European Journal of Cardiovascular Medicine

Budd-Chiari syndrome (BCS) is a rare clinical condition characterized by obstruction of the hepatic venous outflow tract between hepatic veins and the junction of the inferior vena cava with the right atrium; which was described by George Budd (1808-1882) in 1845 long before Hans Chiari (1851-1916) mentioned in his pathological observations as “obliterating endo-phlebitis of the hepatic veins" in the later years in 1899. ^{[1, 12, 13]}

Despite the use of anticoagulation, many patients may need additional Interventional Radiology (I.R) treatment strategies. Algorithms consisting of local Venoplasty, Hepatic Vein Stenting (HVS), Catheter Directed Thrombolysis (CDT), Trans-jugular Intra-hepatic Porto-systemic Shunt (TIPS), Direct Intrahepatic Portacaval Shunt (DIPS) ^[9]  as bridge to Liver Transplantation (LT) have been proposed with treatment choice dictated by a lack of response to a less-invasive treatment regimen and clinical response. ^[1,10,11,12].

Select List of cases with analytics:

Fig 1: USG showing no flow across Hepatic veins.

Fig 2: CECT showing the classic nutmeg appearance.

Fig 3: Preop Venogram showing cannulated Hepatic vein. Flow seen passing through collaterals

Fig 4: Venoplasty of the hepatic veins.

Fig 5: Increased flow across hepatic veins post Venoplasty

Fig 6: IVC Venoplasty

Fig 7: Stent placement and deployment in the hepatic vein and hepatic portion of IVC.

Fig 8: Post stenting Venogram - Good flow seen across stent and into cavo-atrium.

Fig 9: USG showing hepatic vein stent in situ and good flow across the stent.

The clinical manifestation of BCS is heterogeneous, with presentations ranging from completely asymptomatic patient to acute liver failure. The classic triad of  right upper quadrant pain, ascitis and hepatomegaly is commonly present in symptomatic patients with male predilection , with tender hepatomegaly  in 83%, ascitis in 39% of patients, jaundice in 83%,thrombocytopenia/platelet refractoriness in 78%,edema in 50%. ^{[3,8,13,16,17]} Less common clinical manifestations include esophageal bleeding (5%) and hepatic encephalopathy (9%). Up to 20% of patients are completely asymptomatic. The presentation of BCS depends on the extent and rapidity of hepatic venous outflow obstruction and the presence of decompressing venous collaterals. This concept resulted in classifications of BCS as being fulminant, acute, subacute or chronic. ^[1]

BCS is further classified as being primary or secondary, depending on the type of the hepatic venous outflow obstruction. When flow is obscured by compression or invasion of a lesion outside the hepatic venous outflow track, it is regarded as being secondary BCS; examples include malignant and benign extrinsic obstruction. Benign extrinsic compression being lesions like abscess, cyst, adenoma, etc. If flow is obstructed due to an endoluminal cause, then it is classified as being primary BCS. By far the most common cause of primary BCS is thrombosis. ^[2]

Although Deficiency of   protein C primarily  is most commonly prevalent (25%) ; however Protein C,  protein S along with anti-thrombin III  in presence of liver disease with acute thrombus  maybe  low , including  BCS patients; as these circulating proteins are synthesized in the liver and maybe affected in liver dysfunction thus lacking  proof of the primary deficiency. ^[2,15]

Blockage of two or more major hepatic veins increases the sinusoidal pressure and reduces sinusoidal blood flow. Obstruction of a single hepatic vein is generally not evident; two veins must be blocked for clinical disease. The result of these hemodynamic changes is sinusoidal dilation and filtration of interstitial fluid. Filtrated interstitial fluid passes through the liver capsule when it exceeds the capacity of lymphatic drainage. Thus, liver congestion, right upper quadrant pain and ascites occur. Portal pressure increases and perfusion of the liver via portal vein is decreased. The combined effect of these changes in hepatic circulation on liver parenchyma is hypoxic damage of hepatocytes. ^[3]

Within a few weeks after obstruction, fibrosis develops predominantly in the centrilobular area. ^[4]

Progressive fibrosis, nodular regenerative hyperplasia and cirrhosis develop during the course of disease. Interventional portosystemic shunts or development of portal venous collateral system may improve liver functions and delay the cirrhotic process. ^[4]

