Changing demographics and outcomes of lung transplantation recipients with cystic fibrosis

Naritaka Kimura, Muhammad S. Khan, Marc Schecter, Raheel Rizwan, Roosevelt Bryant, Erin Wells, Christopher Towe, Farhan Zafar, David L.S. Morales

Research output: Contribution to journalArticlepeer-review

15 Citations (Scopus)


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 (<18 years old). Patients with CF who underwent LTx were assigned to early (1999–2003), mid (2004–2008), and current (2009–2013) eras based on the date of the procedure as well as before and after implementation of the new lung allocation score system in 2005. 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 <13% in the current era (p < 0.001). In the pediatric cohort, CF was the indication for LTx in 383 (53%) patients, a proportion that also decreased across eras (early, 60%; mid, 53%; current, 47%; 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 < 0.001). Pre-transplant ventilator use and incidence of pan-resistant infections also increased (p < 0.001), whereas pre-transplant forced expiratory volume in 1 second and waitlist times decreased (p < 0.001) in patients with CF. Graft survival across the eras remained similar (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.

Original languageEnglish
Pages (from-to)1237-1244
Number of pages8
JournalJournal of Heart and Lung Transplantation
Issue number10
Publication statusPublished - 2016 Oct 1
Externally publishedYes


  • cystic fibrosis
  • demographics
  • lung allocation score system
  • lung transplantation
  • mechanical ventilation
  • transplant candidacy

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation


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