Changing demographics and outcomes of lung transplantation recipients with cystic fibrosis

Background Cystic fibrosis (CF) is one of the most common diagnoses in adult and pediatric patients undergoing lung transplantation (LTx). A changing pattern of indications for LTx among patients with CF has been noted. This study analyzes the prevalence and characteristics of patients with CF who underwent LTx in the current era. Methods A retrospective analysis was performed using data from the United Network of Organ Sharing database of all LTx performed from 1999 to 2013 ( N = 20,345). Sub-analyses focused on children ( Results CF was the indication for LTx in 14% (2,877) of who patients underwent LTx, a decrease from >17% in the early era to p p = 0.009). The mean age of patients with CF undergoing LTx increased across the eras (early, 28 years ± 10; mid. 28 years ± 10; current, 30 years ± 11; p p p iratory volume in 1 second and waitlist times decreased ( p p > 0.05) at 5.1 years overall. Conclusions The proportion of LTx performed for CF has significantly decreased over time, a trend especially pronounced in pediatric patients. The change in pre-transplant characteristics across eras indicates a trend to perform LTx in more clinically ill and older patients with CF. The overall post-LTx survival has not changed.