Advances and Challenges in Budd-Chiari Syndrome Management: A Review of the Literature
DOI:
https://doi.org/10.24871/2722026173-181Keywords:
Budd-Chiari syndrome (BCS), Doppler ultrasonography (US), Hepatic Venous Outflow ObstructionAbstract
Budd-Chiari syndrome (BCS) is a medical condition caused by a partial or complete obstruction of hepatic venous outflow. Epidemiologically, BCS was considered rare, and its infrequent diagnosis was attributed to limited diagnostic tools and low clinical awareness. Therefore, this review aimed to enhance clinicians' awareness, from accurate diagnosis to appropriate management of patients with BCS. The symptoms experienced by patients ranged from asymptomatic cases to fulminant forms of the disease. Imaging methods, such as Doppler ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI), were often used to establish the diagnosis. The primary treatment for BCS included anticoagulants, typically warfarin or low-molecular-weight heparin (LMWH). Depending on the patient's clinical status, more invasive therapeutic interventions could also be necessary.
References
Gavriilidis P, Marangoni G, Ahmad J, Azoulay D. State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review. J Clin Med Res. 2022;14(4):147–57. doi:10.14740/jocmr4724.
Shukla A, Shreshtha A, Mukund A, Bihari C, Eapen CE, Han G, et al. Budd-Chiari syndrome: consensus guidance of the Asian Pacific Association for the study of the liver (APASL). Hepatol Int. 2021;15(3):531–67. doi:10.1007/s12072-021-10189-4. PubMed PMID: 34240318.
Garcia-Pagán JC, Valla DC. Primary Budd–Chiari Syndrome. New England Journal of Medicine. 2023;388(14):1307–16. doi:10.1056/nejmra2207738. PubMed PMID: 37018494.
Ageno W, Dentali F, Pomero F, Fenoglio L, Squizzato A, Pagani G, et al. Incidence rates and case fatality rates of portal vein thrombosis and Budd-Chiari syndrome. Thromb Haemost. 2017;117(4):794–800. doi:10.1160/TH16-10-0781. PubMed PMID: 28180235.
Ollivier-Hourmand I, Allaire M, Goutte N, Morello R, Chagneau-Derrode C, Goria O, et al. The epidemiology of Budd–Chiari syndrome in France. Digestive and Liver Disease. 2018;50(9):931–7. doi:10.1016/j.dld.2018.04.004. PubMed PMID: 29803757.
Li Y, De Stefano V, Li H, Zheng K, Bai Z, Guo X, et al. Epidemiology of Budd-Chiari syndrome: A systematic review and meta-analysis. Clin Res Hepatol Gastroenterol. 2019;43(4):468–74. doi:10.1016/j.clinre.2018.10.014. PubMed PMID: 30528513.
Khan F, Armstrong MJ, Mehrzad H, Chen F, Neil D, Brown R, et al. Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome. Aliment Pharmacol Ther. 2019;49(7):840–63. doi:10.1111/apt.15149. PubMed PMID: 30828850.
Liu L, Qi XS, Zhao Y, Chen H, Meng XC, Han GH. Budd-Chiari syndrome: current perspectives and controversies. PubMed PMID: 27467004.
Afredj N, Guessab N, Nani A, Faraoun SA, Cheikh IO, Kerbouche R, et al. Aetiological factors of Budd-Chiari syndrome in Algeria. World J Hepatol. 2015;7(6):903–9. doi:10.4254/wjh.v7.i6.903.
Noone TC, Semelka RC, Siegelman ES, Cem Balci N, 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. doi:10.1002/(SICI)1522-2586(200001)11:1<44::AID-JMRI6>3.0.CO;2-O. PubMed PMID: 10676619.
Narayanan Menon KV, Shah V, Kamath PS. The Budd-Chiari Syndrome. N Engl J Med. 2004;350(6):578–85. doi:10.1056/NEJMra020282. PubMed PMID: 14762185.
