Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt

The long-term outcome of Budd-Chiari syndrome (BCS) with transjugular intrahepatic portosystemic shunts (TIPS) is not well studied. To address this, the records of 47 consecutive patients with BCS evaluated in one center from January 1989 to April 2004, were analyzed.

Seven patients with liver tumors were excluded from analyses. Eleven patients had Bechet's disease, 14 had thrombophiliac disorders, four had myeloproliferative diseases and 11 patients had other or unknown causes. The site of block was hepatic vein in 16 patients, in the suprhepatic inferior vena cava in 19 and not known in five. The majority of patients (21/40; 52.5%) presented with subacute disease with massive ascites and abdominal pain as the dominant manifestations. Eight patients with membranes or segemental block were treated with transluminal angiopalsty, and six were treated with clinical and biochemical recovery. The TIPS was placed through a transcaval puncture in eight patients with progressive liver disease who were on medical therapy and had thrombosis limited to hepatic veins. One patient bled from portal vein puncture, which was managed by placing stent across the punctured site. The TIPS was very effective in decreasing portal pressure gradient, improving synthetic functions, reducing transaminase levels and controlling ascites. Five patients had TIPS dysfunction needing revision. In two patients it was difficult to maintain TIPS patency due to repeated TIPS dysfunction. However, both these patients were asymptomatic with normal liver function tests. Long-term follow up revealed that patients with TIPS had significantly better survival than those treated with medical therapy alone (log-rank test, P = 0.04). In a multivariate Cox-model analysis four variables, namely, more florid presentation, male sex, no treatment with TIPS and increasing Child-Pugh-Turcotte score, adversely affected the survival.

Budd-Chiari syndrome 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.