Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival

Abstract

Background Budd-Chiari syndrome carries significant mortality, but factors predicting this outcome are uncertain. Aim To determine factors associated with 3-month mortality and compare outcomes after surgical shunting or liver transplantation. Methods From 1985 to 2008, 51 patients with Budd-Chiari syndrome were identified. Results By logistic regression analysis, features associated with higher risk of 3-month mortality were Rotterdam class III, Clichy >6.6, model for end-stage liver disease (MELD) >20 and Child-Pugh C. Rotterdam class III had the best performance to discriminate 3-month mortality with sensitivity of 0.89 and specificity of 0.63, whereas Clichy >6.60 had sensitivity of 0.78 and specificity of 0.69; MELD >20 had sensitivity of 0.78 and specificity of 0.75 and Child-Pugh C had sensitivity of 0.67 and specificity of 0.72. Eighteen patients underwent surgical shunts and 14 received liver transplantation with no significant differences in survival (median survival 10 ± 3 vs. 8 ± 2 years; log-rank, P = 0.9). Conclusions Rotterdam score is the best discrimination index for 3-month mortality in Budd-Chiari syndrome and should be used preferentially to determine treatment urgency. Surgical shunts constitute an important therapeutic modality that may help save liver grafts and prolong transplantation-free survival in a selected group of patients with Budd-Chiari syndrome. © 2009 Blackwell Publishing Ltd.