Budd-Chiari Syndrome and Inferior Vena Cava Obstruction: The Asian Perspective

  • Qiuhe Wang
  • Guohong HanEmail author


Budd-Chiari syndrome (BCS) is a group of heterogeneous liver vascular diseases characterized by hepatic outflow obstruction, with ascites, hepatomegaly, abdominal distension, and pedal edema being common manifestations in Asian patients. A step-wise diagnostic approach aiming at minimal invasiveness is currently recommended. Ultrasonography is the first line modality, and patient with inconclusive results should receive computer tomography (CT) or magnetic resonance (MR) for further investigation. Angiography is recommended as the final modality for validation due to its invasiveness, despite that it is the standard approach for identifying the extent and morphology of obstruction. Liver biopsy, on the other hand, is not a must since the rate of sampling error can be high. Although most Asian patients are idiopathic, routine etiology work-up should be performed. In addition, the existence of portal vein thrombosis should be examined, and close screening for hepatocellular carcinoma should be instituted, since its incidence in Asian patients is relatively high.


Budd-Chiari syndrome Ultrasonography Computer tomography Magnetic resonance Angiography Stenting TIPS 



Conflict of Interest: The authors declare that they have no conflict of interest.


  1. 1.
    Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004;350:578–85.CrossRefPubMedGoogle Scholar
  2. 2.
    EASL Clinical Practice Guidelines. Vascular diseases of the liver. J Hepatol. 2016;64:179–202.CrossRefGoogle Scholar
  3. 3.
    Valla DC. Budd-Chiari syndrome and veno-occlusive disease/sinusoidal obstruction syndrome. Gut. 2008;57:1469–78.CrossRefPubMedGoogle Scholar
  4. 4.
    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.CrossRefPubMedGoogle Scholar
  5. 5.
    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.CrossRefPubMedGoogle Scholar
  6. 6.
    DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology. 2009;49:1729–64.CrossRefPubMedGoogle Scholar
  7. 7.
    Martens P, Nevens F. Budd-Chiari syndrome. United European Gastroenterol J. 2015;3:489–500.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Hadengue A, Poliquin M, Vilgrain V, Belghiti J, Degott C, Erlinger S, et al. The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases. Gastroenterology. 1994;106:1042–7.CrossRefPubMedGoogle Scholar
  9. 9.
    Amarapurkar DN, Punamiya SJ, Patel ND. Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment. World J Gastroenterol. 2008;14:278–85.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Cheng D, Xu H, Lu ZJ, Hua R, Qiu H, Du H, et al. Clinical features and etiology of Budd-Chiari syndrome in Chinese patients: a single-center study. J Gastroenterol Hepatol. 2013;28:1061–7.CrossRefPubMedGoogle Scholar
  11. 11.
    Han G, Qi X, Zhang W, He C, Yin Z, Wang J, et al. Percutaneous recanalization for Budd-Chiari syndrome: an 11-year retrospective study on patency and survival in 177 Chinese patients from a single center. Radiology. 2013;266:657–67.CrossRefPubMedGoogle Scholar
  12. 12.
    Nozari N, Vossoghinia H, Malekzadeh F, Kafami L, Mirheidari M, Malekzadeh R. Long-term outcome of Budd-Chiari syndrome: a single center experience. Middle East J Dig Dis. 2013;5:146–50.PubMedPubMedCentralGoogle Scholar
  13. 13.
    Qi X, Guo W, He C, Zhang W, Wu F, Yin Z, 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.CrossRefPubMedGoogle Scholar
  14. 14.
    Cui YF, Fu YF, Li DC, Xu H. Percutaneous recanalization for hepatic vein-type Budd-Chiari syndrome: long-term patency and survival. Hepatol Int. 2016;10:363–9.CrossRefPubMedGoogle Scholar
  15. 15.
    Ki M, Choi HY, Kim KA, Kim BH, Jang ES, Jeong SH. Incidence, prevalence and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study. Liver Int. 2016;36:1067–73.CrossRefPubMedGoogle Scholar
  16. 16.
    Kucukay F, Akdogan M, Bostanci EB, Ulus AT, Kucukay MB. Percutaneous transluminal angioplasty for complete membranous obstruction of suprahepatic inferior vena cava: long-term results. Cardiovasc Intervent Radiol. 2016;39:1392–9.CrossRefPubMedGoogle Scholar
  17. 17.
