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. (HEPATOLOGY 2013;57:1962-1968

Timing of transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: An Italian hepatologist’s perspective

“…Moreover, patients receiving only medical therapy often have a bad outcome. In fact, the widest longterm multicenter experience on BCS reported that almost 30% of patients who received only medical therapy died, a high rate considering the availability of further effective invasive treatments [8] . Furthermore, a recent systematic review confirmed the bad outcome of BCS on only medical therapy, with survival worsening during long-term follow up in patients on only medical therapy [46] .…”

“…In fact, hepatocellular carcinoma (HCC) development is surely a concern during follow up of BCS [6] . Moreover, the outcome of underlying disease is other issue, since about 10% of the patients are reported to die because of hematological disease progression [7,8] . Finally, both different sources of bleeding [9] and thrombosis of other organs [8] are further complications potentially affecting BCS outcome.…”

“…Moreover, the outcome of underlying disease is other issue, since about 10% of the patients are reported to die because of hematological disease progression [7,8] . Finally, both different sources of bleeding [9] and thrombosis of other organs [8] are further complications potentially affecting BCS outcome. Since BCS is rare, both classification and management are empirical and not evidence-based, and follow opinion leaders' suggestion [1][2][3][4] .…”

Budd-chiari syndrome management: Timing of treatment is an open issue

“…1 As stated before, medical therapy is suggested as the first-line treatment, angioplasty/stenting the second-line, transjugular intrahepatic portosystemic shunt (TIPS) the further step, and liver transplantation (LT) the last chance. 2 Step-wise management suggests moving forward when no response to therapy appears.…”

“…Overall survival was 74% at 5 years. 1 TIPS is the most used treatment for BCS 2,3 because it is effective also in the case of thrombosis extension to the portal vein tree. [5][6][7] However, timing for TIPS was not stated.…”