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Good long‐term outcome of Budd‐Chiari syndrome with a step‐wise management
Author(s) -
Seijo Susana,
Plessier Aurelie,
Hoekstra Jildou,
Dell'Era Alessandra,
Mandair Dalvinder,
Rifai Kinan,
Trebicka Jonel,
Morard Isabelle,
Lasser Luc,
Abraldes Juan G.,
Darwish Murad Sarwa,
Heller Jörg,
Hadengue Antoine,
Primignani Massimo,
Elias Elwyn,
Janssen Harry L.A.,
Valla Dominique C.,
GarciaPagan JuanCarlos
Publication year - 2013
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.26306
Subject(s) - medicine , budd–chiari syndrome , liver transplantation , post thrombotic syndrome , surgery , cohort , transplantation , thrombosis , venous thrombosis , inferior vena cava
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. (H EPATOLOGY 2013;)