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Pediatric lung transplantation: expanding indications, 1985 to 1993
J M Armitage1, F J Fricker, G Kurland
1Department of Surgery, University of Pittsburgh School of Medicine, PA.
Insights
Pediatric lung transplantation shows promising survival rates, with 78% overall survival at 1.8 years. Outcomes were better for non-cystic fibrosis patients (87%) compared to those with cystic fibrosis (55%).
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Transplant Medicine
Background:
- Lung transplantation in children is an extension of adult programs.
- Thirty-two pediatric patients underwent heart-lung, double-lung, or single-lung transplants.
- Diverse etiologies of end-stage lung disease were observed, including cystic fibrosis and primary pulmonary hypertension.
Purpose of the Study:
- To evaluate the outcomes and survival rates of pediatric lung transplantation.
- To compare the efficacy of different immunosuppressive regimens.
- To analyze factors influencing survival in pediatric lung transplant recipients.
Main Methods:
- Retrospective analysis of 32 pediatric lung transplant recipients (ages 1-18).
- Procedures included heart-lung, double-lung, and single-lung transplants.
- Immunosuppression utilized cyclosporine or FK 506-based therapy with azathioprine and steroids.
Main Results:
- Overall survival rate was 78% at a mean follow-up of 1.8 years.
- Survival was higher in recipients without cystic fibrosis (87%) compared to those with cystic fibrosis (55%).
- FK 506-based immunosuppression showed significantly fewer rejection episodes after 90 days compared to cyclosporine (p < 0.001).
Conclusions:
- Pediatric lung transplantation is a viable option with encouraging survival rates.
- Cystic fibrosis remains a significant challenge impacting long-term survival.
- FK 506 demonstrates superior long-term efficacy in preventing rejection compared to cyclosporine in pediatric lung transplant recipients.
Abstract:
The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplant program, which began in 1982. Thirty-two pediatric patients (age range 1 to 18 years) have undergone heart-lung (n = 16), double-lung (n = 14), and single-lung (n = 2) transplantation procedures. The cause of end-stage lung disease was primary pulmonary hypertension (n = 7), congenital heart disease (n = 7), cystic fibrosis (n = 9), pulmonary arteriovenous malformation (n = 2), desquamative interstitial pneumonitis (n = 2), graft-versus-host disease (n = 1), emphysema (n = 1), rheumatoid lung (n = 1), cardiomyopathy (n = 1), and Proteus syndrome (n = 1). Six patients (19%) underwent pretransplantation thoracic surgical procedures. The survival rate was 78% at a mean follow-up of 1.8 years. The survival rate in the 23 recipients without cystic fibrosis was 87% (95% since 1985). The actuarial 1-year survival rate in the nine recipients with cystic fibrosis was 55%. Immunosuppression was cyclosporine (n = 9) or FK 506 (n = 23)-based therapy with azathioprine and steroids. Children were followed up by spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection per patient in the groups treated with cyclosporine and FK 506, respectively, was 1.0 and 1.2 at 30 days, 0.67 and 0.38 at 30 to 90 days, and 2.33 and 0.46 at greater than 90 days (p < 0.001, Fisher exact test).(ABSTRACT TRUNCATED AT 250 WORDS)