Controversies in the Management of Budd–Chiari Syndrome

  • Andrea MancusoEmail author


The flowchart of Budd–Chiari Syndrome (BCS) management is not evidence-based and relies to experts’ opinion. The aim of this chapter is to enlighten the controversies about BCS management.

Guidelines about BCS management suggest following a step-wise strategy where sole medical therapy is the initial treatment, revascularization or TIPS the second step, and liver transplant the rescue therapy. However, sole medical therapy generally results in bad long-term outcome. The main debated issue of guidelines is that further intervention is suggested only when hemodynamic consequences of portal hypertension become evident. However, as a theory recently stated, liver fibrosis could be the final result of chronic micro-vascular ischemia. Consequently, in the context of BCS, impaired venous hepatic outflow could result in portal hypertension development so triggering hepatic fibrosis and liver failure through chronic liver ischemic injury. Moreover, treatment induced liver congestion relief might preserve liver function avoiding BCS complications development. Recently, early TIPS was suggested to possibly improve the outcome of BCS.

Future studies should be designed with the aim of evaluating whether the outcome of BCS could be improved with early intervention versus step-wise strategy. Furthermore, researchers should explore, using non-invasive tools, which subgroup of patients on only medical therapy would mostly benefit from early intervention.


Budd–Chiari syndrome TIPS Liver transplant Outcome 


  1. 1.
    Mancuso A. Timing of TIPS in Budd Chiari syndrome: an Italian Hepatologist’s perspective. J Transl Int Med. 2017;5(4):194–9.PubMedPubMedCentralCrossRefGoogle Scholar
  2. 2.
    DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology. 2009;49:1729–64.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 3.
    Mancuso A. Budd-Chiari syndrome management: lights and shadows. World J Hepatol. 2011;3:262–4.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 4.
    Mancuso A. An update on management of Budd-Chiari syndrome. Ann Hepatol. 2014;13(3):323–6.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    Garcia-Pagán JC, Buscarini E, Janssen HLA, et al. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol. 2016;64:179–202.CrossRefGoogle Scholar
  6. 6.
    Qi X, Han G, Guo X, De Stefano V, Xu K, Lu Z, Xu H, Mancuso A, et al. Review article: etiology of primary Budd-Chiari syndrome: difference between West and China. Aliment Pharmacol Ther. 2016;44:1152–67.PubMedCrossRefGoogle Scholar
  7. 7.
    Mancuso A. Management of hepatocellular carcinoma: Enlightening the gray zones. World J Hepatol. 2013;5:302–10.PubMedPubMedCentralCrossRefGoogle Scholar
  8. 8.
    Dang X, Li L, Li S, Wang Y, Li H, Xu S, Xu P. Studies on Budd-chiari syndrome complicated with hepatocellular carcinoma: most patients without inferior vena cava obstruction. Int J Clin Exp Med. 2015;8:9071–8.PubMedPubMedCentralGoogle Scholar
  9. 9.
    Paul SB, Shalimar, Sreenivas V, Gamanagatti SR, Sharma H, Dhamija E, Acharya SK. Incidence and risk factors of hepatocellular carcinoma in patients with hepatic venous outflow tract obstruction. Aliment Pharmacol Ther. 2015;41:961–71.PubMedCrossRefPubMedCentralGoogle Scholar
  10. 10.
    Liu FY, Wang MQ, Duan F, Fan QS, Song P, Wang Y. Hepatocellular carcinoma associated with Budd-Chiari syndrome: imaging features and transcatheter arterial chemoembolization. BMC Gastroenterol. 2013;13:105.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Park H, Yoon JY, Park KH, Kim DY, Ahn SH, Han KH, Chon CY, Park JY. Hepatocellular carcinoma in Budd-Chiari syndrome: a single center experience with long-term follow-up in South Korea. World J Gastroenterol. 2012;18:1946–52.PubMedPubMedCentralCrossRefGoogle Scholar
