Background: Cardiac transplantation is an acceptable therapeutic alternative 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: four with dilated cardiomyopathy, one with cardiac fibroma and two with hypertrophic cardiomyopathy. Age at transplantation ranged from 2 months to 13 years and 5 months, with a follow-up ranging from 15 months to 5 years and 9 months. Prophylaxis of acute rejection consisted of cyclosporine, azathioprine and glucocorticoids.
Results: Two patients presented acute rejection three weeks after cardiac transplantation, with a good response to high dose glucocorticoids. Two patients developed severe infection (sepsis by Staphylococcus aureus) with successful outcome after antibiotic treatment. One patient died in the early postoperative period and other after 4 years 11 months postransplantation because myelodysplastic syndrome. At present only one case is receiving glucocorticoids in immunoprophylaxis. The status is asymptomatic in the other 5 patients with a normal height-weight development.
Conclusions: Heart transplantation provides durable therapy for congenital and myopathic heart disease in infants and children with an excellent quality of life.