Management of Budd-Chiari Syndrome
Initiate anticoagulation immediately upon diagnosis and continue indefinitely, as this is the cornerstone of therapy regardless of subsequent interventions. 1
Immediate Medical Management
Anticoagulation (First-Line Therapy)
- Start low molecular weight heparin (LMWH) for 5-7 days, then transition to oral vitamin K antagonists targeting an INR of 2-3 1
- Anticoagulation reduces clot extension and prevents new thrombotic episodes, and should be continued lifelong 1, 2
- Bleeding complications occur in approximately 17% of patients with modern management (down from 50% in older studies) due to better anticoagulation control during procedures and adequate portal hypertension prophylaxis 1
Treat Underlying Prothrombotic Conditions
- Myeloproliferative disorders are present in approximately 49% of cases and must be treated concurrently 2, 3
- Screen for inherited thrombophilias including Factor V Leiden (accounts for 25% of cases), protein C/S deficiency, antithrombin deficiency, and prothrombin G20210A mutation 4, 2
- Test for JAK2V617F mutation, calreticulin mutation, and consider bone marrow biopsy to identify myeloproliferative neoplasms 2
Portal Hypertension Management
- Manage ascites and varices using the same protocols as for cirrhosis 1
- Diuretics are appropriate for ascites management in conjunction with anticoagulation 3
Stepwise Interventional Approach
Step 1: Angioplasty/Stenting (For Focal Stenoses)
- Indicated for partial or segmental stenoses, present in 60% of patients with IVC obstruction and 25-30% with hepatic vein obstruction 1
- Most effective for short, focal stenoses where physiological drainage can be re-established 1
- Re-stenosis is common but reduced with stent placement 1
- Critical caveat: Misplaced stents may compromise subsequent TIPS or liver transplantation 1
Step 2: TIPS (After Medical Failure or When Angioplasty Fails)
- Consider TIPS when medical treatment fails or angioplasty is ineffective or anatomically impossible 1
- TIPS is specifically indicated for fulminant Budd-Chiari syndrome 1
- Perform Doppler ultrasound early post-procedure and then every 6 months to detect thrombosis or dysfunction 1, 5
- TIPS has largely replaced surgical shunting as the primary invasive procedure 3
Step 3: Liver Transplantation (For Treatment Failures)
- Reserved for patients who fail all other therapeutic options 4, 1
- Best outcomes occur when thrombosis is limited to hepatic veins and the underlying cause can be corrected by liver replacement 4, 1
- Patients with underlying malignancy or combined hepatic and portal vein thrombosis have more perioperative complications and lower long-term benefit 4
- Five-year survival rates of approximately 75% are achievable with transplantation 6
Special Clinical Scenarios
Fulminant Hepatic Failure Presentation
- Refer to transplant center immediately for critical care management 4
- Consider urgent TIPS placement 1
- Patients predicted to have little chance of spontaneous recovery should undergo transplantation as soon as possible 4
- Mortality approaches 60% in acute liver failure due to BCS despite aggressive management 7
Chronic Presentation with Intractable Ascites
- Chronic, indolent cases are more common than acute presentations 8
- Approximately 49% of patients can be managed noninvasively with anticoagulation and diuretics alone 3
- One- and two-year survival rates are 87% and 82% respectively with contemporary management 3
Diagnostic Confirmation Requirements
- Doppler ultrasound is first-line with >75% sensitivity, showing absent/tortuous hepatic veins, intrahepatic collaterals (>80% of cases), and caudate lobe enlargement 2, 5
- Direct X-ray venography is essential for precise delineation before planning interventional treatment 5
- MRI with hepato-specific contrast is useful for detecting hypervascular liver nodules prior to treatment 5