PROJECTIONS FOR PEDIATRIC HEART-LUNG AND LUNG TRANSPLANTATION

There is limited international experience in heart-lung or lung transplantation in children less than 16 years of age. Although the indications for lung transplantation are quite different in children compared with adults, the indications for heart-lung versus lung transplantation in children are quite similar. These indications consist of end-stage pulmonary disease, such as cystic fibrosis, or end-stage pulmonary vascular disease that is either primary or associated with correctable or corrected congenital heart disease. The results reported in the international registries show little difference in early and intermediate survival between children who underwent transplantation by the heart-lung or the lung techniques. Actuarial survival at 1 year is approximately 70%, with a survival at 2 years of 40% to 50%. These results are probably slightly inferior to the results in adults, even if differences in indications for transplantation are taken into account. Children may be more susceptible to rejection, infection, and viral challenges, the consequences of which may be more significant in pediatric lung transplantation than in pediatric heart transplantation. In addition, lymphoproliferative diseases are more common in children and may be harder to control by decreasing immunosuppressive therapy than in adults. In spite of these caveats, surviving children often have good functional results and a markedly improved quality of life after heart-lung and lung transplantation unless or until severe chronic rejection and obliterative bronchiolitis occur. The severe limitation of pediatric donor organs and distribution problems make maximum utilization of donor organs critical. For this reason, lung transplantation and heart repair offers some advantages over heart-lung transplantation; however, patients who require heart-lung transplantation must still be adequately served by the organ distribution network. The advantages of lung over heart-lung transplantation include the maximum utilization of scarce donor organs and the possible avoidance of coronary artery disease in the donor heart. Heart-lung transplantation is preferable to double or single lung transplantation techniques in children who have severe left ventricular dysfunction and possibly in children who require complex repairs because of congenital heart disease, increasing the likelihood of future reoperation. Single lung transplantation may have a particular role in children with diaphragmatic hernia or bronchopulmonary dypsplasia as a bridge to growth and development of the remaining normal lung. Although early results have been promising, heart-lung or lung transplantation in children must be considered palliative therapy for a majority of recipients.