TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy

Authors

  • Antonia Perelló,

    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Juan Carlos García-Pagán M.D.,

    Corresponding author
    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
    • Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, C/ Villarroel 170, Barcelona 08036, Spain. fax: (34) 93 451 55 22.
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  • Rosa Gilabert,

    1. Centre de Diagnòstic per la Imatge; and, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Yanette Suárez,

    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Eduardo Moitinho,

    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Francisco Cervantes,

    1. Hematology Service, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Juan Carlos Reverter,

    1. Hemotherapy and Hemostasis Service; Hospital Clinic, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Angels Escorsell,

    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Jaume Bosch,

    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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  • Juan Rodés

    1. Hepatic Hemodynamic Laboratory, Liver Unit, Institut de Malalties Digestives, IDIBAPS, University of Barcelona, Barcelona, Catalunya, Spain
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Abstract

Patients with Budd-Chiari syndrome (BCS) may require treatment with portal decompressive surgery or liver transplantation. Transjugular intrahepatic portosystemic shunt (TIPS) represents a new treatment alternative, but its long-term effect on BCS outcome has not been evaluated. Twenty-one patients with BCS consecutively admitted to our unit were evaluated. The mean follow-up was 4 ± 3 years. Seven patients had nonprogressive forms and were successfully controlled with medical therapy; 1 case, with a short-length hepatic vein stenosis was successfully treated by angioplasty. All 8 patients are alive and asymptomatic. The remaining 13 patients, had a TIPS because of clinical deterioration (in one of them, because early TIPS thrombosis a successful side-to-side portacaval shunt [SSPCS] was performed) followed by an improvement in clinical condition. However, a patient with fulminant liver failure before TIPS insertion, died 4 months later and another patient with cirrhosis at diagnosis had liver transplantation 2 years later. The remaining 11 patients are alive and free of ascites. In 3 of these patients TIPS is patent after 3, 6, and 12 months. The remaining 8 patients developed late TIPS dysfunction. In two of these cases, after angioplasty and restenting, TIPS is patent after a follow-up of 9 and 80 months. In 5 other patients, recurring TIPS occlusion was not further corrected because no signs of portal hypertension were present. In conclusion, in patients with BCS uncontrolled with medical therapy, TIPS is a highly effective technique that is associated with long-term survival.

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