Long-term efficacy of Transjugular Intrahepatic Portosystemic Shunt treatment for Budd-Chiari Syndrome

Stavros Spiliοpoulos, Charalampos Lalenis, Chrysostomos Konstantos, Konstantinos Palialexis, Maria Tsitskari, Lazaros Reppas, Elias Brountzos


Purpose: To investigate long-term efficacy of transjugular intrahepatic porto-systemic shunt (TIPS) creation for the management of symptomatic Budd Chiari Syndrome (BCS) refractory to drug therapy.

Material and methods: This is a retrospective, single-centre analysis of 27 consecutive patients (17 female and 10 male patients; mean age: 50.8 ± 15.0 years) who underwent TIPS, between July 2003 and June 2016, due to symptomatic BCS not responding to anticoagulation therapy. Model for end-stage liver disease (MELD) score, BCS-TIPS prognostic index (BSC-TIPS PI) scores and procedural details were recorded. Primary outcome measure was orthotopic liver transplant (OLT)-free survival. Secondary outcome measures included primary patency (PP) and reintervention-free interval as well as the identification of factors influencing outcomes.

Results: Mean time follow-up was 46.5 ± 38.7 months (range 1-139). Mean MELD and BSC-TIPS PI scores were 13.8 ± 4.9 (range 6-25) and 4.9 ± 1.3 (range: 3.25 to 8.48) respectively. According to Kaplan-Meier survival analysis, estimated OLT-free survival rates were 96.3%, 96.3%, 82.5% at 2, 5 and 10 years follow up respectively. PP was 77.4%, 55.3% and 26.3% and reintervention-free interval was 80.4%, 57.4% and 30.8% at 1, 2 and 8 years follow up, respectively. Univariate subgroup analysis demonstrated that stent grafts were correlated with increased survival (HR: 0.0045; 95% CI 0.00003 to 0.701; p=0.035) and PP (HR: 0.36; 95% CI 2.503 to 3.053; p=0.03).

Conclusions: TIPS achieved high long-term OLT-free survival and satisfactory reintervention rates in patients with symptomatic BCS refractory to anticoagulation. Stent graft use was correlated with increased survival and primary patency.


Budd-Chiari syndrome; hepatic veins occlusion, Transjugular Intrahepatic Portosystemic Shunt (TIPS); orthotopic liver transplantation, stent graft

Full Text:



Parker RGF. Occlusion of the hepatic veins in man. Medicine 1959; 38: 369-402.

Budd G. On diseases of the liver. John Churchill, London 1845, pp 135. Brit Lib. 000518193.

Chiari H. Erfahrungen über Infarktbildungen in der Leber des Menschen. Zeitschrift für Heilkunde. Prague 1898, pp 475-512.

Janssen HL, Garcia-Pagan JC, Elias, E et al. Budd-Chiari syndrome: A review by an expert panel. J Hepatol 2003; 38: 364-371.

DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology 2009; 49: 1729-1764.

Shin N, Kim YH, Xu H. et al. Redefining Budd-Chiari syndrome: A systematic review. World J Hepatol 2016; 8(16): 691-702.

Qi X, Zhang C, Han G, et al. Prevalence of the JAK2V617F mutation in Chinese patients with Budd-Chiari syndrome and portal vein thrombosis: a prospective study. J Gastroenterol Hepatol 2012; 27: 1036-1043.

Rossle M, Olschewski M, Siegerstetter V, et al. The Budd-Chiari syndrome: Outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery 2004; 135(4): 394-403.

Lopez RR, Benner KG, Hall L, et al. Expandable venous stents for treatment of the Budd-Chiari Syndrome. Gastroenterology 1991; 100: 1435-1441.

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(10): 1494-1502.

Plessier A, Sibert A, Consigny Y, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd–Chiari syndrome. Hepatology 2006; 44: 1308-1316.

Bismuth H, Sherlock DJ. Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg 1991; 214: 581-589.

Henderson JM, Warren WD, Millikan WJ, et al. Surgical options, hematologic evaluation, and pathologic changes in Budd-Chiari syndrome. Am J Surg 1990; 159: 41-50.

Garcia-Pagán JC, Heydtmann M, Raffa S, et al. TIPS for Budd-Chiari syndrome: Long-term results and prognostics factors in 124 patients. Gastroenterology 2008; 135(3): 808-815.

