Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis

Anne L Stephenson; Jenna Sykes; Yves Berthiaume; Lianne G Singer; Shawn D Aaron; George A Whitmore; Sanja Stanojevic

doi:10.1016/j.healun.2015.05.003

Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis

J Heart Lung Transplant. 2015 Sep;34(9):1139-45. doi: 10.1016/j.healun.2015.05.003. Epub 2015 May 7.

Authors

Anne L Stephenson¹, Jenna Sykes², Yves Berthiaume³, Lianne G Singer⁴, Shawn D Aaron⁵, George A Whitmore⁶, Sanja Stanojevic⁷

Affiliations

¹ 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.
² Keenan Research Centre in the Li Ka Shing Knowledge Institute of St Michael's Hospital, Toronto, Ontario.
³ Institut de Recherches Cliniques de Montréal and Centre hospitalier de l'Université de Montréal, Montreal, Quebec.
⁴ Department of Medicine, University of Toronto, Toronto, Ontario; Toronto Lung Transplant Program, University Health Network, Toronto, Ontario.
⁵ The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, Ontario.
⁶ The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, Ontario; Desautels Faculty of Management, McGill University, Montreal, Quebec.
⁷ Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.

PMID: 26087666
DOI: 10.1016/j.healun.2015.05.003

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.

Keywords: B. cepacia complex; cystic fibrosis; lung transplantation; survival.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
Age Factors
Burkholderia Infections / complications
Burkholderia cepacia complex
Child
Child, Preschool
Cystic Fibrosis / mortality*
Cystic Fibrosis / surgery*
Exocrine Pancreatic Insufficiency / complications
Female
Humans
Kaplan-Meier Estimate
Lung Transplantation / mortality*
Male
Middle Aged
Proportional Hazards Models
Risk Factors
Young Adult

Grants and funding

Canadian Institutes of Health Research/Canada