CRITICAL ISSUES IN PEDIATRIC LUNG TRANSPLANTATION

Forty children (aged 1 to 18 years, 27 female and 13 male) have underg one heart-lung (21), double lung (17), and single lung (2) transplant procedures at our center from 1985 through April 1994. The indications for transplantation have been diverse, primary pulmonary hypertension (10), cystic fibrosis (11), congenital heart disease (10), arterioven ous malformation (3), emphysema (1), graft-versus-host disease (1), rh eumatoid lung (1), cardiomyopathy (1), desquamative interstitial pneum onitis (1), and Proteus syndrome (1). The actuarial 1-year survival wa s 73% (mean follow-up 2 years). One-year actuarial survival for diseas e groups ranged from 60% for cystic fibrosis to 90% for congenital hea rt disease. We have identified six issues critical to the patient and programatic survival of pediatric lung transplantation. Our experience and management strategies in these areas are reviewed. Cytomegaloviru s: Cytomegalovirus disease developed in six of eight patients with cyt omegalovirus mismatching (donor +/recipient-) and in seven of 32 patie nts who survived more than 30 days (23%). All but cytomegalovirus dono r -/recipient- patients were treated with ganciclovir for 4 weeks afte r transplantation. Obliterative bronchiolitis: Obliterative bronchioli tis developed in seven of 32 (25%) patients who survived more than 30 days, Obliterative bronchiolitis was manifest within the first posttra nsplantation year as a rapid decline in small airway function. Aggress ive augmentation of immunosuppression has been used with little succes s. Posttransplantation lymphoproliferative disease: Posttransplantatio n lymphoproliferative disease developed in five of 32 (15%) patients w ho survived more than 30 days developed. One patient died (17% mortali ty) despite retransplantation. In four patients the disease resolved w ith reduction in immunosuppression alone, and one required the additio n of interferon alfa. Cystic fibrosis: We have changed our management strategies to avoid triple drug immunosuppression, perioperative blood and bronchial cultures, aggressive antimicrobial therapy, and exclusi on of patients with panresistant organisms; this has resulted in elimi nation of infectious mortalities thus far in the pediatric cystic fibr osis group. Airways: In 21 heart-lung recipients with tracheal anastom oses we have had no airway complications. The double and single lung t ransplant recipients accounted for 34 bronchial and one tracheal anast omoses. Three (9%) bronchial stenoses developed. Two were treated with silicone stents and one with balloon dilation. Finances: The average charge for lung transplant evaluation was $18,000 and for transplantat ion, $175,000.