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Original communication| Volume 135, ISSUE 4, P394-403, April 2004

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The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt

  • Martin Rössle
    Correspondence
    Reprint requests: Martin Rössle, MD, Professor of Medicine, Medizinische Klinik, University Hospital, Hugstetterstrasse 55, D-79106 Freiburg, Germany.
    Affiliations
    From the Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg; the Institute of Medical Biometry, University of Freiburg, Städtisches Klinikum Saarbücken (Grandt), Germany
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  • Manfred Olschewski
    Affiliations
    From the Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg; the Institute of Medical Biometry, University of Freiburg, Städtisches Klinikum Saarbücken (Grandt), Germany
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  • Volker Siegerstetter
    Affiliations
    From the Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg; the Institute of Medical Biometry, University of Freiburg, Städtisches Klinikum Saarbücken (Grandt), Germany
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  • Elisabeth Berger
    Affiliations
    From the Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg; the Institute of Medical Biometry, University of Freiburg, Städtisches Klinikum Saarbücken (Grandt), Germany
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  • Katharina Kurz
    Affiliations
    From the Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg; the Institute of Medical Biometry, University of Freiburg, Städtisches Klinikum Saarbücken (Grandt), Germany
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  • Daniel Grandt
    Affiliations
    From the Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg; the Institute of Medical Biometry, University of Freiburg, Städtisches Klinikum Saarbücken (Grandt), Germany
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      Abstract

      Background

      The role of portosystemic shunting in the treatment of the Budd-Chiari syndrome is still under debate. Medical therapy and liver transplantation are alternative treatments. The aim of this study was to determine the outcome of a transjugular intrahepatic portosystemic shunt implantation.

      Methods

      Thirty-five patients with severe Budd-Chiari syndrome and a Child-Pugh score of 9.2±1.9, who were not responsive to medical therapy, were elected for the transjugular shunt treatment, which was successfully accomplished in 33. Eleven patients had a fulminant/acute (history <2 months); 13, a subacute (<6 months); and 11, a chronic course of the disease. The shunt was established by using conventional self-expandable stents in 25 patients and polytetrafluoroethylene-covered stents in 8 patients. The mean follow-up was 37±29 months.

      Results

      The shunt reduced the portosystemic pressure gradient from 29±7 to 10±4 mm Hg and improved the portal flow velocity from 9.2±11 to 51±17 cm/s. Clinical symptoms as well as the biochemical test results improved significantly during 4 weeks after shunt treatment. Three patients died and 2 received liver transplants. The cumulative 1- and 5-year survival rate without transplantation in all patients was 93% and 74%, respectively, and in patients with fulminant/acute disease 91% and 91% respectively (no deaths in this time period). On the average, 1.4±2.2 revisions per patient were needed during the mean follow-up of 3 years with a 1-year probability of 47%.

      Conclusions

      The transjugular shunt provides an excellent outcome in patients with severe fulminant/acute, subacute, and chronic Budd-Chiari syndrome. It may be regarded as a treatment for the acute and long-term management of these patients.
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