    Shalimar, Kumar A, Kedia S, Sharma H, Gamanagatti SR, Gulati GS, et al. Hepatic venous outflow tract obstruction: treatment outcomes and development of a new prognostic score. Aliment Pharmacol Ther. 2016;43:1154–67.CrossRefPubMedGoogle Scholar
  18. 18.
    Zhang CQ, Fu LN, Xu L, Zhang GQ, Jia T, Liu JY, et al. Long-term effect of stent placement in 115 patients with Budd-Chiari syndrome. World J Gastroenterol. 2003;9:2587–91.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Li WD, Yu HY, Qian AM, Rong JJ, Zhang YQ, Li XQ. Risk factors for and causes and treatment of recurrence of inferior vena cava type of Budd-Chiari syndrome after stenting in China: a retrospective analysis of a large cohort. Eur Radiol. 2017;27:1227–37.CrossRefPubMedGoogle Scholar
  20. 20.
    Valla DC. Primary Budd-Chiari syndrome. J Hepatol. 2009;50:195–203.CrossRefPubMedGoogle Scholar
  21. 21.
    Bolondi L, Gaiani S, Li Bassi S, Zironi G, Bonino F, Brunetto M, et al. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology. 1991;100:1324–31.CrossRefPubMedGoogle Scholar
  22. 22.
    Goel RM, Johnston EL, Patel KV, Wong T. Budd-Chiari syndrome: investigation, treatment and outcomes. Postgrad Med J. 2015;91:692–7.CrossRefPubMedGoogle Scholar
  23. 23.
    Bargallo X, Gilabert R, Nicolau C, Garcia-Pagan JC, Ayuso JR, Bru C. Sonography of Budd-Chiari syndrome. AJR Am J Roentgenol. 2006;187:W33–41.CrossRefPubMedGoogle Scholar
  24. 24.
    Brancatelli G, Vilgrain V, Federle MP, Hakime A, Lagalla R, Iannaccone R, et al. Budd-Chiari syndrome: spectrum of imaging findings. AJR Am J Roentgenol. 2007;188:W168–76.CrossRefPubMedGoogle Scholar
  25. 25.
    Bargallo X, Gilabert R, Nicolau C, Garcia-Pagan JC, Bosch J, Bru C. Sonography of the caudate vein: value in diagnosing Budd-Chiari syndrome. AJR Am J Roentgenol. 2003;181:1641–5.CrossRefPubMedGoogle Scholar
  26. 26.
    Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007;13:2693–6.CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Erden A. Budd-Chiari syndrome: a review of imaging findings. Eur J Radiol. 2007;61:44–56.CrossRefPubMedGoogle Scholar
  28. 28.
    Ferral H, Behrens G, Lopera J. Budd-Chiari syndrome. AJR Am J Roentgenol. 2012;199:737–45.CrossRefPubMedGoogle Scholar
  29. 29.
    Trivedi CR, Thakkar H, Sannananja B, Shah HU. Ultrasound and Doppler features of Budd-Chiari syndrome in pediatric population. Ultrasound Q. 2015;31:45–54.CrossRefPubMedGoogle Scholar
  30. 30.
    Kamath PS. Budd-Chiari syndrome: Radiologic findings. Liver Transpl. 2006;12:S21–2.CrossRefPubMedGoogle Scholar
  31. 31.
    Zimmerman MA, Cameron AM, Ghobrial RM. Budd-Chiari syndrome. Clin Liver Dis. 2006;10:259–73. viiiCrossRefPubMedGoogle Scholar
  32. 32.
    Mathieu D, Vasile N, Menu Y, Van Beers B, Lorphelin JM, Pringot J. Budd-Chiari syndrome: dynamic CT. Radiology. 1987;165:409–13.CrossRefPubMedGoogle Scholar
  33. 33.
    Yamada R, Sato M, Kawabata M, Nakatsuka H, Nakamura K, Kobayashi N. Segmental obstruction of the hepatic inferior vena cava treated by transluminal angioplasty. Radiology. 1983;149:91–6.CrossRefPubMedGoogle Scholar
  34. 34.
    Yang XL, TO C, Chen CR. Successful treatment by percutaneous balloon angioplasty of Budd-Chiari syndrome caused by membranous obstruction of inferior vena cava: 8-year follow-up study. J Am Coll Cardiol. 1996;28:1720–4.CrossRefPubMedGoogle Scholar
  35. 35.
