Despite improvement in surgical and medical management of children with con
genital and acquired heart disease, cardiac transplantation remains an impo
rtant therapeutic option for infants and children with end-stage heart dise
ase. Ultimate survival in patients who are listed for transplantation is a
function of both mortality while awaiting transplantation and survival afte
r transplantation. Survival of heart transplantation is affected by the sev
erity of illness before transplantation, the unique pathophysiology of cert
ain defects, and the availability of donor hearts. Outcome following listin
g for transplantation is best studied with the use of recent modifications
in statistical methods of competing outcomes analysis. By this analysis a p
redicted mortality while waiting among all pediatric patients is 20% at 1 y
ear, with 67% undergoing transplantation, 10% still on the list awaiting tr
ansplant, and 3% removed from the list. Among infants, most of them with hy
poplastic left heart syndrome, 60% will have transplantation by 6 months af
ter listing, with 27% of patients dying while waiting. In infants the major
risk factors for death while waiting are the need for inotropic support at
listing, smaller size, and recipient blood type. In older children risk fa
ctors for death while waiting are Status 1 at listing and a need for mechan
ical ventilation. Intermediate-term survival after transplant is excellent
in all age groups with 86% alive at 6 months, 84% at 1 year, and 73% at 5 y
ears. Survival after transplant in infants is comparable to survival in old
er children, although the early mortality after transplantation is greater.
Infants who have recently undergone sternotomy or received organs from don
ors who did not die of closed head trauma are more likely to die early afte
r transplant. Among older children risk factors for death after transplanta
tion include the need for a mechanical support device or a younger age in p
atients greater than 1 year of age. Death following transplantation is prim
arily related to early graft failure in infants, whereas rejection, infecti
on, and sudden death account for the majority of deaths in older children.
Although improved immunosuppressive agents promise to lead to even better s
urvival rates after transplantation, greater access to donors is essential
if overall survival is to be improved. (C) 2000 Elsevier Science Ireland Lt
d. All rights reserved.