title: Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis.
abstract: BACKGROUND
  Contemporary 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.
  
  
  METHODS
  The 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.
  
  
  RESULTS
  Between 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.
  
  
  CONCLUSIONS
  After 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.
authors.affiliation: 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.
authors.forename: Anne L
authors.initials: AL
authors.lastname: Stephenson
authors.affiliation: Keenan Research Centre in the Li Ka Shing Knowledge Institute of St Michael's Hospital, Toronto, Ontario.
authors.forename: Jenna
authors.initials: J
authors.lastname: Sykes
authors.affiliation: Institut de Recherches Cliniques de Montréal and Centre hospitalier de l'Université de Montréal, Montreal, Quebec.
authors.forename: Yves
authors.initials: Y
authors.lastname: Berthiaume
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario; Toronto Lung Transplant Program, University Health Network, Toronto, Ontario.
authors.forename: Lianne G
authors.initials: LG
authors.lastname: Singer
authors.affiliation: The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, Ontario.
authors.forename: Shawn D
authors.initials: SD
authors.lastname: Aaron
authors.affiliation: The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, Ontario; Desautels Faculty of Management, McGill University, Montreal, Quebec.
authors.forename: George A
authors.initials: GA
authors.lastname: Whitmore
authors.affiliation: Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Sanja
authors.initials: S
authors.lastname: Stanojevic
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D019121:Burkholderia Infections / Q000150:complications; D042602:Burkholderia cepacia complex; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000401:mortality* / Q000601:surgery*; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012307:Risk Factors; D055815:Young Adult
keywords: B. cepacia complex; cystic fibrosis; lung transplantation; survival