Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (658 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
A condition in which the hepatic venous outflow is obstructed anywhere from the small hepatic veins to the junction of the inferior vena cava and the right atrium. Usually the blockage is extrahepatic and caused by blood clots (thrombus) or fibrous webs. Parenchymal fibrosis is uncommon.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Budd Chiari Syndrome; Chiari's Syndrome; Chiaris Syndrome; Chiari Syndrome; Hepatic Venous Outflow Obstruction; Hepatic Vein Thrombosis; Hepatic Vein Thromboses; Thromboses, Hepatic Vein.
Where it fits in the MeSH classification
- Broader topics
- Liver Diseases; Venous Thrombosis
Related concepts
- Hepatic Veins
- Inferior Vena Cava
- Right Atrium
- Thrombus
- Fibrosis
Research landscape
PubMed indexes 3,698 records tagged with the MeSH heading “Budd-Chiari Syndrome” (counts retrieved 2026-10-29). 335 of them (9.1%) were published from 2020 onward, and the busiest period so far is through 1999 with 1,798 records.
Reviews account for 408 records (11.0%) and meta-analyses for 18. Randomized controlled trials number 5 (0.1%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 1,798 |
| 2000-2009 | 778 |
| 2010-2019 | 787 |
| 2020 onward | 335 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 408 | 11.0% |
| Meta-analyses | 18 | 0.5% |
| Randomized controlled trials | 5 | 0.1% |
Method: PubMed query "Budd-Chiari Syndrome"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in cardiovascular medicine
Cardiovascular medicine has an unusually large body of randomized trials, so the type of outcome matters: hard clinical events such as death, heart attack and stroke carry more weight than changes in blood pressure, cholesterol or imaging markers that only predict risk. Observational studies can show associations but are confounded by age, lifestyle and other illness. Check whether a result is about prevention or treatment, the baseline risk of participants, and the absolute rather than only the relative size of any benefit.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Behcet's disease in Budd-Chiari syndrome | 2014 | Research article Design not recorded in structured metadata | 40 | cc by PMID 25213625 · DOI · PMC4392879 |
| Initial imaging analysis of Budd-Chiari syndrome in Henan province of China: most cases have combined inferior vena cava and hepatic veins involvement | 2014 | Research article Design not recorded in structured metadata | 32 | cc by PMID 24416352 · DOI · PMC3885682 |
| Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome with diffuse occlusion of hepatic veins | 2016 | Research article Design not recorded in structured metadata | 24 | cc by PMID 27805025 · DOI · PMC5090639 |
| Intravenous leiomyomatosis presenting as Budd-Chiari syndrome: a case report and literature review | 2025 | Review article Design not recorded in structured metadata | 1 | cc by PMID 39905462 · DOI · PMC11796087 |
Behcet's disease in Budd-Chiari syndrome [1]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundBehcet's disease (BD) is a well-known cause of Budd-Chiari syndrome (BCS).
- Closing excerpt
- Therefore early management with immunosuppressive and anticoagulation therapy appears to be the treatment of choice in patients with BCS and BD.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (112 words)
BackgroundBehcet's disease (BD) is a well-known cause of Budd-Chiari syndrome (BCS). Data are lacking on the presentation and outcome of BCS related to BD.MethodsWe investigated the relationship between BD and BCS in 14 patients with both diseases and compared the results to 92 BCS patients without BD.ResultsMale gender (p = 0.003), North African origin (P = 0.007) and inferior vena cava obstruction (P ConclusionThis study shows a higher frequency of IVC obstruction in patients with BCS and BD. Medical treatment with anticoagulation and immunosuppressive agents may improve the symptoms of BCS. Therefore early management with immunosuppressive and anticoagulation therapy appears to be the treatment of choice in patients with BCS and BD.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 25213625 · DOI · PMC4392879
Initial imaging analysis of Budd-Chiari syndrome in Henan province of China: most cases have combined inferior vena cava and hepatic veins involvement [2]
Abstract reading summary — source excerpts
- Opening excerpt
- AimTo evaluate the type of venous involvement in Chinese Budd-Chiari syndrome (BCS) patients and the relative diagnostic accuracy of the different imaging modalities.
- Closing excerpt
- The combination of CDUS and CTA or MRI is useful for diagnosis of BCS and guiding therapy.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (172 words)
AimTo evaluate the type of venous involvement in Chinese Budd-Chiari syndrome (BCS) patients and the relative diagnostic accuracy of the different imaging modalities.MethodsUsing digital subtraction angiography (DSA) as a reference standard, color Doppler ultrasound (CDUS), computed tomography angiography (CTA), and magnetic resonance angiography (MRA) were performed on 338 patients with BCS. We analyzed the course of the main and any accessory hepatic veins (HVs) and the inferior vena cava (IVC) to assess the etiology of obstructed segments and diagnostic accuracy of CDUS, CTA and MRA.ResultsAmong the 338 cases, there were 8 cases (2.4%) of isolated IVC membranous obstruction, 45 cases (13.3%) of isolated HV occlusion, and 285 cases (84.3%) with both IVC membranous obstruction and HV occlusion. Comparing with DSA, CDUS, CTA had a diagnostic accuracy of 89.3% and 80.2% in detecting BCS, and 83.4% of cases correctly correlated by MRA.ConclusionIn Henan Province, most patients with BCS have complex lesions combining IVC and HV involvement. The combination of CDUS and CTA or MRI is useful for diagnosis of BCS and guiding therapy.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 24416352 · DOI · PMC3885682
Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome with diffuse occlusion of hepatic veins [3]
Abstract reading summary — source excerpts
- Opening excerpt
- Either acute or sub-acute Budd-Chiari syndrome (BCS) with diffuse occlusion of hepatic veins has a high mortality rate and remains challenging for clinical treatment.