Ryu RK, DurhamJD, Krysl J, et al. Role of TIPS as a bridge to hepatic transplantation in Budd-Chiari syndrome. J Vasc Interv Radiol 1999; 10(6): 799-805.

Fitsiori K, Tsitskari M, Kelekis A, et al. Transjugular Intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome patients: Results from a single center. Cardiovasc Intervent Radiol 2014; 37: 691-697.

de Franchis R. Evolving consensus in portal hypertension report of the Baveno IV Consensus Workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol 2005; 43: 167-217.

Haskal ZJ, Duszak R Jr, Furth EE. Transjugular intrahepatic transcaval portosystemic shunt: The gun-sight approach. J Vasc Interv Radiol 1996; 7: 139-142.

Kanterman RY, Darcy MD, Middleton WD, et al. Doppler sonography findings associated with transjugular intrahepatic portosystemic shunt malfunction. AJR Am J Roentgenol 1997; 168 (2): 467-472.

Yamada R, Sato M, Kawabata M, et al. Segmental obstruction of the hepatic inferior vena cava treated by transluminal angioplasty. Radiology 1983; 149: 91-94.

Peltzer MY, Ring EJ, LaBerge JM. Treatment of Budd-Chiari syndrome with a transjugular intrahepatic portosystemic shunt. J Vasc Interv Radiol 1993; 4: 263-267.

Ochs A, Sellinger M, Haag K, et al. Transjugular intrahepatic portosystemic stent-shunt (TIPS) in the treatment of Budd-Chiari syndrome. J Hepatol 1993; 18: 217-225.

Moreno GE, Garcia GI, Gomez SR, et al. Liver transplantation in patients with thrombosis of the portal, splenic or superior mesenteric vein. Br J Surg 1993; 80: 81-85.

Attwell A, Ludkowski M, Nash R, et al. Treatment of Budd-Chiari syndrome in a liver transplant unit, the role of transjugular intrahepatic porto-systemic shunt and liver transplantation. Aliment Pharmacol Ther 2004; 20: 867-873.

Ganger DR, Klapman JB, McDonald V, et al. Transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome or portal vein thrombosis. Am J Gastroenterol 1999; 94: 603-608.

Tsetis D, Kehagias E, Samonakis D, et al. Percutaneous rheolytic mechanical thrombectomy in thrombosed direct intrahepatic portosystemic shunt: Report of two cases. Interv Med Appl Sci 2015; 7(4): 171-175.

Bettinger D, Schultheiss M, Boettler T, et al. Procedural and shunt-related complications and mortality of the transjugular intrahepatic portosystemic shunt (TIPSS). Aliment Pharmacol Ther 2016; 44(10): 1051-1061.

Copelan A, Kapoor B, Sands M. Transjugular intrahepatic portosystemic shunt: Indications, contraindications, and patient work-up. Semin Intervent Radiol 2014; 31: 235-242.

Salerno F, Merli M, Cazzaniga M, et al. MELD score is better than Child-Pugh score in predicting 3-month survival of patients undergoing transjugular intrahepatic portosystemic shunt. J Hepatol 2002; 36(4): 494-500.

Fanelli F. The evolution of transjugular intrahepatic portosystemic shunt: Tips. ISRN Hepatol 2014; 2014: 762096.

Rautou PE, Moucari R, Escolano S, et al. Prognostic indices for Budd-Chiari syndrome: Valid for clinical studies but insufficient for individual management. Am J Gastroenterol 2009; 104(5): 1140-1146.

Qi X, Yang M, Fan D, et al. Transjugular intrahepatic portosystemic shunt in the treatment of Budd-Chiari syndrome: A critical review of literatures. Scand J Gastroenterol 2013; 48(7): 771-784.

Qi X, Tian Y, Zhang W, et al. Covered versus bare stents for transjugular intrahepatic portosystemic shunt: an updated meta-analysis of randomized controlled trials. Therap Adv Gastroenterol 2017; 10(1): 32-41.

Hayek G, Ronot M, Plessier A, et al. Long-term outcome and analysis of dysfunction of transjugular intrahepatic portosystemic shunt placement in chronic primary Budd-Chiari Syndrome. Radiology 2017; 283(1): 280-292.


  • There are currently no refbacks.