepwise therapeutic algorithm, beginning with anticoagulation therapy, followed by angioplasty/stenting when applicable, then TIPS, and finally liver transplantation for refractory cases. 1

Initial Management

• Anticoagulation therapy should be initiated as soon as possible and continued indefinitely to reduce the risk of clot extension and new thrombotic episodes 1
• Initial treatment includes low molecular weight heparin (LMWH) for at least 5-7 days, followed by oral vitamin K antagonists (VKA) with a target INR between 2 and 3 1
• LMWH can be discontinued when the INR is within target range for two consecutive measurements 1
• Treatment of the underlying prothrombotic cause (particularly myeloproliferative disorders) should be initiated concurrently 1, 2
• Management of portal hypertension complications (ascites and varices) should follow the same guidelines as for cirrhosis 1

Interventional Procedures

Angioplasty/Stenting

• Indicated for patients with partial or segmental stenoses, present in 60% of patients with IVC obstruction and 25-30% of those with hepatic vein obstruction 1
• Most effective in patients with short, focal stenoses where physiological drainage of portal and sinusoidal blood can be re-established 1
• Post-angioplasty re-stenosis is common but can be reduced with stent placement 1
• Angioplasty/stenting is the definitive treatment for less than 10% of Western BCS patients 1

Transjugular Intrahepatic Portosystemic Shunt (TIPS)

• TIPS should be considered after failure of medical treatment and when angioplasty/stenting is ineffective or impossible 1
• TIPS is also indicated in patients with fulminant Budd-Chiari syndrome 1
• Following TIPS placement, Doppler ultrasound should be performed early and then every 6 months to detect thrombosis or TIPS dysfunction 1
• TIPS has largely replaced surgical shunting as the most common invasive therapeutic procedure 3

Thrombolysis

• Limited experience with thrombolysis in BCS 1
• May be beneficial in patients with recent and incomplete thrombosis when administered locally in combination with angioplasty or stenting 1, 4
• Systemic thrombolysis has shown limited success and carries risk of potentially fatal complications 1, 4

Liver Transplantation

• Considered for patients who fail other therapeutic options 1
• Best results achieved in patients with thrombosis limited to the hepatic veins and when the underlying cause can be corrected by liver replacement 1
• Patients with underlying malignancy or combined hepatic and portal vein thrombosis have more perioperative complications and lower long-term benefit 1

Monitoring and Follow-up

• Regular monitoring of anticoagulation therapy is essential to maintain target INR 1
• Doppler ultrasound should be performed regularly to assess shunt patency after TIPS placement 1
• Screening for hepatocellular carcinoma is recommended as patients with BCS may develop hypervascular liver nodules 1

Prognosis

• With contemporary management including a stepwise approach, one-year survival rates of 87% and two-year survival rates of 82% can be achieved 3, 5
• The Rotterdam score is excellent for predicting intervention-free survival, while the BCS-TIPS prognostic index (based on INR, bilirubin, and age) is superior for predicting overall survival 5

Potential Complications

• Bleeding complications while on anticoagulation have been reported in up to 50% of patients in older studies, but more recent data shows lower rates (17%) due to better management of anticoagulation during invasive procedures and adequate prophylaxis for portal hypertension-related bleeding 1
• TIPS placement may require multiple procedures to maintain patency 6
• Misplacement of stents may compromise subsequent TIPS performance or liver transplantation 1