What is the prognosis of Budd-Chiari syndrome?

Updated: Oct 10, 2018
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD  more...
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Answer

The 5-year survival rate for patients with the syndrome is 38-87% following portosystemic shunting. The actuarial 5-year survival rate following liver transplantation is 70%. [2, 3, 4] Long-term follow-up in adults has demonstrated 10-year survival rates as high as 55%.

The prognosis is poor, however, in patients with Budd-Chiari syndrome who remain untreated, with death resulting from progressive liver failure in 3 months to 3 years from the time of diagnosis. [1]

In a University of Pennsylvania retrospective study (2008-2013) comprising 47 patients with Budd-Chiari syndrome, there were no significant differences in the treatment outcomes among those receiving anticoagulation therapy alone, transjugular intrahepatic portosystemic shunt (TIPS) placement alone, and TIPS in conjunction with anticoagulation. [16] The investigators noted that the significant prognostic predictors for liver transplantation were age, presence of cirrhosis, and presence of chronic kidney disease.


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