Cardiac transplantation has become a more frequently used therapeutic
modality for select cardiac pathology in infants and children. Since J
une 1986, 30 pediatric patients (19 male and 11 female) ranging in age
from 4 days to 15 years (11 less than or equal to 1 month old) have u
ndergone orthotopic cardiac transplantation at our institution. Indica
tions included idiopathic cardiomyopathy (n = 8), hypoplastic left hea
rt syndrome (n = 13), and other forms of complex congenital heart dise
ase (n = 9). There have been four operative and three late deaths only
in the groups with hypoplastic left heart syndrome and other forms of
complex congenital heart disease. Cumulative survival is 77% after a
mean follow-up of 30 months (range, 6 to 77 months). Three operative d
eaths were attributable to pulmonary hypertension, and the other was d
ue to pulmonary hemorrhage. Two late deaths were secondary to allograf
t rejection, and the third was due to infection. There has been unifor
m survival in the group with idiopathic cardiomyopathy, and intermedia
te-term survival rates are 78% and 62% in the groups with complex cong
enital heart diseases and hypoplastic left heart syndrome, respectivel
y (p = 0.15). Although longer-term results are necessary, orthotopic c
ardiac transplantation appears to be an acceptable mode of therapy for
endstage heart disease in the pediatric age group and technically can
be performed despite complex malformations of the great arteries or a
tria.