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Etiology, Management, and Outcome of the Budd-Chiari Syndrome

Sarwa Darwish Murad, MD, PhD; Aurelie Plessier, MD; Manuel Hernandez-Guerra, MD; Federica Fabris, MD; Chundamannil E. Eapen, MD; Matthias J. Bahr, MD; Jonel Trebicka, MD; Isabelle Morard, MD; Luc Lasser, MD; Joerg Heller, MD; Antoine Hadengue, MD; Philippe Langlet, MD; Helena Miranda, MD, PhD; Massimo Primignani, MD; Elwyn Elias, MD, BSc, FRCP; Frank W. Leebeek, MD, PhD; Frits R. Rosendaal, MD, PhD; Juan-Carlos Garcia-Pagan, MD, PhD; Dominique C. Valla, MD, PhD; Harry L.A. Janssen, MD, PhD, for EN-Vie (European Network for Vascular Disorders of the Liver)
[+] Article, Author, and Disclosure Information

For a list of the EN-VIE Investigators, see the Appendix.

From Erasmus MC University Hospital Rotterdam, Rotterdam, and Leiden University Medical Center, Leiden, the Netherlands; Hôpital Beaujon, AP-HP, INSERM U773, and Université Denis Diderot-Paris 7, Clichy, France; Hospital Clinic, IDIBAPS and Ciberehd, Barcelona, Spain; IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Milan, Italy; Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom; Hannover Medical School, Hannover, and University Hospital of Bonn, University of Bonn, Bonn, Germany; Geneva University Hospitals, Geneva, Switzerland; Centre Hospitalier Universitaire Brugmann, Brussels, Belgium; and Hospital General Santo Antonio, Porto, Portugal.

Acknowledgment: The authors thank the study investigators, without whom this study would have not been possible, and the study participants.

Grant Support: The EN-Vie project was financially supported by the Fifth Framework Programme of the European Commission (contract QLG1-CT-2002-01686) and public funding from GIS Maladies Rares (France 2001). Dr. Darwish Murad is a Mosaic Laureate of the Netherlands Organization for Scientific Research. Dr. Janssen is a Clinical Fellow and VIDI laureate of the Netherlands Organization for Scientific Research. Ciberehd is funded by Instituto de Salud Carlos III.

Potential Financial Conflicts of Interest: None disclosed.

Reproducible Research Statement:Study protocol: Available from Dr. Janssen (e-mail, h.janssen@erasmusmc.nl). Statistical code: Not available. Data set: Available in a limited fashion to approved individuals with a relevant research proposal by contacting Dr. Janssen (e-mail, h.janssen@erasmusmc.nl).

Requests for Single Reprints: Harry L.A. Janssen, MD, PhD, Department of Gastroenterology and Hepatology, Erasmus MC University Hospital Rotterdam, 's Gravendijkwal 230, 3015 CE Rotterdam, the Netherlands; e-mail, h.janssen@erasmusmc.nl.

Current Author Addresses: Drs. Darwish Murad and Janssen: Department of Gastroenterology and Hepatology, Erasmus MC University Hospital Rotterdam, 's Gravendijkwal 230, 3015 CE Rotterdam, the Netherlands.

Drs. Plessier and Valla: Service d'Hépatologie, Hôpital Beaujon, AP-HP, INSERM U773, and Université Denis Diderot-Paris 7, 100 boulevard du général Leclerc, 92110 Clichy, France.

Dr. Hernandez-Guerra and Garcia-Pagan: Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic, IDIBAPS and Ciberehd, Carrer de Villarroel 170, 08036 Barcelona, Spain.

Dr. Fabris and Primignani: Gastroenterology Unit, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Via Pace 9, I-20122 Milan, Italy.

Drs. Eapen and Elias: Liver Unit, Queen Elizabeth Hospital Birmingham, Metchley Park Road, Edgbaston, B15 2TH Birmingham, United Kingdom.

Dr. Bahr: Department of Gastroenterology, Hepatology, and Endocrinology, Hannover Medical School, Carl Neuberg Strasse 1, D-30623 Hannover, Germany.

