Good long-term outcome of Budd-Chiari syndrome with a step-wise management

• DOI: 10.1002/hep.26306
• Publication Date: 2013-02-07T02:00:44Z
• Authors: Susana Seijo; Aurelie Plessier; Jildou Hoekstra; Alessandra Dell'Era; Dalvinder Mandair; Kinan Rifai; Jonel Trebicka; Isabelle Morard; Luc Lasser; Juan G. Abraldes; Sarwa Darwish Murad; Jörg Heller; Antoine Hadengue; Massimo Primignani; Elwyn Elias; Harry L.A. Janssen; Dominique C. Valla; Juan-Carlos Garci...
• Keywords: Budd–Chiari syndrome; Post-thrombotic syndrome

Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim the study was to assess long-term outcome and identify prognostic factors in BCS patients managed step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), orthotopic liver transplantation (OLT). We reviewed data on 157 previously included European Network for Vascular Disorders Liver, multicenter prospective newly diagnosed nine countries. Patients were followed median 50 months (range, 0.1-74.0). During study, 88 (56%) received at least one invasive intervention (22 62 TIPS, 20 OLT) 36 (22.9%) died. Most interventions and/or deaths occurred first 2 years after diagnosis. Rotterdam score excellent predicting intervention-free survival, no other variable could significantly improve its ability. Moreover, BCS-TIPS index (PI) (based international normalized ratio, bilirubin, age) strongly associated with survival had discriminative capacity, which superior score.The current confirms, large cohort recruited over short period, that treatment provides good survival. In addition, validates PI