The Transjugular Intrahepatic Portosystemic Shunt (TIPS) for Budd–Chiari Syndrome

  • Martin RössleEmail author


Since its introduction in 1988, the transjugular intrahepatic portosystemic shunt (TIPS) has become an important treatment for the Budd–Chiari syndrome (BCS). It almost completely replaced surgical shunt treatment and reduced the need of liver transplantation to few cases where TIPS treatment failed. The TIPS intervention is always on top of the medical treatment which consists of anticoagulation and specific treatment of an underlying hematological disease. With the advent of transcaval puncture in cases with occluded hepatic veins, TIPS can successfully be implanted in more than 95% of patients with a very low complication rate. With the use of PTFE-covered stents, long-term patency of the shunt is acceptable with revision required in 40% of patients during 2 years of follow-up and a secondary patency rate of almost 100%.

The present clinical practice guidelines of the European Association for the Study of the Liver recommend a stepwise therapeutic algorithm by order of increasing invasiveness beginning with anticoagulation, angioplasty in patients with web-like BCS, TIPS, and finally, liver transplantation. However, medical treatment is ineffective in more than 80–90% of patients. In addition, it may prolong the time of insufficient hepatic blood supply which may result in disease progression. In contrast, the TIPS leads to a rapid and effective drainage of the hepatic and splanchnic vascular beds, thus improving hepatic function and ameliorating portal hypertension. In this regard, early vascular intervention, e.g., TIPS or angioplasty in cases of short BCS, may be favorable in patients with acute and subacute disease. Survival after TIPS is excellent in both, acute and chronic BCS with a 5 and 10-year survival rate of 90% and 80%, respectively. About half of the patients die from their underlying hematological disease. The TIPS compares favorably with surgical shunt treatment and primary liver transplantation. In addition, it does not compromise later liver transplantation, a fact which justifies a strategy which places liver transplantation at the very end.


Budd–Chiari syndrome Transjugular intrahepatic portosystemic shunt TIPS Ascites Liver transplantation Portosystemic shunt 


