20 - Pediatric Lung Transplantation

Abstract

Indications for pediatric lung transplantation vary by age, with some conditions leading to transplant throughout childhood (e.g., pulmonary hypertension) and others predominating in younger (e.g., surfactant disorders) or older (e.g., cystic fibrosis) children. Transplants for pulmonary hypertension and cystic fibrosis in children have decreased as effective medical therapies have been developed. Since the implementation of the Lung Allocation Score in the United States and other allocation systems, waiting times for children have decreased, but the children receiving lung transplants are likely to be sicker at the time of transplant. The number of absolute contraindications to transplant has diminished as centers develop greater expertise. Strategies such as extracorporeal membrane oxygenation (ECMO) are being used as a “bridge” to transplant in some patients with severe lung disease. After patients undergo lung transplant, they require lifetime immunosuppression, which increases their ongoing risk of infection and posttransplant lymphoproliferative disease (PTLD). Acute vascular rejection and antibody mediated rejection can begin to appear in the period immediately following transplant. In the years following transplant, medication side effects, particularly renal dysfunction, become increasingly common. PTLD, a form of lymphoma often associated with Epstein-Barr virus (EBV), can occur. Most importantly, chronic lung allograft dysfunction (CLAD) becomes increasingly common. Treatment for CLAD is challenging, and this complication limits long-term allograft survival. Mean survival in pediatric lung transplantation is just over 5 years. Extending patient survival and improving organ supply will be vital to the future of the field.