Drs. Trebicka and Heller: Department of Internal Medicine I, University Hospital of Bonn, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn, Germany.

Drs. Morard and Hadengue: Division of Gastroenterology and Hepatology, Geneva University Hospitals, 24 rue Micheli-du-Crest, CH-1211 Geneva, Switzerland.

Drs. Lasser and Langlet: Department of Hepatogastroenterology, Centre Hospitalier Universitaire Brugmann, Place Van Gehuchten 4, B-1020 Brussels, Belgium.

Dr. Miranda: Liver Transplantation Unit, Hospital General Santo Antonio, Largo Abel Salazar, P-4090-001, Porto, Portugal.

Dr. Leebeek: Department of Hematology, Erasmus University Medical Center Rotterdam, 's Gravendijkwal 230, 3015 CE Rotterdam, the Netherlands.

Dr. Rosendaal: Department of Clinical Epidemiology and Department of Thrombosis and Haemostasis, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, the Netherlands.

Author Contributions: Conception and design: S. Darwish Murad, A. Plessier, F. Fabris, M.J. Bahr, A. Hadengue, M. Primignani, E. Elwyn, F.R. Rosendaal, J.C. Garcia-Pagan, D.C. Valla, H.L.A. Janssen.

Analysis and interpretation of the data: S. Darwish Murad, A. Plessier, C.E. Eapen, M.J. Bahr, J. Trebicka, A. Hadengue, E. Elwyn, F.W. Leebeek, J.C. Garcia-Pagan, D.C. Valla, H.L.A. Janssen.

Drafting of the article: S. Darwish Murad, A. Plessier, E. Elwyn, J.C. Garcia-Pagan, H.L.A. Janssen.

Critical revision of the article for important intellectual content: A. Plessier, F. Fabris, M.J. Bahr, J. Trebicka, J. Heller, M. Primignani, E. Elwyn, F.W. Leebeek, F.R. Rosendaal, J.C. Garcia-Pagan, D.C. Valla, H.L.A. Janssen.

Final approval of the article: A. Plessier, F. Fabris, M.J. Bahr, J. Heller, M. Primignani, E. Elwyn, F.W. Leebeek, F.R. Rosendaal, J.C. Garcia-Pagan, D.C. Valla, H.L.A. Janssen.

Provision of study materials or patients: S. Darwish Murad, A. Plessier, M. Hernandez-Guerra, F. Fabris, C.E. Eapen, M.J. Bahr, L. Lasser, J. Heller, P. Langlet, H. Miranda, M. Primignani, E. Elwyn, F.W. Leebeek, H.L.A. Janssen.

Statistical expertise: S. Darwish Murad, A. Plessier, F.R. Rosendaal, J.C. Garcia-Pagan, H.L.A. Janssen.

Obtaining of funding: M.J. Bahr, H. Miranda, E. Elwyn, J.C. Garcia-Pagan, H.L.A. Janssen.

Administrative, technical, or logistic support: S. Darwish Murad, A. Plessier, M.J. Bahr, H.L.A. Janssen.

Collection and assembly of data: S. Darwish Murad, A. Plessier, M. Hernandez-Guerra, F. Fabris, C.E. Eapen, M.J. Bahr, J. Trebicka, I. Morard, L. Lasser, J. Heller, P. Langlet, H. Miranda, M. Primignani, J.C. Garcia-Pagan, H.L.A. Janssen.

Ann Intern Med. 2009;151(3):167-175. doi:10.7326/0003-4819-151-3-200908040-00004
Text Size: A A A

Background: The Budd–Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases.

Objective: To characterize the causes and treatment of incident BCS.

Design: Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006.

Setting: Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland.

Patients: Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded.

Measurements: Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival.

Results: 163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years.

Limitation: Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients.

Conclusion: Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure.

Primary Funding Source: Fifth Framework Programme of the European Commission.


Grahic Jump Location
Figure 1.
Study flow diagram.

