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Lung transplantation in children
Charles B Huddleston1, Jeffrey B Bloch, Stuart C Sweet
1Department of Surgery, Washington University School of Medicine, St. Louis Childrens' Hospital, Missouri 63110, USA. huddlestonc@msnotes.wustl.edu
Insights
Pediatric lung transplantation (LTX) offers a viable, though high-risk, treatment for children with end-stage lung disease. Bronchiolitis obliterans remains the primary challenge for long-term survival in these young patients.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Transplantation Medicine
Background:
- Pediatric lung transplantation (LTX) presents unique challenges compared to adult procedures, including recipient size, indications, and post-transplant care.
- This study analyzes the largest series of pediatric lung transplants globally, offering insights into patient selection and outcomes.
Purpose of the Study:
- To detail the authors' experience with pediatric lung transplantation (LTX).
- To provide an overview of patient selection criteria and outcomes for pediatric LTX.
- To identify key factors influencing survival and complications in pediatric lung transplant recipients.
Main Methods:
- Analysis of 207 isolated lung transplants performed on 190 children under 18 years old from 1990 to the present.
- Categorization of patients by age at transplant (under 1, 1-5, 5-10, 10-18 years) and primary diagnosis (cystic fibrosis, pulmonary vascular disease, bronchiolitis obliterans, etc.).
- Kaplan-Meier survival analysis and assessment of causes of early and late mortality, as well as incidence of post-transplant complications.
Main Results:
- Five-year survival rates for pediatric lung transplantation were 77% at 1 year, 62% at 3 years, and 55% at 5 years.
- The most common causes of early death were graft failure, while late deaths were primarily due to bronchiolitis obliterans (57%), infection (21%), and posttransplant malignancies (18%).
- Bronchiolitis obliterans occurred in 46% of long-term survivors, with risk factors including age over 3 years, multiple acute rejection episodes, and prolonged organ ischemic time.
Conclusions:
- Pediatric lung transplantation (LTX) is a high-risk but feasible treatment option for children with end-stage pulmonary and vascular diseases.
- Bronchiolitis obliterans is the principal obstacle to achieving better long-term survival rates in pediatric lung transplant recipients.
- Further research and management strategies are needed to mitigate the impact of bronchiolitis obliterans and improve outcomes in pediatric LTX.
Objective:
To report the authors' experience with pediatric lung transplantation (LTX) to provide an overview of patients selected for this procedure and their outcomes.
Summary Background Data:
Pediatric LTX differs from adults in many ways, including recipient size, indications, posttransplant care, and rehabilitation.
Methods:
Two hundred seven isolated lung transplants on 190 children under the age of 18 years were performed from 1990 to the present. This represents the single largest series of lung transplants in children in the world. Thirty-two patients were less than 1 year of age, 22 were 1 to 5 years of age, 32 were 5 to 10 years of age, and 121 were 10 to 18 years old. The groups by major diagnostic category were cystic fibrosis (n = 89), pulmonary vascular disease (n = 44), bronchiolitis obliterans (n = 21), pulmonary alveolar proteinosis (n = 12), pulmonary fibrosis (n = 15), and other (n = 26). The average age at the time of transplant was 9.5 +/- 5.9 years (range 36 days to 18 years).
Results:
Survival by Kaplan-Meier analysis was 77% at 1 year, 62% at 3 years, and 55% at 5 years. There was no significant difference in survival according to primary diagnosis leading to LTX or age at LTX. There were 25 early (<60 days) and 61 late deaths. The most common cause of early deaths was graft failure (13/25, 52%). The most common causes of late death were bronchiolitis obliterans (35/61, 57%), infection (13/61, 21%), and posttransplant malignancies (11/61, 18%). No patient died of acute rejection. In those surviving greater than 3 months (mean follow-up 3.5 years, range 3 months to 11 years), the overall rate of occurrence of bronchiolitis obliterans was 46% (80/175) and the overall incidence of posttransplant malignancies was 24/175 (14%). Major risk factors for the development of bronchiolitis obliterans were age older than 3 years, more than two episodes of acute rejection, and organ ischemic time longer than 180 minutes.
Conclusions:
In children, LTX is a high-risk but viable treatment for end-stage pulmonary parenchymal and vascular disease. The major hurdle to overcome in long-term survival is bronchiolitis obliterans.