    Park H, Yoon JY, Park KH, Kim DY, Ahn SH, Han KH, et al. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Lim JH, Park JH, Auh YH. Membranous obstruction of the inferior vena cava: comparison of findings at sonography, CT, and venography. AJR Am J Roentgenol. 1992;159:515–20.CrossRefPubMedGoogle Scholar
  37. 37.
    Gupta S, Barter S, Phillips GW, Gibson RN, Hodgson HJ. Comparison of ultrasonography, computed tomography and 99mTc liver scan in diagnosis of Budd-Chiari syndrome. Gut. 1987;28:242–7.CrossRefPubMedPubMedCentralGoogle Scholar
  38. 38.
    Camera L, Mainenti PP, Di Giacomo A, Romano M, Rispo A, Alfinito F, et al. Triphasic helical CT in Budd-Chiari syndrome: patterns of enhancement in acute, subacute and chronic disease. Clin Radiol. 2006;61:331–7.CrossRefPubMedGoogle Scholar
  39. 39.
    Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol. 2011;3:169–77.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B. Diagnosis and management of Budd Chiari syndrome: an update. Cardiovasc Intervent Radiol. 2015;38:1–12.CrossRefPubMedGoogle Scholar
  41. 41.
    Bas K, Yaprak O, Dayangac M, Ulusoy OL, Dogusoy GB, Yuzer Y, et al. Living-donor liver transplant in 3 patients with Budd-Chiari syndrome: case report. Exp Clin Transplant. 2012;10:172–5.CrossRefPubMedGoogle Scholar
  42. 42.
    Heller MT, Hattoum A. Imaging of acute conditions affecting the hepatic vasculature. Emerg Radiol. 2012;19:329–39.CrossRefPubMedGoogle Scholar
  43. 43.
    Jang HJ, Khalili K, Yu H, Kim TK. Perfusion and parenchymal changes related to vascular alterations of the liver. Abdom Imaging. 2012;37:404–21.CrossRefPubMedGoogle Scholar
  44. 44.
    Patil P, Deshmukh H, Popat B, Rathod K. Spectrum of imaging in Budd Chiari syndrome. J Med Imaging Radiat Oncol. 2012;56:75–83.CrossRefPubMedGoogle Scholar
  45. 45.
    Noone TC, Semelka RC, Siegelman ES, Balci NC, Hussain SM, Kim PN, et al. Budd-Chiari syndrome: spectrum of appearances of acute, subacute, and chronic disease with magnetic resonance imaging. J Magn Reson Imaging. 2000;11:44–50.CrossRefPubMedGoogle Scholar
  46. 46.
    Brancatelli G, Federle MP, Grazioli L, Golfieri R, Lencioni R. Large regenerative nodules in Budd-Chiari syndrome and other vascular disorders of the liver: CT and MR imaging findings with clinicopathologic correlation. AJR Am J Roentgenol. 2002;178:877–83.CrossRefPubMedGoogle Scholar
  47. 47.
    McKusick MA. Imaging findings in Budd-Chiari syndrome. Liver Transpl. 2001;7:743–4.CrossRefPubMedGoogle Scholar
  48. 48.
    Maetani Y, Itoh K, Egawa H, Haga H, Sakurai T, Nishida N, et al. Benign hepatic nodules in Budd-Chiari syndrome: radiologic-pathologic correlation with emphasis on the central scar. AJR Am J Roentgenol. 2002;178:869–75.CrossRefPubMedGoogle Scholar
  49. 49.
    de Franchis R. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: stratifying risk and individualizing care for portal hypertension. J Hepatol. 2015;63:743–52.CrossRefPubMedGoogle Scholar
  50. 50.
    Klein AS, Molmenti EP. Surgical treatment of Budd-Chiari syndrome. Liver Transpl. 2003;9:891–6.CrossRefPubMedGoogle Scholar
  51. 51.
    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.CrossRefPubMedGoogle Scholar
  52. 52.
    Plessier A, Valla DC. Budd-Chiari syndrome. Semin Liver Dis. 2008;28:259–69.CrossRefPubMedGoogle Scholar
  53. 53.
