The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt

Background

The role of portosystemic shunting in the treatment of the Budd-Chiari syndrome is still under debate. Medical therapy and liver transplantation are alternative treatments. The aim of this study was to determine the outcome of a transjugular intrahepatic portosystemic shunt implantation.

Methods

Thirty-five patients with severe Budd-Chiari syndrome and a Child-Pugh score of 9.2±1.9, who were not responsive to medical therapy, were elected for the transjugular shunt treatment, which was successfully accomplished in 33. Eleven patients had a fulminant/acute (history <2 months); 13, a subacute (<6 months); and 11, a chronic course of the disease. The shunt was established by using conventional self-expandable stents in 25 patients and polytetrafluoroethylene-covered stents in 8 patients. The mean follow-up was 37±29 months.

Results

The shunt reduced the portosystemic pressure gradient from 29±7 to 10±4 mm Hg and improved the portal flow velocity from 9.2±11 to 51±17 cm/s. Clinical symptoms as well as the biochemical test results improved significantly during 4 weeks after shunt treatment. Three patients died and 2 received liver transplants. The cumulative 1- and 5-year survival rate without transplantation in all patients was 93% and 74%, respectively, and in patients with fulminant/acute disease 91% and 91% respectively (no deaths in this time period). On the average, 1.4±2.2 revisions per patient were needed during the mean follow-up of 3 years with a 1-year probability of 47%.

Conclusions

The transjugular shunt provides an excellent outcome in patients with severe fulminant/acute, subacute, and chronic Budd-Chiari syndrome. It may be regarded as a treatment for the acute and long-term management of these patients.