Context Patients with cystic fibrosis (CF) are the second largest group of
lung transplant recipients in the United States. The survival effect of tra
nsplantation on a general CF population has not previously been measured.
Objective To determine the impact of bilateral lung transplantation on surv
ival in patients with CF.
Design, Setting, and Patients Retrospective observational cohort study of 1
1630 CF patients who did not undergo lung transplantation (controls) and 46
8 transplant recipients with CF from 115 CF centers in the United States, 1
992-1998. Patients were stratified into 5 groups based on a 5-year survival
prediction model (survival group 1: <30%; survival group 2:30 to <50%; sur
vival groups 3-5: 50 to <100%.)
Main Outcome Measure Five-year survival from date of transplantation in 199
2-1997 in the transplant group and from January 1, 1993, in the control gro
up.
Results Lung transplantation increased 5-year survival of CF patients in su
rvival group 1. Survival group 2 had equivocal survival effects, and groups
3-5 had negative survival effects from transplantation. From 1994-1997, th
ere was a mean annual prevalence of 238 patients in survival group 1 and me
an annual incidence of 154 patients entering the group, approximately 1.5 t
imes the number of lung transplantations performed each year in CF patients
(mean, 104). Use of the criterion of forced expiratory volume in 1 second
of less than 30% resulted in an equivocal survival benefit and identified 1
458 potential candidates for transplantation in 1993.
Conclusions Cystic fibrosis patients in group 1 have improved 5-year surviv
al after lung transplantation. The majority of patients with CF have equivo
cal or negative Survival effects from the procedure. Selection of patients
with CF for transplantation based on group 1 survival predictions maximizes
survival benefits to individuals and may reduce the demand for scarce dono
r organs.