Pediatric Lung Transplantation: A 10-Year Nationwide Cohort Study

Purpose Lung transplantation (LT) has become the standard of care in selected children with end-stage parenchymal and vascular pulmonary diseases. In patients with pulmonary hypertension (PH) and cystic fibrosis (CF), new highly effective medical therapies have extended the period before lung transplantation is needed. These innovations will change pediatric LT indications and candidate outcomes. This study aimed to examine waitlist outcomes and posttransplant survival in pediatric lung transplant candidates listed during the 2010-2020 period in France. Methods All candidates registered on the national waiting list before 18 years old between January 2010 and December 2020 were included in this cohort study using the national registry Cristal. Candidate demographics, primary diagnosis, clinical status and laboratory values were analyzed. Cumulative incidence of transplantation and waitlist mortality were estimated with the competing risk analysis with transplantation and death or delisting for worsening medical condition as the competing events. Posttransplant survival curve was estimated using the Kaplan-Meier method. Univariate analyses were performed to identify variables associated with outcomes. Results The 139 listings in 131 candidates resulted in 121 LT. At listing, 122 candidates were ≥11 years of age, 76 were females, 84 had CF, 35 pulmonary fibrosis (PF) and 16 PH, 80 were CMV-negative, 63 had HLA alloantibodies, 73 were hospitalized, 15 were on ECMO support, 4 had mechanical ventilation without ECMO, 61 were with high-urgency status. Cumulative incidence of transplantation and death or delisting for medical condition at 12-month was 80% [72-86] and 7% [4-13], respectively. ECMO support was associated with waitlist mortality. At transplant, 110 candidates were ≥11 years of age, 76 had CF, 28 PF and 14 PH, 54 had HLA alloantibodies, 73 were hospitalized, 21 were on ECMO support, 4 had mechanical ventilation without ECMO. One-year posttransplant survival was 82%. Pretransplant ECMO and mechanical ventilation, and donor-recipient height ratio >1.2 were associated with posttransplant mortality. Conclusion Our findings will be useful for evaluation of the impact of new medical therapies on future indications and results of pediatric LT. Lung transplantation (LT) has become the standard of care in selected children with end-stage parenchymal and vascular pulmonary diseases. In patients with pulmonary hypertension (PH) and cystic fibrosis (CF), new highly effective medical therapies have extended the period before lung transplantation is needed. These innovations will change pediatric LT indications and candidate outcomes. This study aimed to examine waitlist outcomes and posttransplant survival in pediatric lung transplant candidates listed during the 2010-2020 period in France. All candidates registered on the national waiting list before 18 years old between January 2010 and December 2020 were included in this cohort study using the national registry Cristal. Candidate demographics, primary diagnosis, clinical status and laboratory values were analyzed. Cumulative incidence of transplantation and waitlist mortality were estimated with the competing risk analysis with transplantation and death or delisting for worsening medical condition as the competing events. Posttransplant survival curve was estimated using the Kaplan-Meier method. Univariate analyses were performed to identify variables associated with outcomes. The 139 listings in 131 candidates resulted in 121 LT. At listing, 122 candidates were ≥11 years of age, 76 were females, 84 had CF, 35 pulmonary fibrosis (PF) and 16 PH, 80 were CMV-negative, 63 had HLA alloantibodies, 73 were hospitalized, 15 were on ECMO support, 4 had mechanical ventilation without ECMO, 61 were with high-urgency status. Cumulative incidence of transplantation and death or delisting for medical condition at 12-month was 80% [72-86] and 7% [4-13], respectively. ECMO support was associated with waitlist mortality. At transplant, 110 candidates were ≥11 years of age, 76 had CF, 28 PF and 14 PH, 54 had HLA alloantibodies, 73 were hospitalized, 21 were on ECMO support, 4 had mechanical ventilation without ECMO. One-year posttransplant survival was 82%. Pretransplant ECMO and mechanical ventilation, and donor-recipient height ratio >1.2 were associated with posttransplant mortality. Our findings will be useful for evaluation of the impact of new medical therapies on future indications and results of pediatric LT.