BUDD-CHIARI SYNDROME

Abstract

Purpose: The long-term outcome of Budd-Chiari syndrome (BCS) with transjugular intrahepatic portosystemic shunts (TIPS) is not well studied. Methods: To address this we analyzed the records of 47 consecutive patients with BCS evaluated in one centre from January 1989 to April 2004. TIPS was introduced as a treatment option in November 1999. Results: Seven patients with liver tumors were excluded from analyses. Eleven patients had Bechet's disease, 14 had thrombophiliac disorders, 4 had myeloproliferative diseases and 11 patients had other or unknown causes. The site of block was in hepatic veins in 16 patients, in the suprhepatic inferior vena cava in 19 and not known in 5. Eight patients with membranes were treated with transluminal angiopalsty, with clinical and biochemical recovery in 6 patients. TIPS was placed through a transcaval puncture in 8 patients with progressive liver disease on medical therapy and thrombosis limited to hepatic veins. TIPS was very effective in decreasing protal pressure gradient, improving synthetic functions, reducing transaminase levels and controlloing ascites. Five patients had TIPS dysfunction needing revision. In 2 patients it was difficult to mantain TIPS patency due repeated TIPS dysfunction. However, both these patients were asymptomatic with normal liver function tests. Survival rates were 83 ± 7%, 66 ± 9%, and 58 ± 10% at 3, 12 and 36 months in 24 patients treated before introduction of TIPS. In contrast, corresponding survival rates in 16 patients treated after introduction of TIPS were 93.7 ± 6%, 75 ± 17% and 75 ± 17% (log-rank test, P = 0.04). In a multivariate Cox-model anlayses four variables namely increasing INR, Child-Pugh-Turcotte score and serum bilirubin; and florid clincial presentation were predictors of survival. Conclusions: BCS needs an individualized multidisciplinary approach and TIPS is indicated in a subgroup of patients with progressive liver disease. It is safe, feasible and improves survival.