OLT = orthoptic liver transplantation; PSS = portosystemic shunt; PTA = percutaneous transluminal angioplasty; TIPS = transjugular intrahepatic portosystemic shunt.

* 2 patients underwent TIPS, and 1 underwent surgical shunting.

† 22 patients proceeded directly to recanalization, 46 proceeded directly to decompression, and 15 proceeded directly to OLT.

‡ Patients can have had >1 procedure.

§ Revision of TIPS by recanalization procedures.

Grahic Jump Location
Grahic Jump Location
Figure 2.
Cumulative probability of overall survival, OLT-free survival, and intervention-free survival.

OLT = orthoptic liver transplantation.

Grahic Jump Location




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Budd Chiari Syndrome: Is Celiac disease a potential cause for thrombosis?
Posted on August 14, 2009
Harish V Iyer
Albert Einstein Medical Center, Philadelphia
Conflict of Interest: None Declared

We read with interest the review of Budd-Chiari syndrome (BCS) by Murad and colleagues (1) in which they discuss the thrombotic risk factors associated with the development of BCS at length. We believe that there may be a hitherto unmentioned association between BCS and another systemic illness: Celiac disease.

We recently cared for a 37 year old lady with newly diagnosed Celiac Disease (CD) who developed BCS within a month of presentation. Hypercoagulable work-up showed no obvious abnormality. She was anticoagulated with Warfarin and has been asymptomatic on follow up. Literature review yielded another ten cases of this peculiar association (2). In fact, when the search was expanded to thrombosis in any vascular bed (venous or arterial), another twenty-five thrombotic events were reported in the literature.

Of the patients with BCS and CD, the initial case reports described patients from the North African region. This geographic clustering lead to the thought that there may be some common underlying genetic or dietary factors. But more recently, this association has been noted in Spain, Turkey, Argentina, and now in the United States of America (our case) arguing against this.

Though CD is not classically thought to predispose to thrombosis, there are several pathophysiological mechanisms that have been noted in CD that may contribute to the development of a potentially prothrombotic milieu. Hyperhomocysteniemia caused by vitamin deficiencies (especially folic acid deficiency) and by methylenetetrahydrofolate reductase (MTHFR) gene variants are known to occur in CD more than in controls (3,4). High levels of Thrombin-activatable Fibrinolysis inhibitor (TAFI) are noted in CD, and this predisposes to thrombosis (5). Other potential mechanisms include transient vitamin K deficiency and thrombocytosis.

A recently published, large population based study by Ludvigsson et al. looking at the association of venous thromboembolism in CD showed that patients with CD were more likely to have a thrombotic event than matched controls (6).

It is unclear if the occurrence of BCS or thrombosis in CD is merely coincidental, but the data seems to suggest that there may be a hitherto unrecognized association. We appreciate that these are retrospective studies and case reports, and do not implicate causality, but it underscores the need for large prospective studies to clarify this. Given the increased estimated prevalence of CD, this is of paramount importance to internists and specialists alike.


1.Etiology, management, and outcome of the Budd-Chiari syndrome. Murad SD, Plessier A, Hernandez-Guerra M et. al. Ann Intern Med. 2009 ;151: 167-75.[PMID: 19652186]

2.[Celiac disease associated with Budd-Chiari syndrome] Ouakaa-Kchaou A, Ennaifer R, Belhadj N et. al. Presse Med. 2008;37: 239-41[PMID: 18096356]

3.Wilcox GM, Mattia AR. Celiac sprue, hyperhomocysteinemia, and MTHFR gene variants. J Clin Gastroenterol. 2006;40:596-601 [PMID: 16917400]

4.Saibeni S, Lecchi A, Meucci G et al. Prevalence of hyperhomocysteinemia in adult gluten-sensitive enteropathy at diagnosis: role of B12, folate, and genetics. Clin Gastroenterol Hepatol. 2005;3:574 [PMID: 15952099]

5.Saibeni S, Bottasso B, Spina L, et al. Assessment of thrombin-activatable fibrinolysis inhibitor (TAFI) plasma levels in inflammatory bowel diseases. Am J Gastroenterol. 2004;99:1966-70.[PMID: 15447757]

