Liver and Bile
Dig Liver Dis. 2025;57(10):1958-1964
Long-term outcomes of transjugular intrahepatic portosystemic shunt in Budd-Chiari syndrome: A multicenter study
Background: Transjugular intrahepatic portosystemic shunt (TIPS) is an important therapeutic option for Budd-Chiari syndrome (BCS), but long-term data are limited.
Aim: Evaluate response rates, long-term outcomes, complications, and predictors of mortality, new decompensation, hepatic encephalopathy (HE), and restenosis after TIPS.
Methods: Retrospective analysis of symptomatic BCS patients who underwent TIPS at three centers in India (2010–2025) from a prospectively maintained database. The authors evaluated response rates, new decompensations (HE, variceal bleed, ascites), restenosis and survival outcomes and their predictors.
Results: Among 318 patients (mean age 29.4 ± 9.8 years, 50.6% males, median follow-up 4.4 years), 244 (76.7%) had a clinical response to TIPS; non-response (23.3%) was mainly persistent ascites (two re-bleeds). Of 282 with ascites at presentation, complete/partial resolution at 3-months was 75.2%/17.0%. New decompensation developed in 32.7%, HE in 14.8%, and restenosis in 33.6%. Transplant-free survival at 1, 5, and 10 years were 95.5%, 87.4%, and 78.4%, respectively. Predictors of mortality included non-response to TIPS, bilirubin, albumin, and HE at 3 months. Age, non-response to TIPS, creatinine, and albumin predicted new decompensation. Post-TIPS complications occurred in 8.2%.
Conclusion: TIPS is a safe, effective intervention for symptomatic BCS, leading to high response rates and long-term survival. Non-response to TIPS and liver function identifies high-risk patients who should be evaluated for transplantation.
DOI: 10.1016/j.dld.2025.06.026
PD Dr. Michael Schultheiß
Head of the Interdisciplinary Ultrasound Center and Clinical Head of the TIPS Section, University Medical Center Freiburg, Department of Internal Medicine II, Hugstetter Str. 55, 79106 Freiburg, Germany
Budd-Chiari-Syndrome and TIPS: Nothing new!
The study by Swaroop et al. on Budd–Chiari syndrome (BCS) is a retrospective analysis without any substantial new insights. Nevertheless, it provides a welcome opportunity to address the topic of BCS. With an incidence of fewer than 1 case per 1,000,000 persons per year, BCS is a rare disease and therefore an uncommon condition even for hepatologists. This rarity likely contributes to the persistent “uncertainty” regarding the therapeutic algorithm. Given the young age of the patients and the often severe, potentially life-threatening clinical course, a more focused and systematic approach to BCS is warranted to support rational well-informed treatment decisions.
Today, the use of a transjugular intrahepatic portosystemic shunt (TIPS) is a clearly established standard of care in BCS. This insight is supported in part by work from the Freiburg TIPS group led by Prof. Martin Rössle in the 1990s. Interestingly, data published both by Rössle et al. in 2004 (1) from the Freiburg cohort and by Garcia-Pagán et al. in 2008 (2) from a multicenter European cohort demonstrate survival rates that are largely comparable with those presented by Swaroop et al. Transplant-free 1-year survival was 93% in Rössle, 88% in Garcia-Pagán, and 95.5% in Swaroop et al. Five-year survival was 74%, 78%, and 87.4% respectively, and 10-year survival was 69% in Garcia-Pagán and 78.4% in Swaroop et al.
Rates of stent thrombosis or need for revision also remain relatively high—even following the introduction of covered TIPS stents: 54% in Rössle et al. (23% covered stents), 41% in Garcia-Pagán et al. (51% covered stents), and 33.6% in Swaroop et al.
However, comparison of the studies also reveals several striking differences, which may be explained by the long time interval between the studies, as well as by differences between patient cohorts. As mentioned above, an important distinction is the proportion of covered TIPS stents used. In the European cohorts, coagulopathies are typically reported in 70% to 80% of patients, which may help explain the high rates of TIPS stenosis, dysfunction, and subsequent revision. In contrast, the Indian cohort studied by Swaroop et al. included only about 28% of patients with a coagulation disorder.
The introduction of TIPS has fundamentally revolutionized the treatment of BCS. Whereas long-term survival among untreated BCS patients was previously less than 10%, contemporary data indicate that 70% to 80% of patients are still alive 10 years after initial diagnosis. Nevertheless, we need to do better. The current study suggests that survival has improved only marginally compared with the first TIPS studies published more than 20 years ago. Ten-year mortality in the Swaroop et al. cohort is still greater than 20%. From my perspective, this is essentially a catastrophe for a patient cohort with a median age of 29 years.
Accordingly, the timing of TIPS placement must therefore continue to be discussed in the future. The updated European Association for the Study of the Liver (EASL) guidelines continue to recommend a stepwise approach with anticoagulation, angioplasty, TIPS, and finally liver transplant (3). But how long can and should we wait before TIPS placement? Several findings—including those from the study by Swaroop et al.—argue in favor of early TIPS placement:
– Hepatic encephalopathy in BCS (15% in this study) is often attributed to hypoxic liver failure and may be preventable with timely TIPS.
– Failure of TIPS therapy is associated with worse outcomes.
– Two-thirds of BCS patients ultimately require TIPS despite initial anticoagulation or angioplasty (3).
The logical consequence would therefore be early TIPS placement in BCS to allow prompt evaluation for liver transplant if treatment fails.
References:
(1) Doi: 10.1016/j.surg.2003.09.005
(2) Doi: 10.1053/j.gastro.2008.05.051
(3) Doi: 10.1016/j.jhep.2025.08.001