Good Long-Term Outcome of Budd-Chiari Syndrome With a Step-Wise Management

Hepatology. 2013 May;57(5):1962-8. doi: 10.1002/hep.26306.

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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Anticoagulants / therapeutic use
  • Budd-Chiari Syndrome / diagnosis*
  • Budd-Chiari Syndrome / mortality
  • Budd-Chiari Syndrome / therapy*
  • Cohort Studies
  • Disease Management*
  • Europe
  • Female
  • Humans
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Portasystemic Shunt, Transjugular Intrahepatic
  • Prognosis
  • Prospective Studies
  • Retrospective Studies
  • Survival Rate
  • Thrombolytic Therapy
  • Young Adult

Substances

  • Anticoagulants