Forty children (aged 1 to 18 years, 27 female and 13 male) have undergone 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),
arteriovenous malformation (3),
emphysema (1),
graft-versus-host disease (1), rheumatoid lung (1),
cardiomyopathy (1), desquamative
interstitial pneumonitis (1), and
Proteus syndrome (1). The actuarial 1-year survival was 73% (mean follow-up 2 years). One-year actuarial survival for disease groups ranged from 60% for
cystic fibrosis to 90% for
congenital heart 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. Cytomegalovirus: Cytomegalovirus disease developed in six of eight patients with cytomegalovirus mismatching (donor +/recipient-) and in seven of 32 patients who survived more than 30 days (23%). All but cytomegalovirus donor -/recipient- patients were treated with
ganciclovir for 4 weeks after
transplantation. Obliterative
bronchiolitis: Obliterative
bronchiolitis developed in seven of 32 (25%) patients who survived more than 30 days. Obliterative
bronchiolitis was manifest within the first posttransplantation year as a rapid decline in small airway function. Aggressive augmentation of immunosuppression has been used with little success. Posttransplantation lymphoproliferative disease: Posttransplantation lymphoproliferative disease developed in five of 32 (15%) patients who survived more than 30 days developed. One patient died (17% mortality) despite retransplantation. In four patients the disease resolved with reduction in immunosuppression alone, and one required the addition 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 exclusion of patients with panresistant organisms; this has resulted in elimination of infectious mortalities thus far in the pediatric
cystic fibrosis group. Airways: In 21 heart-lung recipients with tracheal anastomoses we have had no airway complications. The double and single lung transplant recipients accounted for 34 bronchial and one tracheal anastomoses. Three (9%) bronchial
stenoses developed. Two were treated with
silicone stents and one with balloon dilation.(ABSTRACT TRUNCATED AT 400 WORDS)