6.Ludvigsson JF, Welander A, Lassila R, et al. Risk of thromboembolism in 14,000 individuals with coeliac disease. Br J Haematol. 2007;139:121-7.[PMID: 17854316]

Conflict of Interest:

None declared

Not Etiology, but just Risk Factors
Posted on September 7, 2009
Guohong Han
Xijing Hospital of Digestive Diseases, Fourth Military Medical University
Conflict of Interest: None Declared

We read with great interest the large prospective multicenter study from the European Network for Vascular Disorders of Liver (1), which produced beyond doubt the most reliable evidence for guiding therapeutic strategy selection of Budd-Chiari Syndrome (BCS) at present. However, it seems to be extremely inadequate for explaining ¡°etiology¡± or ¡°cause¡± as put forward in the title and abstract.

Researchers only summarized the prevalence of various thrombotic risk factors in 163 patients with BCS without any suggestions of the causal relationship between risk factors and BCS. In other words, risk factors were examined for the first time together with newly diagnosed BCS enrolled in the observational study. Which on earth was the cause, BCS or risk factors? Did BCS induce or arise from JAK2 mutation? The answer was ambiguous in the prospective study, despite JAK2 mutation was closely associated with BCS (2). We would be glad to accept the etiology if the researchers added another controlled group into the study, for example, comparision between BCS and no BCS or no risk factors and risk factors in order to calculate odd ratio (OR) or relative risk (RR) of risk factors in BCS respectively. Besides, it was unknown whether or not any risk factor was properly treated, which may influence re-thrombosis or re-stenosis of shunt if prothrombotic disorder was not eliminated and even long-term survival due to its progression into leukemia.

Secondly, it is still confusing to clarify whether deficiencies of protein C and S and antithrombin in BCS with liver disease are hereditary or not, because of the affinity between coagulation abnormalities and liver disease. A deficiency was considered hereditary in the study without hepatocellular dysfunction which was judged if bilirubin level was less than 2 times the upper limit of normal. Nevertheless, two recent reviews recommended that the diagnosis of primary defect would be established, if coagulation inhibitors were at a low level but coagulation factors entirely normal (3) or screening of a first-degree relative was positive (4). However, in most cases, protein C, protein S and antithrombin deficiencies may be not genetic in origin (5). Facing to the uncertainties, we have to question whether it is indispensable to distinguish between inherited and acquired deficiencies in patients with liver disease particularly its low prevalence (3-4%) as demonstrated in the study.

Finally, we are very interested in why only 77 of 163 patients were screened for paroxysmal nocturnal hemoglobinuria by comparison with other risk factors screened in most of patients.


1. Murad SD, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, et al; European Group for the Study of Vascular Disorders of the Liver. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009;151:167-175. [PMID:19652186]

2. Primignani M, Barosi G, Bergamaschi G, Gianelli U, Fabris F, Reati R, et al. Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis. Hepatology. 2006;44:1528-1534. [PMID: 17133457]

3. Valla DC. Thrombosis and anticoagulation in liver disease. Hepatology. 2008;47:1384-1393. [PMID:18318436]

4. DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology. 2009;49:1729-1764. [PMID: 19399912]

5. Dubuisson C, Boyer-Neumann C, Wolf M, Meyer D, Bernard O. Protein C, protein S and antithrombin III in children with portal vein obstruction. J Hepatol. 1997;27:132-I35. [PMID: 9252086]

Conflict of Interest:

None declared

Budd-Chiari syndrome: varied underlying thrombophilic aetiology in different geographical regions
Posted on September 8, 2009
Kanjaksha Ghosh
National Institute of Immunohaematology
Conflict of Interest: None Declared

Budd-Chiari syndrome: varied underlying thrombophilic aetiology in different geographical regions






We read with interest the report on the underlying thrombophilia and the various

management options in Budd-Chiari syndrome (BCS) cases, culled from several European centers (1)


