The decade from 1982 through 1992 witnessed tremendous growth in pediatric
cardiac transplantation. At Children's Hospital of Pittsburgh 66 cardiac transplants were performed during this period (age range 7 hours to 18 years). The cause of
cardiomyopathy was congenital (
n = 30),
cardiomyopathy (n = 29),
myocarditis (n = 2),
doxorubicin toxicity (n = 2), ischemic (n = 1), valvular (n = 1), and cardiac
angiosarcoma (n = 1). Nine children (14%) required mechanical circulatory support before
transplantation:
extracorporeal membrane oxygenation (n = 8) and Novacor left ventricular assist system (n = 1) (Baxter Healthcare Corp., Novacor Div., Oakland, Calif.). The mean follow-up time was 2 years (range 4 months to 8 years). The overall survival in the group was 67%. In children with
congenital heart disease (> 6 months of age) the perioperative (30 day) mortality was 66% before mid-1988 (n = 10) and 0% since mid-1988 (n = 11). The late mortality (> 30 days) in children with
cardiomyopathy transplanted prior to mid-1988 was 66% (n = 14) and 7% since mid-1988 (n = 15). Since mid-1988 1- and 3-year survival was 82% in children with
congenital heart disease and 90% in children with
cardiomyopathy. Twenty-six children have had
FK 506 as their primary immunosuppressive therapy since November 1989. Survival in this group was 82% at 1 and 3 years. The actuarial freedom from grade 3A rejection in the FK group was 60% at 3 and 6 months after
transplantation versus 20% and 12%, respectively, in the 15 children operated on before the advent of
FK 506, who were treated with
cyclosporine-based triple-
drug therapy (p < 0.001, Mantel-Cox and Breslow). Twenty of 24 children (83%) in the
FK 506 group are receiving no
steroids. The prevalence of posttransplantation
hypertension was 4% in the
FK 506 group versus 70% in the
cyclosporine group (p < 0.001, Fisher). Renal toxicity in children treated with
FK 506 has been mild. Additionally, eight children have been switched to
FK 506 because of refractory rejection and
drug toxicity.
FK 506 has not produced
hirsutism,
gingival hyperplasia, or abnormal facial bone growth. The absence of these debilitating side effects, together with the observed immune advantage and
steroid-sparing effects of
FK 506, hold tremendous promise for the young patient facing
cardiac transplantation and a future wedded to immunosuppression.