Objective This study was undertaken to review the incidence and causes
of death in children who have survived long-term (more than 1 year) a
fter liver transplantation (LT). Summary Background Data No studies of
the causes of late mortality in pediatric LT recipients are currently
available in the literature. Methods The study group consists of 212
pediatric patients who survived more than 1 year after LT. Twenty-thre
e of these patients subsequently died (mean follow-up = 5.3 yr). Hospi
tal records, office charts, and autopsy records were reviewed retrospe
ctively to identify the causes of death. The patients who died were fu
rther evaluated by age, gender, length of survival, primary diagnosis,
immunosuppression, and retransplantation. Results The most common cau
se of death was graft failure, followed closely by infection. In patie
nts dying from graft failure, eight of the nine patients underwent ret
ransplantation and no child survived more than three liver transplants
. Overwhelming infections occurred suddenly in eight children who had
been previously healthy. Noncompliance was the third most common cause
of death, primarily in older children. One child died from a posttran
splant lymphoproliferative disorder (PTLD). Actuarial survival at 10 y
ears is 83.7% (based on 100% survival at 1 year). There was no differe
nce in survival based on primary disease. Retransplantation was far mo
re prevalent in the nonsurvivors (47.8%) compared with survivors (13.7
%) (p < 0.05). There were no significant differences in survival based
on age, gender, or immunosuppression. Conclusions Late mortality in c
hildren continues to be directly related to complications of LT and im
munosuppression, even after the first year of transplantation. This is
in contrast to adult liver transplant recipients, where approximately
50% of late deaths were related to LT and the remainder were because
of unrelated illnesses.