title: Good long-term outcome of Budd-Chiari syndrome with a step-wise management.
abstract: Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim of the study was to assess long-term outcome and identify prognostic factors in BCS patients managed by a step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), and orthotopic liver transplantation (OLT). We reviewed long-term data on 157 patients previously included by the European Network for Vascular Disorders of the Liver, a multicenter prospective study of newly diagnosed BCS patients in nine European countries. Patients were followed for a median of 50 months (range, 0.1-74.0). During the study, 88 patients (56%) received at least one invasive intervention (22 patients angioplasty/thrombolysis, 62 TIPS, and 20 OLT) and 36 (22.9%) died. Most interventions and/or deaths occurred in the first 2 years after diagnosis. The Rotterdam score was excellent in predicting intervention-free survival, and no other variable could significantly improve its prognostic ability. Moreover, BCS-TIPS prognostic index (PI) score (based on international normalized ratio, bilirubin, and age) was strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score.
  
  
  CONCLUSIONS
  The current study confirms, in a large cohort of patients with BCS recruited over a short period, that a step-wise treatment approach provides good long-term survival. In addition, the study validates the Rotterdam score for predicting intervention-free survival and the BCS-TIPS PI score for predicting survival.
authors: Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic, IDIBAPS and CIBERehd, Barcelona, Spain.
authors: Susana
authors: S
authors: Seijo
authors: Aurelie
authors: A
authors: Plessier
authors: Jildou
authors: J
authors: Hoekstra
authors: Alessandra
authors: A
authors: Dell'era
authors: Dalvinder
authors: D
authors: Mandair
authors: Kinan
authors: K
authors: Rifai
authors: Jonel
authors: J
authors: Trebicka
authors: Isabelle
authors: I
authors: Morard
authors: Luc
authors: L
authors: Lasser
authors: Juan G
authors: JG
authors: Abraldes
authors: Sarwa
authors: S
authors: Darwish Murad
authors: Jörg
authors: J
authors: Heller
authors: Antoine
authors: A
authors: Hadengue
authors: Massimo
authors: M
authors: Primignani
authors: Elwyn
authors: E
authors: Elias
authors: Harry L A
authors: HL
authors: Janssen
authors: Dominique C
authors: DC
authors: Valla
authors: Juan-Carlos
authors: JC
authors: Garcia-Pagan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000925:Anticoagulants / Q000627:therapeutic use; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D015331:Cohort Studies; D019468:Disease Management; D005060:Europe; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D015996:Survival Rate; D015912:Thrombolytic Therapy; D055815:Young Adult
chemical_list: D000925:Anticoagulants