In India, we see lot more patients with BCS and their gross epidemiology is reviewed in several previous publications (2). At our center in Mumbai with an average population of 15 million, during the last 10 years, we have seen 320 BCS patients which is five times more than the prevalence described by the authors.� 130 (41 %) of these were acute cases, presented within 2-3 weeks of onset of the symptomsThe anticoagulation therapy generally involved heparin to begin with followed by oral anticoagulation to maintain an INR between� 2.5 - 3.0. 56 (17.5%) patients had a prothrombin time (PT) > 5 seconds than the control samples in which the vitamin K dependent factors could not be estimated. The thrombophilia profile of our patients is given in table 1.


It may be clearly seen that JAK2 mutation is responsible only for a minor fraction of patients with BCS in our country, in contrast to what has been reported in literature (3). Authors have rightly pointed out the discrepancy between diagnosis of myeloproliferative disorders and JAK2 mutation in BCS cases. In this context, one should consider the fact that the prevalence of JAK2 mutation varies greatly across the three rather well defined myeloproliferative disorders and in early cases the marrow morphology is not unequivocally diagnostic.In a similar manner, factor V Leiden was found to be significantly high in Indian BCS cases as compared to other thrombosis groups or the normal controls (4). Prothrombin gene G20210A polymorphism has not been seen either in patients or controls in Indian population.


Another interesting observation is the high prevalence of� pregnancy associated������� BCS (12 %) in our series which further limits the treatment options of oral������� anticoagulation in these patients. Whether conditioned borderline folic acid deficiency leading to hyperhomocysteinemia acts as a trigger in the presence of�� additional underlying acquired or inherited thrombophilia is not known. The prevalence of common MTHFR C677T polymorphism which is strongly associated with hyperhomocysteinemia is however not very different in both patients and controls. In 18 % of the chronic BCS cases, the oral anticoagulation could not be given due to associated thrombocytopenia and liver dysfunction. Transjugular Intrahepatic Portosystemic Shunt (TIPS) as the primary therapy showed promising results in these patients.




Table 1. Prevalence of thrombophilia markers in BCS cases


marker Protein C*������ Protein S*������ Antithrombin*����������� Lupus anticoagulant��� Anticardiolipin

antibodies������� Factor V Leiden��������� PT gene

polymorphism JAK2

Number positive (%)�� 34 (12.9)��������� 16 (6.1)����������� 8 (3)���� 29 (9.1)����������� 35 (10.9)��������� 75 (23.43)������� 0��������� 26 (8.1)

OR ( 95% CI, P value)����������� 14.64 (1.975-108.5) 0.0002��� 3.161(0.7133-14.011)0.1731� 6.661(0.3806-116.56)����������� 9.866(1.326-73.41) 0.034������ 6.01891.42-25.49) 0.004�������� 9.898 (3.048 -32.147) P <0.0001������� -����������� 18.087 ( 1.091 -299.72) 0.0013

* Total number screened -264



1.�������� 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(3):167-75 [PMID: 19652186].

2.�������� � 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 Jan;73(1):21-36 [PMID: 8309360]

3.�������� � Colaizzo D, Amitrano L, Tiscia GL, Iannaccone L, Gallone A, Grandone E et al. Occurrence of the JAK2 V617F mutation in the Budd-Chiari syndrome. Blood Coagul Fibrinolysis. 2008 Jul;19(5):459-62 [PMID: 18600100].

4.�������� Mohanty D, Shetty S, Ghosh K, Pawar A, Abraham P. Hereditary thrombophilia as a cause of Budd-Chiari syndrome: a study from Western India. Hepatology. 2001 Oct;34(4 Pt 1):666-70 [PMID: 11584361].



