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

par 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 H.L.A.;Valla, Dominique;Garcia-Pagan, Juan-Carlos
Référence Hepatology, 57, 5, page (1962-1968)
Publication Publié, 2013-05

Article révisé par les pairs

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Résumé :

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. (HEPATOLOGY 2013;) Copyright © 2013 American Association for the Study of Liver Diseases.