BACKGROUND: Cardiac transplantation is an acceptable therapeutic alter
native for cardiac diseases refractory to other forms of management in
adults as well as in infants and children. METHODS: Between 1987-1992
7 children (4 girls and 3 boys) underwent cardiac transplantation: fo
ur with dilated cardiomyopathy, one with cardiac fibroma and two with
hypertrophic cardiomyopathy. Age at transplantation ranged from 2 mont
hs to 13 years and 5 months, with a follow up ranging from 15 months t
o 5 years and 9 months. Prophylaxis of acute rejection consisted of cy
closporine, azathioprine and glucocorticoids. RESULTS: TWO patients pr
esented acute rejection three weeks after cardiac transplantation, wit
h a good response to high dose glucocorticoids. Two patients developed
severe infection (sepsis by Staphylococcus aureus) with successful ou
tcome after antibiotic treatment. One patient died in the early postop
erative period and other after 4 years II months postransplantation be
cause myelodysplastic syndrome. At present only one case is receiving
glucocorticoids in immunoprophylaxis. The status is asymptomatic in th
e other 5 patients with a normal height-weight development. CONCLUSION
S: Heart transplantation provides durable therapy for congenital and m
yophatic heart disease in infants and children with an excellent quali
ty of life.