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Lung transplantation in children and young adults with cardiovascular disease
N D Bridges1, G B Mallory, C B Huddleston
1Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri.
Insights
Lung transplantation is a viable treatment for children with pulmonary hypertension and congenital heart disease. Survival rates are encouraging, though long-term outcomes require further study.
Area of Science:
- Pediatric Cardiology
- Thoracic Surgery
- Pulmonology
Background:
- Pulmonary hypertension and congenital heart disease significantly impact children's quality of life.
- Lung transplantation is a potential, albeit complex, treatment option for select pediatric cases.
Purpose of the Study:
- To evaluate the outcomes of single or bilateral lung transplantation in pediatric patients with pulmonary hypertension or inadequate pulmonary vascular beds, often associated with congenital heart disease.
- To assess hospital survival, late mortality, and functional status post-transplantation.
Main Methods:
- Retrospective analysis of 20 pediatric patients undergoing single or bilateral lung transplantation.
- Comparison with a contemporary cohort of 41 pediatric patients transplanted for pulmonary disease.
Main Results:
- Hospital survival was 70% (14/20).
- Late deaths were primarily due to obliterative bronchiolitis.
- 10 of 11 survivors achieved New York Heart Association class I functional status at a mean follow-up of 19 months.
- Outcomes were comparable to pediatric lung transplantation for primary pulmonary disease.
Conclusions:
- Single or bilateral lung transplantation is an acceptable therapy for children with pulmonary hypertension, congenital heart disease, or both.
- Further research is needed on pretransplantation survival, operative risk factors, and long-term outcomes for specific patient groups.
Abstract:
Single or bilateral lung transplantation was performed in 20 patients with pulmonary hypertension or an inadequate pulmonary vascular bed; all but 1 had congenital heart disease. The average age was 6.3 years (range, 3 months to 23.9 years). All were in New York Heart Association class IV, and 6 were hospitalized and receiving intensive support before transplantation. Hospital survival was 70% (14/20), with three additional deaths at 7, 11, and 27 months. A prior thoracic operation contributed to three of six hospital deaths from hemorrhage. All late deaths were due directly or indirectly to obliterative bronchiolitis. At a mean follow-up of 19 months (range, 2 to 48 months), 10 of 11 survivors are in New York Heart Association class I. Survival after hospital discharge and incidence of obliterative bronchiolitis are similar in a contemporary group of 41 patients of comparable age who underwent lung transplantation for pulmonary disease (p = not significant). Single or bilateral lung transplantation is an acceptable therapy for children with pulmonary hypertension, congenital heart disease, or both. Further investigation in the areas of pretransplantation survival, operative risk factors, and long-term outcome of single-lung recipients and recipients with hemodynamically insignificant intracardiac lesions are needed to develop optimal decision-making strategies for these patients.