title: Twenty years of liver transplantation for Budd-Chiari syndrome: a national registry analysis.
abstract: Several treatment options exist for the management of Budd-Chiari syndrome (BCS), yet the relative role and timing of liver transplantation (LT) remain poorly defined. Small case series published to date have not been able to delineate the impact of comorbidities and thromboembolic complications of BCS on survival after LT. To better understand the outcomes after LT for BCS, we analyzed 510 liver transplants performed for this disease in the United States between 1987 and 2006. Risk factors predicting graft loss or patient death included increased recipient age, hyperbilirubinemia, elevated creatinine, life support or hospitalization at the time of transplantation, prior transplantation, prior abdominal surgery, increased donor age, and prolonged cold ischemic time (CIT). Prior transjugular intrahepatic portosystemic shunt (TIPS) was not associated with worse outcomes. Transplantation in the Model for End-Stage Liver Disease (MELD) era was associated with significantly lower risk of graft loss (hazard ratio [HR], 0.50; 95% confidence interval [CI], 0.30-0.86; P = 0.012) and death (HR, 0.52; 95% CI, 0.29-0.93; P = 0.027). Similarly, MELD era was associated with significantly lower risk of early graft loss (odds ratio [OR], 0.35; 95% CI, 0.16-0.79, P = 0.012) and early death (odds ratio, 0.37; 95% CI, 0.14-0.95; P = 0.040). However, patients with BCS transplanted in the MELD era were less likely to have life support, hospitalization, prior transplants, and prolonged cold ischemia times. In conclusion, outcomes of LT for BCS are excellent, with further improvements since 2002 associated with a selection shift imposed by MELD-based organ allocation.
authors.affiliation: Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA. dorry@jhmi.edu
authors.forename: Dorry L
authors.initials: DL
authors.lastname: Segev
authors.forename: Geoffrey C
authors.initials: GC
authors.lastname: Nguyen
authors.forename: Jayme E
authors.initials: JE
authors.lastname: Locke
authors.forename: Christopher E
authors.initials: CE
authors.lastname: Simpkins
authors.forename: Robert A
authors.initials: RA
authors.lastname: Montgomery
authors.forename: Warren R
authors.initials: WR
authors.lastname: Maley
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Thuluvath
mesh_terms: D006502:Budd-Chiari Syndrome / Q000601:surgery*; D002102:Cadaver; D006085:Graft Survival; D006801:Humans; D017093:Liver Failure / Q000601:surgery; D016031:Liver Transplantation / Q000401:mortality / Q000502:physiology / Q000706:statistics & numerical data* / Q000639:trends; D012042:Registries; D012307:Risk Factors; D016019:Survival Analysis; D014019:Tissue Donors / Q000706:statistics & numerical data*; D016896:Treatment Outcome; D014481:United States