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Pediatric lung transplantation for pulmonary hypertension and congenital heart disease
T L Spray1, G B Mallory, C E Canter
1St. Louis Children's Hospital, Washington University School of Medicine, Missouri 63110.
Insights
Lung transplantation offers a viable solution for children with congenital heart disease and pulmonary hypertension. Survivors demonstrate improved mobility and normalized pulmonary artery pressures post-transplant.
Area of Science:
- Cardiology
- Pulmonology
- Pediatric Surgery
Background:
- Congenital heart disease (CHD) can lead to end-stage pulmonary hypertension (PH) and respiratory insufficiency.
- Pediatric PH presents complex management challenges, often requiring advanced interventions.
Purpose of the Study:
- To evaluate the outcomes of lung transplantation in pediatric patients with PH and CHD.
- To assess the safety and efficacy of various lung transplant strategies in this cohort.
Main Methods:
- Retrospective analysis of five pediatric patients undergoing lung transplantation for PH associated with CHD.
- Procedures included bilateral sequential, single-lung transplants, and extracorporeal membrane oxygenation (ECMO) support.
- Surgical interventions for associated cardiac defects were performed concurrently.
Main Results:
- All five patients survived the initial operation.
- One late death occurred due to lymphoproliferative disease.
- Four survivors are ambulatory, off oxygen, with normalized pulmonary artery pressures at 9-12 months post-transplant.
Conclusions:
- Lung transplantation is a feasible and effective treatment for pediatric end-stage PH secondary to CHD.
- Successful outcomes include improved functional status and hemodynamic stability.
- This approach offers a life-saving option for critically ill children with complex cardiopulmonary conditions.
Abstract:
Five children underwent lung transplantation for end-stage pulmonary hypertension and respiratory insufficiency associated with congenital heart disease. One (17 mo) had pulmonary hypertension with a patent ductus arteriosus and required two periods of preoperative extracorporeal membrane oxygenation before successful bilateral sequential lung transplantation. One (21 mo) required bilateral lung transplantation for pulmonary hypertension and bronchopulmonary dysplasia associated with iatrogenic injury to the left pulmonary artery. This child also had patent ductus arteriosus ligation and preoperative catheter closure of an atrial septal defect. Extracorporeal membrane oxygenation was required for early postoperative pulmonary support. One child underwent right single-lung transplantation and closure of an atrial septal defect for pulmonary hypertension. Two patients had single-lung transplantation for Eisenmenger's syndrome: 1 with muscular inlet ventricular septal defect closure, atrial septal defect closure, and right single-lung transplantation; 1 with ventricular septal defect closure, patent ductus arteriosus ligation, right ventricular outflow tract patch repair, and single-lung transplantation. All patients survived operation, with one late death (lymphoproliferative disease). The 4 survivors are all ambulatory without oxygen and have evidence of normal pulmonary artery pressure 9 to 12 months after transplantation.