From July 1990 to April 1993, 36
lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a
ventilator for 3 days to 4.5 years before
transplantation and three were supported by
extracorporeal membrane oxygenation. Indications for
lung transplantation in this pediatric population included the following:
cystic fibrosis (n = 13),
pulmonary hypertension, and associated
congenital heart disease (n = 10),
pulmonary atresia,
ventricular septal defect and nonconfluent pulmonary arteries (n = 3),
pulmonary fibrosis (n = 6), and
acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1).
Pulmonary fibrosis was related to complications of treatment of acute of
myelogenous leukemia with
bone marrow transplantation in two children and to
bronchiolitis obliterans,
bronchopulmonary dysplasia,
interstitial pneumonitis, and
Langerhans cell histiocytosis in four others. Thirteen children underwent
lung transplantation and concomitant cardiac repair. Bilateral
lung transplantation,
ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined
heart-lung transplantation. Two patients had
ventricular septal defect closure and single lung transplant for
Eisenmenger's syndrome, two had
ligation of a
patent ductus arteriosus and
transplantation, three additional children underwent
atrial septal defect closure and
lung transplantation, and two underwent
lung transplantation for congenital
pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%).
Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary.
Lung transplantation and repair of complex
congenital heart defects is possible;
heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary
vascular disease.
Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.