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Fig. 13D.1. Age distribution of pediatric lung recipients by year of transplant. (From Boucek MM et al. The Registry of the International Society of Heart and Lung Transplantation: Fifth Official Pediatric Report-2001 to 2002. J Heart Lung Transplant 2002; 21:827-840.)

Fig. 13D.2. Indications for pediatric lung transplant beween 1991 and 2001, by age of recipient. (From Boucek MM et al. The Registry of the International Society of Heart and Lung Transplantation: Fifth Official Pediatric Report-2001 to 2002. J Heart Lung Transplant 2002; 21:827-840.)

common indication. Among adolescents, the principal indication for lung transplantation is cystic fibrosis (CF). It is important to note that diagnoses leading to end-stage parenchymal disease in children differ from adults (Fig. 13D.2). Leading indications for transplantation in adults include pulmonary fibrosis and emphysema, both of which are uncommon in children.

Most patients with CF survive well into adulthood with current medical regimens. However, a relatively small percentage of patients with CF become refractory to medical therapy and require lung transplantation as children and adolescents. Indications for pediatric lung transplantation secondary to CF include increasingly frequent hospitalizations for pulmonary infections and pulmonary distress, progressive weight loss despite nutritional supplementation, and worsening gas exchange evidenced by progressive hypercapnia and hypoxemia.

Pulmonary hypertension (PH) may be primary or secondary to congenital heart disease. Primary PH is uncommon in children and is seldom life threatening during childhood. Therefore, children undergoing lung transplantation for PH usually have secondary PH arising from congenital heart disease. Pulmonary hypertension ultimately progresses to loss of exercise tolerance, cyanosis, poly-cythemia, and right heart failure. If lung transplantation is indicated because of a correctable cardiac anomaly, the cardiac defect should be repaired at the time of

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