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

  1. Sarwa Darwish Murad, MD, PhD;
  2. Aurelie Plessier, MD;
  3. Manuel Hernandez-Guerra, MD;
  4. Federica Fabris, MD;
  5. Chundamannil E. Eapen, MD;
  6. Matthias J. Bahr, MD;
  7. Jonel Trebicka, MD;
  8. Isabelle Morard, MD;
  9. Luc Lasser, MD;
  10. Joerg Heller, MD;
  11. Antoine Hadengue, MD;
  12. Philippe Langlet, MD;
  13. Helena Miranda, MD, PhD;
  14. Massimo Primignani, MD;
  15. Elwyn Elias, MD, BSc, FRCP;
  16. Frank W. Leebeek, MD, PhD;
  17. Frits R. Rosendaal, MD, PhD;
  18. Juan-Carlos Garcia-Pagan, MD, PhD;
  19. Dominique C. Valla, MD, PhD;
  20. Harry L.A. Janssen, MD, PhD; and
  21. for EN-Vie (European Network for Vascular Disorders of the Liver)*
  1. 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.

    Abstract

    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.

    Article and Author Information

    • 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{at}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{at}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{at}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.

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

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