Related Experiment Videos
Critical issues in pediatric lung transplantation
J M Armitage1, G Kurland, M Michaels
1University of Pittsburgh Medical Center, PA 15213.
Insights
Pediatric lung transplantation survival rates improved with focused management of cytomegalovirus, obliterative bronchiolitis, and post-transplant lymphoproliferative disease. Strategies for cystic fibrosis and airway complications were also refined.
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Transplant Immunology
Background:
- Pediatric lung transplantation is a complex procedure with varying indications including congenital heart disease, cystic fibrosis, and pulmonary hypertension.
- Survival rates and management strategies for pediatric lung transplant recipients require continuous evaluation and refinement.
Purpose of the Study:
- To review critical issues impacting patient and program survival in pediatric lung transplantation.
- To analyze outcomes and management strategies for specific complications such as cytomegalovirus disease, obliterative bronchiolitis, and post-transplantation lymphoproliferative disease.
Main Methods:
- Retrospective review of 40 pediatric lung transplant cases (heart-lung, double lung, single lung) performed between 1985 and April 1994.
- Analysis of patient data focusing on indications for transplantation, complications, and survival rates.
- Evaluation of management strategies for cytomegalovirus, obliterative bronchiolitis, post-transplantation lymphoproliferative disease, cystic fibrosis, and airway issues.
Main Results:
- Overall 1-year actuarial survival was 73%, with variations by disease group (60% for cystic fibrosis to 90% for congenital heart disease).
- Cytomegalovirus disease occurred in 23% of long-term survivors; obliterative bronchiolitis in 25%, and post-transplantation lymphoproliferative disease in 15%.
- Modified strategies for cystic fibrosis patients eliminated infectious mortalities, and airway complications were minimal in heart-lung recipients.
Conclusions:
- Critical issues in pediatric lung transplantation include managing cytomegalovirus, obliterative bronchiolitis, and post-transplantation lymphoproliferative disease.
- Refined management strategies, particularly for cystic fibrosis, can improve outcomes and reduce infectious complications.
- Continued focus on these critical areas is essential for enhancing patient and program survival 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)