    Tang TJ, Batts KP, de Groen PC, van Hoek B, Haagsma EB, Hop WC, et al. The prognostic value of histology in the assessment of patients with Budd-Chiari syndrome. J Hepatol. 2001;35:338–43.CrossRefPubMedGoogle Scholar
  54. 54.
    Budd G. Diseases of the liver. Philadelphia: Lea & Blanchard; 1846.Google Scholar
  55. 55.
    Langlet P, Escolano S, Valla D, Coste-Zeitoun D, Denie C, Mallet A, et al. Clinicopathological forms and prognostic index in Budd-Chiari syndrome. J Hepatol. 2003;39:496–501.CrossRefPubMedGoogle Scholar
  56. 56.
    Ludwig J, Hashimoto E, McGill DB, van Heerden JA. Classification of hepatic venous outflow obstruction: ambiguous terminology of the Budd-Chiari syndrome. Mayo Clin Proc. 1990;65:51–5.CrossRefPubMedGoogle Scholar
  57. 57.
    Valla D, Hadengue A, el Younsi M, Azar N, Zeitoun G, Boudet MJ, et al. Hepatic venous outflow block caused by short-length hepatic vein stenoses. Hepatology. 1997;25:814–9.CrossRefPubMedGoogle Scholar
  58. 58.
    Kreel L, Freston JW, Clain D. Vascular radiology in the Budd-Chiari syndrome. Br J Radiol. 1967;40:755–9.CrossRefPubMedGoogle Scholar
  59. 59.
    Menu Y, Alison D, Lorphelin JM, Valla D, Belghiti J, Nahum H. Budd-Chiari syndrome: US evaluation. Radiology. 1985;157:761–4.CrossRefPubMedGoogle Scholar
  60. 60.
    Qi X, Guo X, Fan D. Difference in Budd-Chiari syndrome between the West and China. Hepatology. 2015;62:656.CrossRefPubMedGoogle Scholar
  61. 61.
    De Stefano V, Qi X, Betti S, Rossi E. Splanchnic vein thrombosis and myeloproliferative neoplasms: molecular-driven diagnosis and long-term treatment. Thromb Haemost. 2016;115:240–9.CrossRefPubMedGoogle Scholar
  62. 62.
    Qi X, Wu F, Ren W, He C, Yin Z, Niu J, et al. Thrombotic risk factors in Chinese Budd-Chiari syndrome patients. An observational study with a systematic review of the literature. Thromb Haemost. 2013;109:878–84.CrossRefPubMedGoogle Scholar
  63. 63.
    Augustin S, Pons M, Santos B, Ventura M, Genescà J. Portal hypertension VI: Proceedings of the sixth Baveno consensus workshop: stratifying risk and individualizing care. Switzerland: Springer International Publishing; 2016.Google Scholar
  64. 64.
    Bosy-Westphal A, Ruschmeyer M, Czech N, Oehler G, Hinrichsen H, Plauth M, et al. Determinants of hyperhomocysteinemia in patients with chronic liver disease and after orthotopic liver transplantation. Am J Clin Nutr. 2003;77:1269–77.CrossRefPubMedGoogle Scholar
  65. 65.
    Frederiksen J, Juul K, Grande P, Jensen GB, Schroeder TV, Tybjaerg-Hansen A, et al. Methylenetetrahydrofolate reductase polymorphism (C677T), hyperhomocysteinemia, and risk of ischemic cardiovascular disease and venous thromboembolism: prospective and case-control studies from the Copenhagen City Heart Study. Blood. 2004;104:3046–51.CrossRefPubMedGoogle Scholar
  66. 66.
    Poisson J, Plessier A, Kiladjian JJ, Turon F, Cassinat B, Andreoli A, et al. Selective testing for calreticulin gene mutations in patients with splanchnic vein thrombosis: a prospective cohort study. J Hepatol. 2017;67(3):501–7.CrossRefPubMedGoogle Scholar
  67. 67.
    Zhang P, Ma H, Min Q, Zu M, Lu Z. CALR mutations in Chinese Budd-Chiari syndrome patients. Eur J Gastroenterol Hepatol. 2016;28:361–2.CrossRefPubMedGoogle Scholar
  68. 68.
    Rautou PE, Moucari R, Cazals-Hatem D, Escolano S, Denie C, Douarin L, et al. Levels and initial course of serum alanine aminotransferase can predict outcome of patients with Budd-Chiari syndrome. Clin Gastroenterol Hepatol. 2009;7:1230–5.CrossRefPubMedGoogle Scholar
  69. 69.