Conflict of Interest:

None declared



More PTAs than TIPSes for Budd-Chiari Syndrome in Asia
Posted on September 11, 2009
Guohong Han
Xijing Hospital of Digestive Diseases, Fourth Military Medical University
Conflict of Interest: None Declared

TO THE EDITOR: Compared with the largest prospective multicenter study at present (1) providing strong evidence of the management of Budd- Chiari syndrome (BCS), any previous retrospective studies (2-4) appeared to be marginal although indispensable for promoting the stepwise therapeutic strategy. However, maybe a high proportion of application of transjugular intrahepatic portosystemic shunt (TIPS) is just appropriate in Western patients, but not Asian. Herein, we attempted to simply describe our unpublished data on management and preliminary outcome of BCS.

Between October 1998 and September 2008 in our center, 98 patients with BCS were successfully treated with percutaneous transluminal angioplasty (PTA), of whom 88 presented with ascites, 8 gastrointestinal bleeding, 4 acute liver failure, 2 hepatic encephalopathy and 1 hepatorenal syndrome; only 19 with TIPS, in whom severe complications of portal hypertension existed. In PTA group, pure inferior vena cava (IVC) obstruction was observed in 34 (34.7%) patients, pure hepatic veins (HV) in 22 (22.4%) and combined HV and IVC in 42 (42.9%); the cumulative primary patency rates at 1-, 3-, and 5-year were 96% (95% CI, 91�C100%), 94% (95% CI, 87�C99%) and 94% (95% CI, 87�C99%),respectively; the cumulative survival rates at 1-, 3-, 5-year were 94% (95% CI, 90�C99%), 91% (95% CI, 85�C98%), and 87% (95% CI, 78�C96%) respectively, which suggested the great potential of PTA alone, unlike nearly deserted utility (14/163) and low recanalization rate (8/14) as shown in the study (1). In addition, an ongoing multicenter study of BCS in China, including our center, showed the similar proportion that application of PTA was far more than TIPS. Therefore, it would bring patients lots of unnecessary sequels such as hepatic encephalopathy, if preference for TIPS in West was completely transplanted into Asia.

Furthermore, a hypothesis was formulated that different proportion of therapeutic strategy between West and China (Table 1.) may lie in large geographic variations in the prevalence of various levels of hepatic venous outflow obstruction and separate natures of venous lesions (5).


Table 1. Comparison between PTA alone and TIPS for Budd-Chiari syndrome: West versus Asia ��

Authors Interventional Therapeutic Strategy


���������������������������������� No. PTA alone� �����No. TIPS� ���������PTA / TIPS

EN-vie (2009)�������������� ��8�������� ����������������56������������������� 0.14

Garcia-Pagan (2008)���� 29�������� ����������������124������������������ �0.23

Plessier (2006)���� ���������6���������� ����������������20������������������� �0.30

Eapen (2006)�������������� �31��������� ���������������30������������������� �1.03

Our center������������������ �98��������� ���������������19������������������� �5.16

PTA=percutaneous transluminal angioplasty; TIPS=transjugular intrahepatic portosystemic shunt. #, at most 8 patients received successful recanalization by PTA alone.




1.    Murad SD, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, et al; European Group for the Study of Vascular Disorders of the Liver. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009;151:167-75. [PMID:19652186]


���� 2. Garcia-Pagan JC, Heydtmann M, Raffa S, Plessier A, Murad S, Fabris F, et al. TIPS for Budd-Chiari Syndrome: Long-term results and prognostics factors in 124���� patients. Gastroenterology. 2008;135:808�C15. [PMID: 18621047]


���� 3. 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. [PMID: 17058215]


���� 4. Eapen CE, Velissaris D, Heydtmann M, Gunson B, Olliff S, Elias E. Favourable medium term outcome following hepatic vein recanalisation and/or transjugular� intrahepatic portosystemic shunt for Budd Chiari syndrome. Gut. 2006;55:878�C84. [PMID: 16174658]


���� 5. Valla DC. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol. 2004;19(Suppl 7):204�C11. Accessed at http://pt.wkhealth.com/pt/re/jgah/abstract.00001753-200412007-00032.htm; jsessionid=KqBh81qhTxtBVvT7zgZnvBCq3mDJJg3lWcyswh2BPQJy0plr2RM9!783167578!181195629!8091! -1


Conflict of Interest:

None declared


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