WS8.2 Pulmonary arterial hypertension in adult CF lung transplantation candidates

Objectives: Lung transplantation is the most effective means of improving survival and quality of life in patients with end-stage CF. Our aim was to determine outcomes of referrals, waiting times and factors associated with a poor outcome. Methods: Data were retrieved retrospectively from our electronic patient record system for all adult patient referred to UK Transplant centres over a 6 year period. Results: 48 patients [27 female, median (range) age at referral 29.7 yrs (16.5–49.5), FEV1 25% predicted (13−41) and BMI 20.2 kg/m 2 (16.6–29.7) were referred for lung transplantation. Median time from initial discussion to referral was 80 days (13–1090). 3 patients died prior to first appointment and 5 are waiting to be seen. 40 patients were assessed and 26 accepted (4 died after first appointment, 2 not accepted and 8 currently under follow up). Of the 26 accepted, 16 have received a transplant [waiting time on active list 177 days (28–865)], 3 died on the active list, 3 were removed (significant clinical deterioration) and 4 are currently waiting. Total referral process from initial assessment to transplant was 1.6 yrs (0.7−3.2). Fifteen patients died during the referral process; there were no significant differences in clinical severity when compared to patients who were transplanted. However patients who died during the process had a significantly longer time from initial discussion to local assessment [112 (13–1090) vs. 56 (29–440) days, p = 0.0481]. Conclusion: Early discussion is important and current waiting times need to be taken into consideration. Increased psychological input is essential as an initial delay in the process may be associated with poor outcome.