  12. 12.
    Gwon D 2nd, Ko GY, Yoon HK, Sung KB, Kim JH, Lee SS, Lee JM, Ohm JY, Shin JH, Song HY. Hepatocellular carcinoma associated with membranous obstruction of the inferior vena cava: incidence, characteristics, and risk factors and clinical efficacy of TACE. Radiology. 2010;254:617–26.PubMedCrossRefPubMedCentralGoogle Scholar
  13. 13.
    Moucari R, Rautou PE, Cazals-Hatem D, Geara A, Bureau C, Consigny Y, Francoz C, Denninger MH, Vilgrain V, Belghiti J, Durand F, Valla D, Plessier A. Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut. 2008;57:828–35.PubMedCrossRefPubMedCentralGoogle Scholar
  14. 14.
    Mancuso A. An update on management of Budd-Chiari syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol. 2015;27:200–3.PubMedCrossRefGoogle Scholar
  15. 15.
    Seijo S, Plessier A, Hoekstra J, et al. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology. 2013;57:1962–8.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    Rautou PE, Douarin L, Denninger MH, et al. Bleeding in patients with Budd-Chiari syndrome. J Hepatol. 2011 Jan;54(1):56–63.PubMedCrossRefGoogle Scholar
  17. 17.
    Wanless IR, Wong F, Blendis LM, Greig P, Heathcote EJ, Levy G. Hepatic and portal vein thrombosis in cirrhosis: possible role in development of parenchymal extinction and portal hypertension. Hepatology. 1995;21:1238–47.PubMedPubMedCentralGoogle Scholar
  18. 18.
    Pan S, Kleppe LS, Witt TA, Mueske CS, Simari RD. The effect of vascular smooth muscle cell-targeted expression of tissue factor pathway inhibitor in a murine model of arterial thrombosis. Thromb Haemost. 2004;92:495–502.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Mancuso A. Cirrhosis development probably arises from chronic micro-vascular ischemia. Med Hypotheses. 2014;82:243–4.CrossRefGoogle Scholar
  20. 20.
    Mancuso A. The ischemic liver cirrhosis theory and its clinical implications. Med Hypotheses. 2016;94:4–6.PubMedCrossRefPubMedCentralGoogle Scholar
  21. 21.
    Simonetto DH, Yang HY, Yin M, et al. Chronic passive venous congestion drives hepatic fibrogenesis via sinusoidal thrombosis and mechanical forces. Hepatology. 2015;61:649–59.Google Scholar
  22. 22.
    Mancuso A. The target of TIPS for Budd-Chiari syndrome should be avoiding liver transplantation. Aliment Pharmacol Ther. 2014;39:1342.PubMedCrossRefPubMedCentralGoogle Scholar
  23. 23.
    Mancuso A. TIPS for Budd-Chiari syndrome: time to anticipate treatment. Liver Int. 2014;34(7):e325.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Mancuso A. Budd-Chiari syndrome management: timing of treatment is an open issue. Hepatology. 2014;59:1213.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Plessier A, Sibert A, Consigny Y, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology. 2006;44:1308–16.PubMedCrossRefPubMedCentralGoogle Scholar
  26. 26.
    De Gottardi A, Trebicka J, Klinger C, Plessier A, Seijo S, Terziroli B, Magenta L, Semela D, Buscarini E, Langlet P, Görtzen J, Puente A, Müllhaupt B, Navascuès C, Nery F, Deltenre P, Turon F, Engelmann C, Arya R, Caca K, Peck-Radosavljevic M, Leebeek FWG, Valla D, Garcia-Pagan JC, Investigators VALDIG. Antithrombotic treatment with direct-acting oral anticoagulants in patients with splanchnic vein thrombosis and cirrhosis. Liver Int. 2017;37:694–9.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Bilbao JI, Pueyo JC, Longo JM, et al. Interventional therapeutic techniques in Budd-Chiari syndrome. Cardiovasc Intervent Radiol. 1997;20:112–9.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Fisher NC, McCafferty I, Dolapci M, et al. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999;44:568–74.PubMedPubMedCentralCrossRefGoogle Scholar
