Pediatric Patients with Pulmonary Vascular and Restrictive Lung Disease Suffer Disproportionately High Waitlist Mortality

Abstract

Purpose Survival after pediatric lung transplantation is the same for major diagnostic groups; however, waitlist outcomes by diagnosis have not been analyzed since the major changes to lung allocation in 2005. Methods The UNOS database was reviewed for pediatric patients ( Results 1,054 patients were listed for pediatric lung transplantation within the diagnostic categories of Cystic Fibrosis (Group A; n=480), Pulmonary Vascular Disease (Group B; n=246), Obstructive Lung Disease (Group C; n=43), and Restrictive Lung Disease (Group D; n=285). Groups A and C had similar 1-year waitlist survival (Log-rank p-value=0.987) while Groups B and D did the worst (Figure 1). There was increased hazard of mortality at one year on the waitlist for Groups B (1.594; p-value=0.023) and D (1.747; p-value=0.006) compared to Group A when adjusting for extracorporeal oxygenation, ventilator support, pan-resistant bacterial infection, and glomerular filtration rate Conclusion Waitlist outcomes in pediatric lung transplant vary greatly by diagnostic category. Since they have similar post-transplant outcomes, all candidates with restrictive lung disease and vascular disease would benefit from prioritization for donor lungs in hopes of earlier transplantation.