Montes López AK, Garcia Rueda JE, Herrera Correa D, Restrepo Gutiérrez JC. Pathophysiology of Primary Budd-Chiari Syndrome: A Narrative Review. Cureus. 2025;17(11):e96539. doi:10.7759/cureus.96539.
Rautou PE, Moga L, Hernandez-Gea V, Ageno W, Darwish Murad S, Garcia-Pagan JC, et al. EASL Clinical Practice Guidelines on vascular diseases of the liver. J Hepatol. 2026;84(2):399–456. doi:10.1016/j.jhep.2025.08.001.
Fu Y, Li F, Jiang L, Pei Q, Qin W, Li Z. Budd-Chiari Syndrome: From Clinical to Ultrasonic Diagnosis. Journal of Biomedical Research & Environmental Sciences. 2022;3(12):1363–6. doi:10.37871/jbres1604.
Hitawala AA, Goosenberg E. Budd-Chiari Syndrome [Internet]. StatPearls [Internet]; 2025 [cited 2026 Jan 27]. Available from: https://pubmed.ncbi.nlm.nih.gov/32644367/. PubMed PMID: 32644367.
Bansal V, Gupta P, Sinha S, Dhaka N, Kalra N, Vijayvergiya R. Budd-Chiari syndrome: imaging review. Br J Radiol. 2018;91:20180441. doi:10.1259/bjr.20180441.
Van Wettere M, Purcell Y, Bruno O, Payancé A, Plessier A, Rautou PE, et al. Low specificity of washout to diagnose hepatocellular carcinoma in nodules showing arterial hyperenhancement in patients with Budd-Chiari syndrome. J Hepatol. 2019;70(6):1123–32. doi:10.1016/j.jhep.2019.01.009. PubMed PMID: 30654065.
Porrello G, Mamone G, Miraglia R. Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives. Diagnostics. 2023. doi:10.3390/diagnostics13132256.
Gupta P, Bansal V, Kumar-M P, Sinha S, Samanta J, Mandavdhare H, et al. Diagnostic accuracy of Doppler ultrasound, CT and MRI in Budd-Chiari syndrome: systematic review and meta-analysis. Br J Radiol. 2020;93:20190847. doi:10.1259/bjr.20190847.
Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An Update on the Management of Budd–Chiari Syndrome. Digestive Diseases and Sciences. 2021:1780–90. doi:10.1007/s10620-020-06485-y. PubMed PMID: 32691382.
Quaglia A. Histopathology of Budd–Chiari Syndrome. Diagnostics. 2023. doi:10.3390/diagnostics13152487.
Wu X, Liang L, Liu J. Effect of Direct Oral Anticoagulants Versus Traditional Anticoagulation in Budd-Chiari Syndrome. Clinical and Applied Thrombosis/Hemostasis. 2025;31:10760296251384904. doi:10.1177/10760296251384904. PubMed PMID: 41026791.
Semmler G, Lindorfer A, Schäfer B, Bartl S, Hametner-Schreil S, Gensluckner S, et al. Outcome of Budd-Chiari Syndrome Patients Treated With Direct Oral Anticoagulants: An Austrian Multicenter Study. Clinical Gastroenterology and Hepatology. 2023;21(4):978–987.e2. doi:10.1016/j.cgh.2022.04.024. PubMed PMID: 35533994.
Amin N, Jafry B, Daglilar E, Kamran A. Safety of DOACs in Budd-Chiari syndrome: A real-world comparison from the TriNetX Global Collaborative Network. Journal of Clinical Oncology. 2025;43(16_suppl). doi:10.1200/JCO.2025.43.16_suppl.e23289.
Miceli G, Ciaccio AM, Tuttolomondo A. Challenges and Opportunities of Direct Oral Anticoagulant (DOAC) Therapy in Complex Clinical Scenarios: A Comprehensive Review and Practical Guide. Journal of Clinical Medicine. 2025. doi:10.3390/jcm14092914.
Hernández-Gea V, De Gottardi A, Leebeek FWG, Rautou PE, Salem R, Garcia-Pagan JC. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. doi:10.1016/j.jhep.2019.02.015.
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