Objective: To review the clinical characteristics, outcomes, and risk
factors for survival among 57 pediatric patients undergoing orthotopic
liver transplantation for fulminant hepatic failure at the University
of California, Los Angeles, Center for the Health Sciences. Design: T
he medical records of 57 consecutive pediatric patients undergoing ort
hotopic liver transplantation for fulminant hepatic failure from July
1, 1984, to June 25, 1997, were reviewed and survival data were analyz
ed via univariate and multivariate statistical methods. The type and i
ncidence of posttransplant complications were determined as eras the q
uality of long-term graft function. Median follow-up period was 3.38 y
ears (range, 0-10.02 years). Results: The 1-, 3-, and 5-year actuarial
patient survival rates were 77%, 77%, and 77%, respectively, while gr
aft survivals were 73%, 65%, and 65%. Stepwise Cox regression analysis
revealed that recipient age and ventilator dependency at the time of
transplantation were independently and significantly correlated with p
atient survival, whereas no association was found between survival and
grade of encephalopathy, prior abdominal surgery, recipient weight, p
retransplantation values for total bilirubin or prothrombin time, ABO
match, allograft type, peak posttransplantation aspartate aminotransfe
rase levels, or the presence of posttransplantation hepatic artery thr
ombosis. Non-ventilator-dependent patients demonstrated a 96% 1-, 3-,
and 5-year survival as compared with only 56% at these same time point
s for those children requiring ventilator support at the time of trans
plantation (P<.001). At the time of most recent followup, median value
s for total bilirubin and aspartate aminotransferase concentrations we
re 10.3 mu mol/L (0.6 mg/dL) and 56 U/L, respectively, in the 40 survi
ving patients. Conclusions: In children undergoing liver transplantati
on for fulminant hepatic failure: (1) overall results are comparable t
o those achieved for less emergent nonneoplastic indications in this s
ame age group; (2) ventilator dependency prior to transplantation is t
he strongest predictor of ultimate survival, followed by recipient age
; (3) 5-year survival exceeds 90% in recipients who are ventilator ind
ependent immediately prior to liver transplantation but is significant
ly compromised once the need for mechanical ventilation supervenes, pa
rticularly in those younger than 4 years; and (4) prompt referral and
timely liver replacement are the cornerstones of optimal outcome.