title: The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt.
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.
authors.affiliation: Departments of Gastroenterology and Hepatology, the University Hospital of Freiburg, Hugstetterstrasse 55, D-79106 Freiburg, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Rössle
authors.forename: Manfred
authors.initials: M
authors.lastname: Olschewski
authors.forename: Volker
authors.initials: V
authors.lastname: Siegerstetter
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Berger
authors.forename: Katharina
authors.initials: K
authors.lastname: Kurz
authors.forename: Daniel
authors.initials: D
authors.lastname: Grandt
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D006502:Budd-Chiari Syndrome / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic / Q000379:methods*; D016896:Treatment Outcome