However long term monitoring with followup imaging and serum Alpha Feto Protein (AFP) should be done to diagnose early or to suggest prompt  appropriate  I.R procedures as needed  since patients with  chronic Budd-Chiari syndrome are at risk for Hepato-Cellular Carcinoma (HCC). ^[14]

Doppler ultrasonography of the liver, with a sensitivity and specificity of 85% or more, is the technique of choice for initial investigation when BCS is suspected. ^[5]

Magnetic resonance imaging (MRI) should be performed as a second-line imaging modality. MRI can show the hepatic vein thrombosis and evaluate the IVC, but it is more expensive than computed tomographic (CT) scanning. MRI is not as effective as sonography in demonstrating the intrahepatic collateral vessels and cannot show flow direction. ^[6]

CT scanning is recommended for imaging the vascular anatomy and the configuration of the liver when endovascular management is considered. ^[6]

Management of Budd Chiari Syndrome is an algorithmic approach, with increasing invasiveness as required depending on the response and prognosis of the disease. ^[7]

Medical management alone is effective in some cases however, others need radiological intervention.

Acute thrombosis - Catheter directed thrombo-lysis and mechanical thrombectomy.

The initial preferable intervention is recanalisation of narrowed or occluded hepatic veins or inferior vena cava to restore hemodynamic hepatic venous drainage by doing Venoplasty with or without stent placement. ^[7]

Transjugular intrahepatic portosystemic shunt (TIPS) or Direct intrahepatic portosystemic shunt (DIPS) is indicated when hepatic venous recanalisation is not feasible. Placement of TIPS/DIPS  in such scenarios is  associated with  better palliative measures as bridge to LT or when best medical management fails. ^[7,17-21]

Surgical portosystemic shunts are alternative but  more invasive measures when TIPS/DIPS are not feasible or fail as bridge to LT.

Now a days  the utility  of self-expanding Nitinol Stent encapsulated with ePTFE (expanded Polytetrafluoroethylene) has not only helped with improved shunt  patency rates and thus decreased TIPS/DIPS failure secondary to occlusion due to thrombosis. Thus preference of  stent grafts as over compared to only bare stents. ^[7,17-21]

Orthotopic liver transplant (LT) is indicated for patients with fulminant BCS and those who do not improve after TIPS placement. TIPS/DIPS can serve as a bridge to transplant in transplant awaited patients as per clinical radiological scenario and feasibility.

In this article, we discuss the radiological findings in patients who were diagnosed with Budd Chiari and applications of interventional radiology in its management. ^[12,13]

Endovascular management has emerged to play an important role in the treatment of Budd-Chiari Syndrome and offers minimally invasive and highly effective methods to restore adequate venous outflow required to overcome portal hypertension, and in turn helps to mitigate long term complications i.e. delay progression to hepatic failure and cirrhosis by salvage as alternative outflow and decongestion.

Recent advances in imaging techniques have contributed significantly in making the endovascular interventions a success as compared to surgical shunting, showing lower morbidity and mortality rates with faster recovery.

There are a few limitations which come along with endovascular management. Complications like stent failure due to thrombosis, restenosis, or any such technical failure. These can be limited or overcome by methods such as right patient selection, proper patient planning and Postop follow ups. 

Furthermore, endovascular techniques might not be suitable for patients with advanced hepatic failure or extensive venous obliteration, where liver transplantation should be considered as the definitive treatment.

