Survival and prognostic indicators of Budd–Chiari syndrome: a systematic review of 79 studies

Abstract

This paper aimed to systematically review the survival of Budd–Chiari syndrome and to identify the most robust prognostic predictors. Overall, 79 studies were included. According to the treatment modalities, the median 1-, 5- and 10-year survival rate was 93, 83 and 73% after interventional radiological treatment; 81, 75 and 72.5% after surgery other than liver transplantation; 82.5, 70.2 and 66.5% after liver transplantation and 68.1, 44.4% and unavailable after medical therapy alone. According to the publication years, the median 1-, 5- and 10-year survival rate was 68.6, 44.4% and unavailable before 1990; 75.1, 69.5 and 57% during the year 1991–1995; 77, 69.6 and 65.6% during the year 1996–2000; 86.5, 74 and 63.5% during the year 2001–2005 and 90, 82.5 and 72% after 2006. Bilirubin, creatinine and ascites were more frequently identified as significant prognostic factors in univariate analyses. But their statistical significance was less frequently achieved in multivariate analyses.

Keywords:

• hepatic vein
• hepatic venous outflow obstruction
• inferior vena cava
• predict
• prognosis

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Key issues

• The survival of Budd–Chiari syndrome (BCS) patients was being gradually improved over time.

• BCS patients receiving interventional radiological treatments could achieve an excellent survival.

• Bilirubin, creatinine and ascites might be more closely associated with the survival of BCS. However, no definitive prognostic algorithms have been established yet.

Notes