Critical issues in pediatric lung transplantation

Abstract

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)

Lung and Heart–Lung Transplantation in Children and Adolescents: A Long-term Single-center Experience

“…Based on this experience, we tend to use a reduced course of immunosuppression, as described elsewhere. 9,11 In contrast, we observed an increased prevalence of acute and chronic rejections in the pediatric patient group. Among our study group, 5 patients died because of severe bronchiolitis obliterans syndrome (BOS), another 5 required pulmonary retransplantation because of severe BOS.…”

“…The incidence of viral infections (i.e., cytomegalovirus, Epstein-Barr virus [EBV] and adenoviruses) is increased in pediatric recipients. 9,11 We observed several severe and long-lasting viral infections in our patients. Eight patients died due to severe infection during the first year post-operatively.…”

“…Bronchial anastomoses are associated with a higher risk of airway complications (i.e., bronchial stenoses) compared with tracheal anastomoses, especially in small children. 9,10 There are other centers that perform DLTx in this age group, but long-term results are still pending. 1…”

Successful lung transplantation for adolescents at a hospital for adults

“…Early series of LTx in younger age groups report an increased incidence of CMV‐mismatched and EBV‐mismatched grafts, largely because of the low rate of prior CMV and EBV exposure in recipients before transplantation 28 , 29 . The advent of antiviral agents specifically targeted against these viruses has reduced the impact of these infections as serious problems after LTx, particularly with prophylactic strategies using ganciclovir or valganciclovir against CMV.…”

Overview of Lung Transplantation

“…The incidence of airway complications is more frequent in pediatric lung transplant recipients, with an estimated range from 9% up to 27% in published series [38][39][40]. The bronchial stenosis can occur as an early and as a late complication of lung transplantation.…”