Survival benefit of lung transplant for cystic fibrosis since lung allocation score implementation

Am J Respir Crit Care Med. 2013 Jun 15;187(12):1335-40. doi: 10.1164/rccm.201303-0429OC.


Rationale: The survival benefit of lung transplantation (LT) in adult patients with cystic fibrosis (CF) is debated.

Objectives: We sought to assess the survival benefit of LT in adult patients with CF.

Methods: We used data from the United Network for Organ Sharing Registry to identify adult patients with CF on a wait list for LT in the United States between 2005 and 2009. Survival times while on the wait list and after LT were modeled by use of a Cox model that incorporated transplantation status as a time-dependent covariate. Evolution in lung allocation score (LAS) while on the wait list was used as a surrogate for disease severity. We fitted a model for the joint distribution of survival and longitudinal disease process (LAS over time).

Measurements and main results: A total of 704 adult patients with CF were registered on a wait list during the study period. The cumulative incidence of LT was 39.3% (95% confidence interval, 35.6-42.9%) at 3 months and 64.7% (61.0-68.4%) at 12 months, whereas the incidence of death while on the wait list at the same times was 8.5% (6.4-10.6%) and 12.9% (10.3-15.5%), respectively. Survival after LT was 96.5% (94.7-98.2%) at 3 months; 88.4% (85.1-91.8%) at 12 months; and 67.8% (59.9-76.8%) at 3 years. LT conferred a 69% reduction in the instantaneous risk of death (51-80%). The interaction between LAS and LT was significant: the higher the LAS, the greater the survival benefit of LT (P < 0.001).

Conclusions: LT confers a survival benefit for adult patients with CF.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Cystic Fibrosis / mortality*
  • Cystic Fibrosis / surgery*
  • Female
  • Humans
  • Lung Transplantation*
  • Male
  • Severity of Illness Index
  • Survival Rate
  • Tissue and Organ Procurement / statistics & numerical data
  • Waiting Lists
  • Young Adult