    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.CrossRefPubMedGoogle Scholar
  70. 70.
    Malinchoc M, Kamath PS, Gordon FD, Peine CJ, Rank J, ter Borg PC. A model to predict poor survival in patients undergoing transjugular intrahepatic portosystemic shunts. Hepatology. 2000;31:864–71.CrossRefPubMedGoogle Scholar
  71. 71.
    Zeitoun G, Escolano S, Hadengue A, Azar N, El Younsi M, Mallet A, et al. Outcome of Budd-Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting. Hepatology. 1999;30:84–9.CrossRefPubMedGoogle Scholar
  72. 72.
    Darwish Murad S, Valla DC, de Groen PC, Zeitoun G, Hopmans JA, Haagsma EB, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology. 2004;39:500–8.CrossRefPubMedGoogle Scholar
  73. 73.
    Mahmoud AE, Helmy AS, Billingham L, Elias E. Poor prognosis and limited therapeutic options in patients with Budd-Chiari syndrome and portal venous system thrombosis. Eur J Gastroenterol Hepatol. 1997;9:485–9.CrossRefPubMedGoogle Scholar
  74. 74.
    Watanabe H, Shinzawa H, Saito T, Ishibashi M, Shirahata N, Miyano S, et al. Successful emergency treatment with a transjugular intrahepatic portosystemic shunt for life-threatening Budd-Chiari syndrome with portal thrombotic obstruction. Hepato-Gastroenterology. 2000;47:839–41.PubMedGoogle Scholar
  75. 75.
    Darwish Murad S, Valla DC, de Groen PC, Zeitoun G, Haagsma EB, Kuipers EJ, et al. Pathogenesis and treatment of Budd-Chiari syndrome combined with portal vein thrombosis. Am J Gastroenterol. 2006;101:83–90.CrossRefPubMedGoogle Scholar
  76. 76.
    Ganger DR, Klapman JB, McDonald V, Matalon TA, Kaur S, Rosenblate H, et al. Transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome or portal vein thrombosis: review of indications and problems. Am J Gastroenterol. 1999;94:603–8.PubMedGoogle Scholar
  77. 77.
    Dilawari JB, Bambery P, Chawla Y, Kaur U, Bhusnurmath SR, Malhotra HS, et al. Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine (Baltimore). 1994;73:21–36.CrossRefGoogle Scholar
  78. 78.
    Paul SB, Shalimar, Sreenivas V, Gamanagatti SR, Sharma H, Dhamija E, et al. Incidence and risk factors of hepatocellular carcinoma in patients with hepatic venous outflow tract obstruction. Aliment Pharmacol Ther. 2015;41:961–71.CrossRefPubMedGoogle Scholar
  79. 79.
    Franchis R. Portal hypertension VI: Proceedings of the sixth Baveno consensus workshop: stratifying risk and individualizing care. Switzerland: Springer International Publishing; 2016.Google Scholar
  80. 80.
    Ren W, Qi X, Yang Z, Han G, Fan D. Prevalence and risk factors of hepatocellular carcinoma in Budd-Chiari syndrome: a systematic review. Eur J Gastroenterol Hepatol. 2013;25:830–41.CrossRefPubMedGoogle Scholar
  81. 81.
    Rector WG Jr, Xu YH, Goldstein L, Peters RL, Reynolds TB. Membranous obstruction of the inferior vena cava in the United States. Medicine (Baltimore). 1985;64:134–43.CrossRefGoogle Scholar
  82. 82.
    Moucari R, Rautou PE, Cazals-Hatem D, Geara A, Bureau C, Consigny Y, et al. Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors. Gut. 2008;57:828–35.CrossRefPubMedGoogle Scholar
  83. 83.
    Shrestha SM. Liver cirrhosis in hepatic vena cava syndrome (or membranous obstruction of inferior vena cava). World J Hepatol. 2015;7:874–84.CrossRefPubMedPubMedCentralGoogle Scholar
  84. 84.
    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.CrossRefPubMedGoogle Scholar

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Authors and Affiliations

  1. 1.Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Diseases and Xijing Hospital of Digestive DiseasesFourth Military Medical UniversityXi’anChina

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