  29. 29.
    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.PubMedPubMedCentralCrossRefGoogle Scholar
  30. 30.
    Perelló A, García-Pagán 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.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    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.PubMedCrossRefGoogle Scholar
  32. 32.
    Rössle M, Olschewski M, Siegerstetter V, Berger E, Kurz K, Grandt D. The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. 2004;135:394–403.PubMedCrossRefGoogle Scholar
  33. 33.
    Mancuso A, Watkinson A, Tibballs J, Patch D, Burroughs AK. Budd-Chiari syndrome with portal, splenic, and superior mesenteric vein thrombosis treated with TIPS: who dares wins. Gut. 2003;52:438.PubMedPubMedCentralCrossRefGoogle Scholar
  34. 34.
    Darwish Murad S, Valla DC, De Groen PC, et al. Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis. Am J Gastroenterol. 2006;101:83–90.PubMedPubMedCentralCrossRefGoogle Scholar
  35. 35.
    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;135:808–15.PubMedCrossRefPubMedCentralGoogle Scholar
  36. 36.
    Qi X, Guo W, He C, et al. Transjugular intrahepatic portosystemic shunt for Budd–Chiari syndrome: techniques, indications and results on 51 Chinese patients from a single centre. Liver Int. 2014;34:1164–75.PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    Tripathi D, Macnicholas R, Kothari C, et al. Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome. Aliment Pharmacol Ther. 2014;39:864–72.PubMedCrossRefGoogle Scholar
  38. 38.
    Ringe B, Lang H, Oldhafer KJ, et al. Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. Hepatology. 1995;21:1337–44.PubMedCrossRefGoogle Scholar
  39. 39.
    Hemming AW, Langer B, Greig P, Taylor BR, Adams R, Heathcote EJ. Treatment of Budd-Chiari syndrome with portosystemic shunt or liver transplantation. Am J Surg. 1996;171:176–80; discussion 180–81.PubMedCrossRefPubMedCentralGoogle Scholar
  40. 40.
    Orloff MJ, Isenberg JI, Wheeler HO, Daily PO, Girard B. Budd-Chiari syndrome revisited: 38 years’ experience with surgical portal decompression. J Gastrointest Surg. 2012;16:286–300; discussion 300.PubMedCrossRefPubMedCentralGoogle Scholar
  41. 41.
    Zhang Y, Zhao H, Yan D, Xue H, Lau WY. Superior mesenteric vein-caval-right atrium Y shunt for treatment of Budd-Chiari syndrome with obstruction to the inferior vena cava and the hepatic veins-a study of 62 patients. J Surg Res. 2011;169:e93–9.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    Srinivas BC, Dattatreya PV, Srinivasa KH, Prabhavathi CN. Inferior vena cava obstruction: long-term results of endovascular management. Indian Heart J. 2012;64:162–9.PubMedPubMedCentralCrossRefGoogle Scholar
  43. 43.
    Halff G, Todo S, Tzakis AG, Gordon RD, Starzl TE. Liver transplantation for the Budd-Chiari syndrome. Ann Surg. 1990;211:43–9.PubMedPubMedCentralCrossRefGoogle Scholar
  44. 44.
    Rao AR, Chui AK, Gurkhan A, et al. Orthotopic liver transplantation for treatment of patients with Budd-Chiari syndrome: a single-center experience. Transplant Proc. 2000;32:2206–7.PubMedCrossRefPubMedCentralGoogle Scholar
  45. 45.
    Ulrich F, Steinmüller T, Lang M, et al. Liver transplantation in patients with advanced Budd-Chiari syndrome. Transplant Proc. 2002;34:2278.PubMedCrossRefPubMedCentralGoogle Scholar
