• Anne L Stephenson, Jenna Sykes, Yves Berthiaume, Lianne G Singer, Shawn D Aaron, George A Whitmore, and Sanja Stanojevic.
• Adult CF Program, St. Michael's Hospital, Toronto, Ontario; Keenan Research Centre in the Li Ka Shing Knowledge Institute of St Michael's Hospital, Toronto, Ontario; Department of Medicine, University of Toronto, Toronto, Ontario; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. Electronic address: stephensona@smh.ca.
• J. Heart Lung Transplant. 2015 Sep 1; 34 (9): 1139-45.

BackgroundContemporary studies evaluating post-transplant survival are limited and often include data from single centers or selected sub-groups. The purpose of this study was to evaluate overall transplant survival and to identify risk factors associated with death after transplant.MethodsThe Canadian Cystic Fibrosis Registry, a population-based cohort, was used to describe survival after lung transplant. Pre-transplant factors associated with post-transplant survival were estimated using Cox proportional hazards models.ResultsBetween 1988 and 2012, 580 patients received a lung transplant. In the entire cohort, post-lung transplant 1-year survival was 87.8%, 5-year survival was 66.7%, and 10-year survival was 50.2%. Median post-transplant survival was 3.3 years (95% confidence interval [CI] = 2.13-6.56) in patients infected with Burkholderia cepacia complex compared with 12.36 years (95% CI = 10.34-17.96) in patients without B cepacia infection (hazard ratio [HR] = 2.63, 95% CI = 2.0-3.44). After adjustment, there was a non-significant trend toward better post-transplant survival with increasing year of transplant (HR = 0.98, 95% CI = 0.96-1.00). Pancreatic sufficiency (HR = 2.13, 95% CI = 1.41-3.20) and age at transplant such that youngest and oldest had the poorest survival (p < 0.001) were significant negative predictors of survival. The risk of death after transplant for patients infected with B cepacia was highest within the first year (HR = 6.29, 95% CI = 3.87-10.21) but remained elevated >1 year after transplant (HR = 1.92, 95% CI = 1.33-2.77) compared with patients without B cepacia infection.ConclusionsAfter lung transplantation, 5-year survival in Canadians with CF is 67%, and 50% of patients live >10 years. Despite these impressive probabilities, age at transplant, pancreatic sufficiency and B cepacia infection remain important determinants of survival after lung transplantation.Copyright © 2015 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

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