FEV(1) as a guide to lung transplant referral in young patients with cystic fibrosis

Pediatr Pulmonol. 2000 Sep;30(3):198-202. doi: 10.1002/1099-0496(200009)30:3<198::aid-ppul3>3.0.co;2-e.


Forced expiratory volume in one second (FEV(1)) is widely used to guide referral of patients with cystic fibrosis (CF) for lung transplantation. We reasoned that the best FEV(1) in a 6-month period (bFEV(1)) would be a useful marker of the need for transplant referral. We examined both the rate of decline and different threshold values of bFEV(1) as prognostic indicators in young CF patients. In a case-control analysis, rates of decline in and threshold values of bFEV(1) of 28 patients dying between ages 7-18 years from 1980-1997 were compared to those of 28 age- and gender-matched controls. The threshold analysis of bFEV(1) values was then applied to all patients under age 19 years followed in our clinic from 1993-1997. The rate of decline in bFEV(1) differed for cases and controls over the 4-year period prior to death, but not from years 2-4 prior to death, the time at which transplant referral decisions should be made. A bFEV(1) value of 50% predicted at 2 years prior to the death of the case selected 14 of 28 cases and one control. When applied to all pediatric patients followed from 1993-1997, a bFEV1 threshold of 50% predicted selected 2 of the 3 patients who died and 3 of the 140 patients who survived. Consideration of lung transplantation referral should begin when young CF patients have a best FEV(1) <50% predicted despite aggressive conventional treatment.

Publication types

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

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Cystic Fibrosis / mortality
  • Cystic Fibrosis / pathology
  • Cystic Fibrosis / therapy*
  • Female
  • Forced Expiratory Volume
  • Humans
  • Lung Transplantation*
  • Male
  • Patient Selection
  • Prognosis
  • Retrospective Studies
  • Severity of Illness Index