  46. 46.
    Srinivasan P, Rela M, Prachalias A, et al. Liver transplantation for Budd-Chiari syndrome. Transplantation. 2002;73:973–7.PubMedCrossRefGoogle Scholar
  47. 47.
    Mentha G, Giostra E, Majno PE, et al. Liver transplantation for Budd-Chiari syndrome: a European study on 248 patients from 51 centres. J Hepatol. 2006;44:520–8.PubMedCrossRefGoogle Scholar
  48. 48.
    Segev DL, Nguyen GC, Locke JE, et al. Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis. Liver Transpl. 2007;13:1285–94.PubMedCrossRefGoogle Scholar
  49. 49.
    Potthoff A, Attia D, Pischke S, et al. Long-term outcome of liver transplant patients with Budd-Chiari syndrome secondary to myeloproliferative neoplasms. Liver Int. 2015;35:2042–9.PubMedCrossRefGoogle Scholar
  50. 50.
    Mancuso A. Time to resize the role of liver transplant for Budd-Chiari syndrome. Liver Int. 2015;35:2339.PubMedCrossRefPubMedCentralGoogle Scholar
  51. 51.
    Mancuso A, Martinelli L, De Carlis L, et al. A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction. World J Hepatol. 2013;27:292–5.CrossRefGoogle Scholar
  52. 52.
    Choi GS, Park JB, Jung GO, et al. Living donor liver transplantation in Budd-Chiari syndrome: a single-center experience. Transplant Proc. 2010;42:839–42.PubMedCrossRefPubMedCentralGoogle Scholar
  53. 53.
    Yoon YI, Lee SG, Moon DB, Ahn CS, Hwang S, Kim KH, Ha TY, Song GW, Jung DH, Park GC, Kim DS, Choo SJ. Surgical techniques and long-term outcomes of living-donor liver transplantation with inferior vena cava replacement using atriocaval synthetic interposition graft for Budd-Chiari syndrome. Ann Surg. 2019;269(4):e43–5.PubMedCrossRefGoogle Scholar
  54. 54.
    Darwish Murad S, Plessier A, Hernandez-Guerra M, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009;151:167–75.PubMedCrossRefPubMedCentralGoogle Scholar
  55. 55.
    Qi X, Ren W, Wang Y, Guo X, Fan D. Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies. Expert Rev Gastroenterol Hepatol. 2015;9:865–75.PubMedCrossRefGoogle Scholar
  56. 56.
    Mancuso A. Management of Budd-Chiari syndrome in children: same debated issues as in adults. Eur J Gastroenterol Hepatol. 2015;27:107–8.PubMedCrossRefPubMedCentralGoogle Scholar
  57. 57.
    Mancuso A. Outcome of Budd-Chiari syndrome in Behçet’s syndrome. Semin Arthritis Rheum. 2015;45:e1.PubMedCrossRefPubMedCentralGoogle Scholar
  58. 58.
    He F, Zhao H, Dai S, et al. Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome with diffuse occlusion of hepatic veins. Sci Rep. 2016;6:36380.PubMedPubMedCentralCrossRefGoogle Scholar
  59. 59.
    Rosenqvist K, Sheikhi R, Eriksson LG, et al. Endovascular treatment of symptomatic Budd-Chiari syndrome - in favour of early transjugular intrahepatic portosystemic shunt. Eur J Gastroenterol Hepatol. 2016;28(6):656–60.PubMedCrossRefPubMedCentralGoogle Scholar
  60. 60.
    Wang HW, Shi HN, Cheng J, Xie F, Luo YK, Tang J. Real-time shear wave elastography (SWE) assessment of short- and long-term treatment outcome in Budd-Chiari syndrome: a pilot study. PLoS One. 2018;13(5):e0197550.PubMedPubMedCentralCrossRefGoogle Scholar
  61. 61.
    Su L, Hu L, Liang P, Wu Y, Sun Q, Gao J. Clinical efficacy of spectral computed tomography for evaluating liver function in patients with Budd-Chiari syndrome. Acad Radiol. 2019;26(4):461–6.PubMedCrossRefPubMedCentralGoogle Scholar

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© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  1. 1.Medicina Interna 1ARNAS Civico - Di Cristina - BenfratelliPalermoItaly

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