Rationale: The optimal timing for listing of cystic fibrosis patients for lung transplantation is controversial.

Objectives: We conducted a retrospective cohort study of 343 patients listed for lung transplantation at four academic medical centers to identify risk factors for death while awaiting transplantation.

Methods: Data on possible risk factors were abstracted from medical records.

Measurements: Time to death, patient demographic characteristics, and risk factors for death while awaiting transplantation were assessed. Univariate and multivariate survival analyses were performed using Cox regression.

Results: By univariate analyses, FEV₁ ⩽ 30% predicted (HR, 3.8; 95% CI, 2.0–7.5), Pa_CO2 ⩾ 50 mm Hg (HR, 1.85; 95% CI, 1.1–3.0), and shorter height (HR, 1.8; 95% CI, 1.1–3.0) were associated with a higher risk of death. Referral from an accredited cystic fibrosis center was associated with a lower risk (HR, 0.53; 95% CI, 0.30–0.92). The final multivariate model included referral from an accredited cystic fibrosis center (HR, 0.5; 95% CI, 0.3–1.0) and listing year after 1996 (HR, 0.4; 95% CI, 0.2–0.7); both were associated with a lower risk of death. FEV₁ ⩽ 30% predicted (HR, 6.8; 95% CI, 2.4–19.3), Pa_CO2 ⩾ 50 mm Hg (HR, 6.9; 95% CI, 1.5–32.1), and use of a nutritional intervention (HR, 2.3; 95% CI, 1.3–4.1) were associated with increased risk. Patients with FEV₁ > 30% predicted had a higher risk of death only when their Pa_CO2 was ⩾ 50 mm Hg (HR, 7.0; 95% CI, 1.5–32), while the increased risk of death with FEV₁ ⩽ 30% was not further influenced by the presence of hypercapnia.

Conclusions: We identified risk factors for waiting list mortality that could impact on transplant listing and allocation guidelines.