Long-term portosystemic shunt patency as a determinant of outcome in Budd–Chiari syndrome

Background/Aims Portosystemic shunting, whether surgical or transjugular intrahepatic, has been a cornerstone of therapy for Budd–Chiari syndrome. However, the long-term impact of shunt dysfunction remains unknown. We have assessed this long-term impact in patients with surgical shunting. Methods Thirty-nine consecutive patients operated on between 1978 and 2000 were analyzed using time-dependent multivariate Cox model. Results Median follow-up was 110 months. Prosthetic shunts and high preshunt portal venous pressure were predictors of subsequent shunt dysfunction. Among 19 patients with persistently patent shunt, as compared to 20 patients with shunt dysfunction, 1 versus 18 developed refractory ascites; 1 versus 7 had variceal bleeding; 7 versus 2 had encephalopathy; 3 versus 11 (55%) died or underwent liver transplantation; and 0 versus 10 died from end-stage liver disease. Shunt dysfunction was associated with a shorter survival (p = 0.001). Out of 20 patients with shunt dysfunction, seven had successful revision of the shunt. None of these seven patients had refractory ascites after revision or died from end-stage liver disease. Conclusions In patients with Budd–Chiari syndrome treated with portosystemic shunting, shunt dysfunction has a major impact on morbidity and mortality.