title: Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd-Chiari syndrome.
abstract: BACKGROUND
  There have been encouraging reports on transjugular intrahepatic portosystemic stent-shunt (TIPSS) for Budd-Chiari syndrome (BCS). Long-term data are lacking.
  
  
  OBJECTIVE
  To assess long-term outcomes and validate prognostic scores following TIPSS for BCS.
  
  
  METHODS
  A single centre retrospective study. Patients underwent TIPSS using bare or polytertrafluoroethane (PTFE)-covered stents.
  
  
  RESULTS
  Sixty-seven patients received successful TIPSS between 1996 and 2012 using covered (n = 40) or bare (n = 27) stents. Patients included had a Male: Female ratio of 21:46, and were characterised (mean ± s.d.) by age 39.9 ± 14.3 years, Model of end stage liver disease (MELD) 16.1 ± 7.0 and Child's score 8.8 ± 2.0. Seventy-eight percent had haematological risk factors. Presenting symptoms were ascites (n = 61) and variceal bleeding (n = 6). Nine patients underwent hepatic vein dilatation or stenting prior to TIPSS. Mean follow-up was 82 months (range 0.5-184 months). Fifteen percent had post-TIPSS encephalopathy. Two have been transplanted. Primary patency rates (76% vs. 27%, P < 0.001) and shunt re-interventions (22% vs. 100%, P < 0.001) significantly favoured covered stents. Secondary patency was 99%. Six-, 12-, 24-, 60- and 120-month survival was 97%, 92%, 87%, 80% and 72% respectively. Six patients had liver related deaths. Two patients developed hepatocellular carcinoma. The BCS TIPS PI independently predicted mortality in the whole cohort, but no prognostic score was a significant predictor of mortality after subgroup validation.
  
  
  CONCLUSIONS
  Long-term outcomes following TIPSS for Budd-Chiari syndrome are very good. PTFE-covered stents have significantly better primary patency. The value of prognostic scores is controversial. TIPSS should be considered as first line therapy in symptomatic patients in whom hepatic vein patency cannot be restored.
authors.affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, UK.
authors.forename: D
authors.initials: D
authors.lastname: Tripathi
authors.forename: R
authors.initials: R
authors.lastname: Macnicholas
authors.forename: C
authors.initials: C
authors.lastname: Kothari
authors.forename: L
authors.initials: L
authors.lastname: Sunderraj
authors.forename: H
authors.initials: H
authors.lastname: Al-Hilou
authors.forename: B
authors.initials: B
authors.lastname: Rangarajan
authors.forename: F
authors.initials: F
authors.lastname: Chen
authors.forename: K
authors.initials: K
authors.lastname: Mangat
authors.forename: E
authors.initials: E
authors.lastname: Elias
authors.forename: S
authors.initials: S
authors.lastname: Olliff
mesh_terms: D000328:Adult; D001201:Ascites / Q000209:etiology / Q000473:pathology; D006502:Budd-Chiari Syndrome / Q000503:physiopathology / Q000601:surgery*; D058625:End Stage Liver Disease / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005465:Fluorocarbon Polymers / Q000737:chemistry; D005500:Follow-Up Studies; D006503:Hepatic Veins; D006801:Humans; D008297:Male; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic / Q000379:methods*; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D015607:Stents; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D005465:Fluorocarbon Polymers; C010946:polytetrafluoroethane