In 1989, we reported on the efficacy of liver transplantation in prima
ry biliary cirrhosis (PBC) by demonstrating that the actual patient su
rvival following transplantation was significantly better than without
transplantation as predicted by a mathematical survival model (''Mayo
natural history model''). Our aim in this investigation was to determ
ine an optimal time to perform liver transplantation in PBC. One hundr
ed forty-three patients with PEC undergoing liver transplantation were
followed prospectively. Disease severity was measured immediately bef
ore transplantation by a summary score (''risk score'') used in the Ma
yo natural history model, namely age, bilirubin, albumin, prothrombin
time, and the presence or absence of edema. Proportional hazards analy
ses were performed assessing patient survival following transplantatio
n. The influence of disease severity immediately pretransplantation on
resource utilization for liver transplantation was assessed. Compared
with our report in 1989, liver transplantation was performed at an ea
rlier stage of disease (e.g., median risk score: 7.5 vs. 8.3; P < .01)
. Following transplantation, patient survival probabilities at 1, 2, a
nd 5 years were 93%, 90%, and 88%, respectively. In the proportional h
azards analysis, the risk of death following transplantation remained
low until reaching a risk score of 7.8. In contrast, risk scores great
er than 7.8 were associated with a progressively increased mortality.
Resource utilization measured by the days in the intensive care unit (
ICU) and hospital and the requirement for intraoperative blood transfu
sions was significantly greater in recipients who had higher risk scor
es before transplantation. Our data suggest that an optimal timing for
liver transplantation, as determined by patient survival and resource
utilization, appears to be at a risk score around 7.8 in patients wit
h PBC.