title: Long term results of lung transplantation for cystic fibrosis.
abstract: OBJECTIVE
  We reviewed our experience with lung transplant for cystic fibrosis (CF) over a 10-year period to identify factors influencing long-term survival.
  
  
  METHODS
  One hundred and twenty-three patients with CF have undergone 131 lung transplant procedures at our institution; 114 have had bilateral sequential lung transplants (DLTX) and nine have had bilateral lower lobe transplants from living donors. Three patients had retransplant for acute graft failure, and five had late retransplant for bronchiolitis obliterans syndrome (BOS). Kaplan-Meier survival was calculated for the entire cohort and for subsets at higher risk of death to determine factors predicting a better outcome.
  
  
  RESULTS
  Actuarial survival for the entire group of DLTX CF patients was 81% at 1 year, 59% at 5 years, and 38% at 10 years. Lobar transplant was associated with a poorer survival (37.5% at 1 and 5 years). Among DLTX patients, colonization with Burkholderia cepacia was present in 22 patients and was associated with poorer outcome (1- and 5-year survival 60 and 36% in B. cepacia patients vs. 86 and 64% in non-cepacia patients). DLTX patients younger than age 20 (n=22) had a similar survival to patients age 20 or older (n=90). Being on a ventilator at the time of transplant was not associated with poorer survival (n=8). BOS affects increasing numbers of survivors with time. Five CF patients have been retransplanted due to BOS with one operative death and 1-year survival of 60%.
  
  
  CONCLUSIONS
  DLTX has acceptable long term survival in CF adults and children with end stage disease. CF patients colonized with B. cepacia have a worse outcome but transplantation is still warranted.
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, University of North Carolina at Chapel Hill, 108 Burnett-Womack Building, UNC, Chapel Hill, NC 27599-7065, USA. ltxtme@med.unc.edu
authors.forename: T M
authors.initials: TM
authors.lastname: Egan
authors.forename: F C
authors.initials: FC
authors.lastname: Detterbeck
authors.forename: M R
authors.initials: MR
authors.lastname: Mill
authors.forename: M S
authors.initials: MS
authors.lastname: Bleiweis
authors.forename: R
authors.initials: R
authors.lastname: Aris
authors.forename: L
authors.initials: L
authors.lastname: Paradowski
authors.forename: G
authors.initials: G
authors.lastname: Retsch-Bogart
authors.forename: B S
authors.initials: BS
authors.lastname: Mueller
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001989:Bronchiolitis Obliterans / Q000601:surgery; D003550:Cystic Fibrosis / Q000401:mortality* / Q000601:surgery*; D005500:Follow-Up Studies; D006084:Graft Rejection; D006801:Humans; D016040:Lung Transplantation / Q000401:mortality*; D011183:Postoperative Complications / Q000601:surgery; D012086:Reoperation; D015996:Survival Rate; D016896:Treatment Outcome