What is the treatment for Budd-Chiari syndrome?

Treatment of Budd-Chiari Syndrome

Treatment of Budd-Chiari syndrome follows a sequential, stepwise algorithm beginning with immediate anticoagulation, followed by angioplasty/stenting for focal stenoses, then TIPS for refractory cases, and finally liver transplantation as salvage therapy. 1, 2

Initial Medical Management

All patients must receive lifelong anticoagulation therapy initiated immediately upon diagnosis, targeting an INR of 2-3, unless major contraindications exist. 2

• Start with low molecular weight heparin (LMWH) for at least 5-7 days, then transition to oral vitamin K antagonists (VKA) with target INR 2-3 2
• Portal hypertension complications (varices, ascites) when adequately treated are NOT contraindications to anticoagulation 2
• Bleeding complications have decreased from 50% to 17% with better procedural management and portal hypertension prophylaxis 2
• Treat the underlying prothrombotic cause concurrently, particularly myeloproliferative disorders 3, 2
• Manage portal hypertension complications (ascites, varices) following the same guidelines as for cirrhosis 3, 2

Sequential Interventional Approach

Step 1: Angioplasty/Stenting (First-Line Invasive Intervention)

Angioplasty with stenting is indicated for patients with short, focal stenoses of hepatic veins or IVC. 2

• Most effective in patients with partial or segmental stenoses (present in 60% of IVC obstruction cases and 25-30% of hepatic vein obstruction) 2
• Stenting reduces re-stenosis rates compared to angioplasty alone 2
• Post-angioplasty re-stenosis is common but can be reduced with stent placement 2
• Caution: Misplacement of stents may compromise subsequent TIPS performance or liver transplantation 2

Step 2: TIPS (Second-Line Intervention)

TIPS using PTFE-covered stents should be considered after failure of medical treatment and when angioplasty/stenting is ineffective or technically impossible. 1, 2

• TIPS is also indicated in patients with fulminant Budd-Chiari syndrome 2
• Symptom resolution exceeds 70% with 5-year survival rates exceeding 70% 2
• PTFE-covered stents improve primary patency 2
• Risk of hepatic encephalopathy is up to 15% 2
• Direct intra-hepatic porto-caval shunt (DIPS) can be performed when all hepatic veins are occluded, with similar clinical outcomes to classical TIPS 2
• Following TIPS placement, perform Doppler ultrasound early and then every 6 months to detect thrombosis or TIPS dysfunction 2
• Continue anticoagulation and treatment of underlying cause after TIPS placement to enhance prognosis 1

Step 3: Surgical Shunting (Alternative When TIPS Fails or Not Feasible)

• Surgical shunting (mesocaval shunt with PTFE or autologous jugular vein) should be discussed when TIPS is not feasible or fails 2

Step 4: Liver Transplantation (Salvage Therapy)

Liver transplantation is reserved for patients who fail all other therapeutic options. 2

• Best results achieved in patients with thrombosis limited to hepatic veins and when the underlying cause can be corrected by liver replacement 2
• Patients with underlying malignancy or combined hepatic and portal vein thrombosis have more perioperative complications and lower long-term benefit 2
• BCS-TIPS score >7 consistently predicts poor outcomes and should prompt transplant consideration 2
• For patients presenting with fulminant hepatic failure, refer to transplant center immediately with consideration of urgent TIPS placement and transplantation as soon as possible 2

Specialized Center Management

All BCS patients must be managed in expert centers with hepatology, interventional radiology expertise, and formal links to liver transplant centers. 1, 2

• TIPS placement in cases of liver vascular disease should be carried out within an expert center, featuring specialized teams in radiology, interventional radiology, hemostasis, pharmacology, anesthesiology, hepatology and liver transplantation surgery 1

Monitoring and Follow-up

• Regular monitoring of anticoagulation therapy to maintain target INR 2
• Doppler ultrasound every 6 months to assess shunt patency after TIPS placement 2
• Screening for hepatocellular carcinoma as patients with BCS may develop hypervascular liver nodules 3
• Primary prophylaxis with beta-blockers or endoscopic variceal ligation for high-risk varices, following the same guidelines as for cirrhosis 3

Evidence Supporting Stepwise Approach

• A large European multicenter study of 157 patients demonstrated that this stepwise treatment approach provides good long-term survival, with 56% requiring at least one invasive intervention and overall survival of 81% at 5 years 4
• Most interventions and deaths occurred in the first 2 years after diagnosis, emphasizing the importance of early aggressive management 4
• A single-center study showed that anticoagulation combined with aggressive early radiological intervention resulted in 96% survival at 1 year and 81% at 5 years, with no patients requiring liver transplantation 5