PEDIATRIC HEART-TRANSPLANTATION WITHOUT CHRONIC MAINTENANCE STEROIDS

From 1986 to February 1993, 40 children aged 2 months to 18 years (ave rage age 10.4 +/- 5.8 years) underwent heart transplantation. Indicati ons for transplantation were idiopathic cardiomyopathy (52%), congenit al heart disease (35%) with and without prior repair (71% and 29%, res pectively), hypertrophic cardiomyopathy (5%), valvular heart disease ( 3%), and doxorubicin cardiomyopathy (5%). Patients were managed with c yclosporine and azathioprine. No prophylaxis with antilymphocyte globu lin was used. Steroids were given to 39% of patients for refractory re jection, but weaning was always attempted and generally successful (64 %). Five patients (14%) received maintenance steroids. Four patients d ied in the perioperative period and one died 4 months later. There hav e been no deaths related to rejection or infection. Average follow-up was 36 +/- 19 months (range 1 to 65 months). Cumulative survival is 88 % at 5 years. In patients less than 7 years of age, rejection was moni tored noninvasively. In the first postoperative month, 89% of patients were treated for rejection. Freedom from serious infections was 83% a t 1 month and 65% at 1 year. Cytomegalovirus infections were treated s uccessfully with ganciclovir in 11 patients. No impairment of growth w as observed in children who underwent transplantation compared with a control population. Twenty-one patients (60%) have undergone annual ca theterizations and no sign of graft atherosclerosis has been observed. Seizures occurred in five patients (14%) and hypertension was treated in 10 patients (28%). No patient was disabled and no lymphoproliferat ive disorder was observed. When this group was compared with 87 heart transplant recipients who were more than 50 years of age and receiving no maintenance steroids, a better postoperative survival rate was fou nd (97% vs 84%; p < 0.05). Our experience suggests that immunosuppress ion without maintenance steroids might improve the prospects for prolo nged survival in the pediatric age group. Long-term survivors have an excellent potential for full rehabilitation.