HEART-TRANSPLANTATION IN CHILDREN - INDICATIONS AND OUTCOMES

To date, 92 heart transplants have been performed as part of the pedia tric heart transplant program started at the Necker Enfants Malades/La ennec Hospital in Paris in 1987. One-third of patients were younger th an two years of age at transplantation, and nine were younger than nin e months. The most common reason for transplantation was cardiomyopath y (64%), which was usually of the dilated hypokinetic type; 33 patient s (36%) had congenital heart defects. Maintenance immunosuppressant th erapy usually consisted of cyclosporin and azathioprine, with in some cases Low-dose corticosteroid therapy. The postoperative complication rate was high. The most common early complication was acute rejection, whose diagnosis relied primarily on endomyocardial biopsy. Infections were.-very common during the first six months; after a phase of domin ant bacterial infections, viral infections became more common. Cyclosp orin caused renal dysfunction in some cases. Immunosuppressant-induced lymphoproliferative disorders occurred in eight patients and were sev ere in five. The main long-term complication was coronary artery disea se, which was responsible for complete coronary artery occlusion in so me cases. Mortality rates were about 22% during the first month and 26 % subsequently. Most late deaths were due to coronary artery disease, rejection, or lymphoproliferative syndrome. Most survivors enjoyed an excellent quality of life and were able to engage in normal physical a ctivities.