- Closing excerpt
- Collectively, TIPS is an effective and safe approach in treating BCS with diffuse occlusion of hepatic veins, which should be performed in time.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (200 words)
Either acute or sub-acute Budd-Chiari syndrome (BCS) with diffuse occlusion of hepatic veins has a high mortality rate and remains challenging for clinical treatment. We aimed to evaluate the feasibility and safety of transjugular intrahepatic portosystemic shunt (TIPS) as a treatment for BCS with diffuse occlusion of hepatic veins. From January 2007 to December 2010, 100 patients were randomly recruited onto this study and 91 patients were treated with TIPS. 14 patients were defined as acute BCS group and 86 patients as sub-acute group. Patients with acute BCS had a significantly higher rate of jaundice whereas a lower rate of abdominal and chest varices, gastroesophageal variceal bleeding and refractory ascites than sub-acute group (P < 0.001). TIPS was technically successful in all 91 patients (12 in acute group). The portosystemic pressure gradient (PSG) was decreased to normal level, while total bilirubin (TBIL) and liver function were significantly improved. During follow-up period, the mortality rate of 91 patients who underwent TIPS was 6.59% (6/91), whereas 88.89% of 9 patients who didn't receive TIPS procedure (2 in acute group). Collectively, TIPS is an effective and safe approach in treating BCS with diffuse occlusion of hepatic veins, which should be performed in time.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 27805025 · DOI · PMC5090639
Intravenous leiomyomatosis presenting as Budd-Chiari syndrome: a case report and literature review [4]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundBudd-Chiari syndrome (BCS) caused by intravenous leiomyomatosis (IVL) is rare.
- Closing excerpt
- Complete tumor resection is the only curative treatment and should be performed as soon as possible.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (174 words)
BackgroundBudd-Chiari syndrome (BCS) caused by intravenous leiomyomatosis (IVL) is rare. Further reports and thorough evaluation are needed to identify and manage this disease.MethodWe described the case of a 49-year-old lady, exhibiting features of BCS secondary to IVL, and reviewed three other previous cases of BCS caused by IVL.ResultsThe mean onset age of these four patients was 54.8 years. All but one (Patient No. 2) had a history of myoma, myomectomy, or hysterectomy. Abdominal pain, bloating or increasing abdominal circumference, and bilateral lower extremity edema were common symptoms. The establishment of clinical diagnoses of IVL and BCS mainly depends on clinical presentations and imaging, such as ultrasonography, computed tomography, and magnetic resonance imaging. Surgical intervention to alleviate the hepatic veins outflow obstruction is the most important treatment.ConclusionsBCS caused by IVL should be considered when the inferior vena cava and right atrium lesions were detected in a patient with characteristics of BCS and a history of uterine myoma or hysterectomy. Complete tumor resection is the only curative treatment and should be performed as soon as possible.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 39905462 · DOI · PMC11796087
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article Vascular disorders of the liver [5]
- Research article Budd-Chiari syndrome: a review by an expert panel [6]
- Research article The Budd-Chiari syndrome [7]
- Research article Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis [8]
- Research article Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature [9]
- Research article Primary Budd-Chiari syndrome [10]
References
- Desbois AC, Rautou PE, Biard L, Belmatoug N, Wechsler B, Resche-Rigon M, et al.. Behcet's disease in Budd-Chiari syndrome. . 2014. PMID 25213625 · DOI · PMC4392879
- Zhou P, Ren J, Han X, Wu G, Zhang W, Ding P, et al.. Initial imaging analysis of Budd-Chiari syndrome in Henan province of China: most cases have combined inferior vena cava and hepatic veins involvement. . 2014. PMID 24416352 · DOI · PMC3885682
- He F, Zhao H, Dai S, Wu Y, Wang L, Huang H, et al.. Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome with diffuse occlusion of hepatic veins. . 2016. PMID 27805025 · DOI · PMC5090639
- Gan J, Ma X, Shao J, Wang J, Cao D. Intravenous leiomyomatosis presenting as Budd-Chiari syndrome: a case report and literature review. . 2025. PMID 39905462 · DOI · PMC11796087
- DeLeve LD, Valla DC, Garcia-Tsao G, American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology. 2009. PMID 19399912 · DOI · PMC6697263
- Janssen HL, Garcia-Pagan JC, Elias E, Mentha G, Hadengue A, Valla DC, et al.. Budd-Chiari syndrome: a review by an expert panel. J Hepatol. 2003. PMID 12586305 · DOI
- Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004. PMID 14762185 · DOI
- Smalberg JH, Arends LR, Valla DC, Kiladjian JJ, Janssen HL, Leebeek FW. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis. Blood. 2012. PMID 23043069 · DOI
- 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. PMID 8309360 · DOI
- Valla DC. Primary Budd-Chiari syndrome. J Hepatol. 2009. PMID 19012988 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.