  1. 1.
    Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC. Budd-Chiari syndrome: a review by an expert panel. J Hepatol. 2003;38:364–71.PubMedPubMedCentralGoogle Scholar
  2. 2.
    Mitchell MC, Boitnott JK, Kaufman S, Cameron JL, Maddrey WC. Budd-Chiari syndrome: etiology, diagnosis, and management. Medicine (Baltimore). 1982;61:199–218.CrossRefGoogle Scholar
  3. 3.
    Valla D. Primary Budd-Chiari syndrome. J Hepatol. 2009;50:195–203.CrossRefGoogle Scholar
  4. 4.
    EASL. Clinical practice guidelines: vascular diseases of the liver. J Hepatol. 2016;64:179–202.CrossRefGoogle Scholar
  5. 5.
    Bismuth H, Sherlock DJ. Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg. 1991;214:581–9.CrossRefGoogle Scholar
  6. 6.
    Wytte CL, Witte MH, Dumont AE, Frist J, Cole WR. Lymph protein in hepatic cirrhosis and experimental hepatic and portal venous hypertension. Ann Surg. 1968;168:567–76.CrossRefGoogle Scholar
  7. 7.
    Henderson JM, Warren WD, Millikan WJ, Galloway JR, Kawasaki S, Stahl RL, Hertzler G. Surgical options, hematologic evaluation, and pathologic changes in Budd-Chiari syndrome. Am J Surg. 1990;159:41–50.CrossRefGoogle Scholar
  8. 8.
    Orloff MJ, et al. Budd-Chiari syndrome revisited: 38 years´ experience with surgical portal decompression. J Gastrointest Surg. 2012;16:286–300.CrossRefGoogle Scholar
  9. 9.
    Darwish Murad S, et al. A prospective follow-up study on 163 patients with Budd-Chiari syndrome: results from the European network for vascular disorders of the liver (EN-vie). J Hepatol. 2007;46:S4.CrossRefGoogle Scholar
  10. 10.
    Darwish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009;151:167–75.Google Scholar
  11. 11.
    Eapen CE, Velissaris D, Heydtmann M, et al. Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome. Gut. 2006;55:878–84.CrossRefGoogle Scholar
  12. 12.
    Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology. 2006;44:1308–16.CrossRefGoogle Scholar
  13. 13.
    Seijo S, Plessier A, Hoekstra J, Dell’Era A, Mandair D, Rifai K, et al. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology. 2013;57:1962–8.CrossRefGoogle Scholar
  14. 14.
    Kohli V, Pande GK, Dev V, Reddy KS, Kaul U, Nundy S. Management of hepatic venous outflow obstruction. Lancet. 1993;342:718–22.CrossRefGoogle Scholar
  15. 15.
    Khuroo MS, Al-Suhabani H, Al-Sebayel M, et al. Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol. 2005;20:1494–502.CrossRefGoogle Scholar
  16. 16.
    Valla D, Hadengue A, El Younsi M, Azar N, Zeitoun G, Boudet M-J, Molas G, et al. Hepatic outflow block caused by short length hepatic vein stenoses. Hepatology. 1997;25:814–9.CrossRefGoogle Scholar
  17. 17.
    Rössle M, et al. The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. 2004;135:394–403.CrossRefGoogle Scholar
  18. 18.
    Kappenschneider T. The Budd-Chiari Syndrome: Change in Treatment since 1990. Dissertation, University Hospital Freiburg, Germany, 2010.Google Scholar
  19. 19.
    Garcia-Pagan JC, et al. TIPS for Budd-Chiari syndrome: long-term results and prognostics factors in 124 patients. Gastroenterology. 2008;135:808–15.CrossRefGoogle Scholar
  20. 20.
    Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol. 2011;28:69–177.Google Scholar
  21. 21.
    Kuo PC, Johnson LB, Hastings G, Pais SO, Plotkin JS, Orens JB, Howell CD, et al. Fulminant hepatic failure from the Budd-Chiari syndrome. A bridge to transplantation with transjugular intrahepatic portosystemic shunt. Transplantation. 1996;62:294–6.CrossRefGoogle Scholar
  22. 22.
    Shreshta R, Durham JD, Wachs M, Bilir BM, Kam I, Trouillot T, Everson GT. Use of transjugular intrahepatic portosystemic shunt as a bridge to transplantation in fulminant hepatic failure due to Budd-Chiari syndrome. Am J Gastroenterol. 1997;92:2304–6.Google Scholar
  23. 23.
    Gandini R, et al. Transjugular intrahepatic portosystemic shunt patency and clinical outcome in patients with Budd-Chiari syndrome: covered versus uncovered stents. Radiology. 2006;241:298–305.CrossRefGoogle Scholar
  24. 24.
    Hernandez-Guerra M, et al. PTFE-covered stents improve TIPS patency in Budd-Chiari syndrome. Hepatology. 2004;40:1197–202.CrossRefGoogle Scholar
  25. 25.
    Zeitoun G, Escolano S, Hadengue A, Azar N, El Younsi M, Mallet A, Boudet M-J, et al. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology. 1999;30:84–9.CrossRefGoogle Scholar
  26. 26.
    Darwish Murad S, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology. 2004;39:500–8.CrossRefGoogle Scholar
  27. 27.
    Rautou PE, Moucari R, Escolano S, Cazals-Hatem D, Denie C, Chagneau-Derrode C, et al. Prognostic indices for Budd-Chiari syndrome: valid for clinical studies but insufficient for individual management. Am J Gastroenterol. 2009;104:1140–6.CrossRefGoogle Scholar
  28. 28.
    Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JAC, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999;44:568–74.PubMedPubMedCentralGoogle Scholar
  29. 29.
    Maier WL, Waller RM, Sones PJ. Budd-Chiari web treated by percutaneous transluminal angioplasty. Am J Roentgenol. 1981;137:1257–8.CrossRefGoogle Scholar
  30. 30.
    Martin LG, Henderson JM, Millikan WJ Jr, Casarella WJ, Kaufman SL. Angioplasty for long-term treatment of patients with Budd-Chiari syndrome. Am J Roentgenol. 1990;154:1007–10.CrossRefGoogle Scholar
  31. 31.
    Zhang CQ, Fu LN, Xu L, et al. Long-term effect of stent placement in 115 patients with Budd-Chiari syndrome. World J Gastroenterol. 2003;9:2587–91.CrossRefGoogle Scholar
  32. 32.
    Blum U, Rössle M, Haag K, Ochs A, Blum HE, Hauenstein KH, Astinet F, et al. Budd-Chiari syndrome - technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology. 1995;197:805–11.CrossRefGoogle Scholar
  33. 33.
    Mancuso A, Fung K, Mela M, et al. TIPS for acute and chronic Budd-Chiari syndrome: a single-centre experience. J Hepatol. 2003;38:751–4.CrossRefGoogle Scholar
  34. 34.
    Perello A, Garcia-Pagan JC, Gilabert R, et al. TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. Hepatology. 2002;35:132–9.CrossRefGoogle Scholar
  35. 35.
    Rössle M. TIPS: 25 years later. J Hepatol. 2013;59:1081–93.CrossRefGoogle Scholar
  36. 36.
    Ryu RK, Durham JD, Krysl J, et al. Role of TIPS as a bridge to hepatic transplantation in Budd-Chiari syndrome. J Vasc Interv Radiol. 1999;10:799–805.CrossRefGoogle Scholar
  37. 37.
    Segev DL, et al. Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl. 2007;13:1285–94.CrossRefGoogle Scholar
  38. 38.
    Langlet P, Valla D. Is surgical portosystemic shunt the treatment of choice in Budd-Chiari syndrome? Acta Gastroenterol Belg. 2002;65:155–60.PubMedGoogle Scholar
  39. 39.
    Bachet JB, Condat B, Hagege H, et al. Long-term portosystemic shunt patency as a determinant of outcome in Budd-Chiari syndrome. J Hepatol. 2007;46:60–8.CrossRefGoogle Scholar
  40. 40.
    Panis Y, Belghiti J, Valla D, et al. Portosystemic shunt in Budd-Chiari syndrome: long-term survival and factors affecting shunt patency in 25 patients in Western countries. Surgery. 1994;115:276–81.PubMedGoogle Scholar
  41. 41.
    Ahn SS, Yellin A, Sheng FC, Colonna JO, Goldstein LI, Busuttil RW. Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management. J Vasc Surg. 1987;5:28–37.CrossRefGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Department of GastroenterologyUniversity HospitalFreiburgGermany

Personalised recommendations