1. Martens P, Nevens F. Budd-Chiari syndrome. United European Gastroenterol J. 2015 Dec;3(6):489-500. doi: 10.1177/2050640615582293. PMID: 26668741; PMCID: PMC4669515
2. (Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol. 2007 May 21;13(19):2693-6. doi: 10.3748/wjg.v13.i19.2693. PMID: 17569137; PMCID: PMC4147117.
3. Bayraktar UD, Seren S, Bayraktar Y. Hepatic venous outflow obstruction: three similar syndromes. World J Gastroenterol. 2007 Apr 7;13(13):1912-27. doi: 10.3748/wjg.v13.i13.1912. PMID: 17461490; PMCID: PMC4146966.
4. Porrello, G.; Mamone, G.; Miraglia, R. Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives. Diagnostics2023, 13, 2256. https://doi.org/10.3390/diagnostics13132256
5. Bansal V, Gupta P, Sinha S, Dhaka N, Kalra N, Vijayvergiya R, Dutta U, Kochhar R. Budd-Chiari syndrome: imaging review. Br J Radiol. 2018 Dec;91(1092):20180441. doi: 10.1259/bjr.20180441. Epub 2018 Jul 24. PMID: 30004805; PMCID: PMC6319835.
6. Erden A, Erden I, Karayalçin S, Yurdaydin C. Budd-Chiari syndrome: evaluation with multiphase contrast-enhanced three-dimensional MR angiography. AJR Am J Roentgenol. 2002;179:1287–1292.
7. Rathod K, Deshmukh H, Shukla A, Popat B, Pandey A, Gupte A, Gupta DK, Bhatia SJ. Endovascular treatment of Budd-Chiari syndrome: Single center experience. J Gastroenterol Hepatol. 2017 Jan;32(1):237-243. doi: 10.1111/jgh.13456. PMID: 27218672.
8. Carreras E, Grañena A, Navasa M, Bruguera M, Marco V, Sierra J, Tassies MD, García-Pagán JC, Martí JM, Bosch J, et al. On the reliability of clinical criteria for the diagnosis of hepatic veno-occlusive disease. Ann Hematol. 1993 Feb;66(2):77-80. doi: 10.1007/BF01695888. PMID: 8448243.
9. Petersen BD, Clark TW. Direct intrahepatic portocaval shunt. Tech Vasc Interv Radiol. 2008 Dec;11(4):230-4. doi: 10.1053/j.tvir.2009.04.006. PMID: 19527850.
10. Mukund A, Gamanagatti S. Imaging and interventions in Budd-Chiari syndrome. World J Radiol 2011; 3(7): 169-177 [PMID: 21860712 DOI: 10.4329/wjr.v3.i7.169]
11. Porrello G, Mamone G, Miraglia R. Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives. Diagnostics (Basel). 2023 Jul 3;13(13):2256. doi: 10.3390/diagnostics13132256. PMID: 37443650; PMCID: PMC10341099.
12. Brancatelli, G., Vilgrain, V., Federle, M. P., Hakime, A., Lagalla, R., Iannaccone, R., & Valla, D. (2007). Budd-Chiari syndrome: spectrum of imaging findings.  American Journal of Roentgenology, 188(2), W168-76. https://doi.org/10.2214/AJR.05.0168
13. Silverstone L, Qasrawi H, Niknejad M, et al. Budd-Chiari syndrome. Reference article, Radiopaedia.org https://doi.org/10.53347/rID-1023
14. Vilgrain V, Paradis V, Van Wettere M, Valla D, Ronot M, Rautou P. Benign and Malignant Hepatocellular Lesions in Patients with Vascular Liver Diseases. Abdom Radiol (NY). 2018;43(8):1968-77. doi:10.1007/s00261-018-1502-7
15. Deltenre P, Denninger MH, Hillaire S, Guillin MC, Casadevall N, Brière J, Erlinger S, Valla DC. Factor V Leiden related Budd-Chiari syndrome. Gut. 2001;48:264–268. doi: 10.1136/gut.48.2.264.
16. Okuda H, Yamagata H, Obata H et al. Epidemiological and Clinical Features of Budd-Chiari Syndrome in Japan. J Hepatol. 1995;22(1):1-9. doi:10.1016/0168-8278(95)80252-5
17. Asif A. Hitawala & Vikas Gupta. Budd Chiari Syndrome. StatPearls Publishing. 2021. https://www.ncbi.nlm.nih.gov/books/NBK558941/
18. Shrestha R, Durham JD, Wachs M, et al. 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.
19. Hernández-Guerra M, Turnes J, Rubinstein P, et al. PTFE-covered stents improve TIPS patency in Budd-Chiari syndrome. Hepatology. 2004; 40: 1197–202.
20. Gandini R, Konda D, Simonetti G. Transjugular intrahepatic portosystemic shunt patency and clinical outcome in patients with Budd-Chiari syndrome: covered versus uncovered stents. Radiology. 2006; 241:298–305.
21. Ulrich F, Pratschke J, Neumann U, et al. Eighteen years of liver transplantation experience in patients with advanced Budd-Chiari syndrome. Liver Transpl. 2008; 14:144-50.