Is Budd-Chiari Syndrome a Contraindication to TIPS?
No, Budd-Chiari syndrome is not a contraindication to TIPS—in fact, TIPS is strongly recommended as a primary treatment option for patients who fail medical therapy with anticoagulation or when hepatic vein interventions are unsuccessful or not technically feasible. 1
TIPS as Primary Treatment for Budd-Chiari Syndrome
Budd-Chiari syndrome represents a specific indication for TIPS, not a contraindication. The 2020 Gut guidelines provide clear direction on when to proceed with TIPS in this population:
TIPS is recommended where patients fail to respond to medical therapy with anticoagulation or hepatic vein interventions (strong recommendation, moderate-quality evidence). 1 This represents a stepwise approach where anticoagulation is initiated first, followed by consideration of hepatic vein interventions (such as angioplasty or stenting for short-segment obstructions), and then TIPS if these measures fail. 1
TIPS can be considered where hepatic vein interventions are not technically feasible (weak recommendation, low-quality evidence). 1 This applies particularly to patients with diffuse hepatic vein thrombosis or complete occlusion of all hepatic veins where direct venous recanalization is impossible. 2
Clinical Outcomes Support TIPS Use
The evidence strongly supports TIPS efficacy in Budd-Chiari syndrome with excellent long-term outcomes:
- 5-year transplant-free survival rates of 71-78% even in high-risk patients, which substantially exceeds predicted survival without TIPS 3
- 1-year survival rates of 80-100% across multiple series 2
- Symptom resolution exceeding 70% with significant improvements in ascites, liver function, and portal hypertension-related complications 1, 4
- Primary assisted patency of 93.3% when shunt dysfunction is appropriately managed 4
Critical Management Requirements
All patients with Budd-Chiari syndrome must be managed in centers of high expertise which are either transplant centers or have formal links with a liver transplant center (strong recommendation, very low-quality evidence). 1 This is non-negotiable given the technical complexity of TIPS in this population and the need for multidisciplinary expertise in hematology, interventional radiology, and hepatology. 1
When to Avoid TIPS in Budd-Chiari Syndrome
While Budd-Chiari syndrome itself is not a contraindication, certain clinical scenarios warrant extreme caution or consideration of direct liver transplantation:
Patients with BCS-TIPS prognostic score >7 have very poor outcomes (7 out of 8 died or required transplantation) and should be considered for early liver transplantation rather than TIPS. 3 This score incorporates bilirubin, age, and INR—the specific variables that independently predict 1-year transplant-free survival. 3
Fulminant Budd-Chiari syndrome with acute liver failure carries high mortality even with TIPS (mortality rates of 50% in some series), and these patients should be evaluated for super-urgent liver transplantation. 1, 5, 6 The 2003 Journal of Hepatology study demonstrated that among 8 patients with hepatic failure, 4 died soon after TIPS from complications including liver rupture, portal vein rupture, liver failure, and pulmonary edema. 5
Patients with poor prognostic scores or those who do not respond to anticoagulation and radiological therapies should be considered for liver transplant assessment (strong recommendation, moderate-quality evidence). 1
Technical Considerations Unique to Budd-Chiari
TIPS placement in Budd-Chiari syndrome is technically more challenging than in cirrhotic portal hypertension. 1 In cases where all hepatic veins are occluded, a direct intrahepatic portocaval shunt (DIPS) may be performed as an alternative, where a tract is created by direct puncture from the IVC through the liver to the portal vein. 1 Clinical outcomes following DIPS are similar to classical TIPS. 1
In 5 out of 15 cases (33%), TIPS required a transcaval approach due to complete hepatic vein occlusion. 4 This underscores the importance of performing these procedures at experienced centers.
Common Pitfalls to Avoid
- Failing to initiate anticoagulation immediately—anticoagulation should be started as soon as Budd-Chiari syndrome is diagnosed and continued indefinitely, even after TIPS placement 7, 8
- Not screening for underlying myeloproliferative disorders—approximately 49% of Budd-Chiari cases are caused by myeloproliferative neoplasms, and the JAK2V617F mutation is found in 45% of patients 7
- Proceeding with TIPS in fulminant presentations without transplant evaluation—these patients may be better served by direct listing for transplantation 1, 5
- Using bare metal stents instead of covered stents—covered PTFE stents significantly improve primary patency rates 1, 2