Budd-Chiari syndrome is a rare and life-threatening disorder secondary to hepatic venous outflow obstruction.
Small series of Budd-Chiari syndrome patients indicate that transjugular intrahepatic portosystemic shunt may be useful.
|1-year liver transplant-free survival was 88%|
The influence of transjugular intrahepatic portosystemic shunt (TIPS) on patient survival, and factors that predict the outcome of transjugular intrahepatic portosystemic shunt in Budd-Chiari syndrome patients remain unknown.
Dr Juan Carlos Garcia–Pagán and colleagues from Spain followed 124 consecutive Budd-Chiari syndrome patients treated with transjugular intrahepatic portosystemic shunt.
The patients were observed in 6 European centers between 1993 and 2006 until death, orthotopic liver transplantation, or last clinical evaluation.
The researchers found that prior to treatment with transjugular intrahepatic portosystemic shunt, Budd-Chiari syndrome patients had a high Model of End Stage Liver Disease and high Rotterdam Budd-Chiari syndrome prognostic index indicating severity of liver dysfunction.
However, 1- and 5-year orthotopic liver transplantation-free survival were 88% and 78%, respectively.
The team found that in the high-risk patients, 5-year orthotopic liver transplantation-free survival was much better than that estimated by the Rotterdam Budd-Chiari syndrome index.
In the whole population, bilirubin, age, and international normalized ratio for prothrombin time independently predicted 1-year orthotopic liver transplantation-free survival.
A prognostic score with a good discriminative capacity was developed from these variables.
The team noted that 7 out of 8 patients with a score over 7 died or underwent transplantation vs 5 out of 114 patients with a score less than 7.
Dr Garcia–Pagán's team concluded, “Long-term outcome for patients with severe Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt is excellent even in high-risk patients, suggesting that transjugular intrahepatic portosystemic shunt may improve survival.”
“We identified a small subgroup of Budd-Chiari syndrome patients with poor prognosis despite transjugular intrahepatic portosystemic shunt who might benefit from early orthotopic liver transplantation.”