The Changing Transplant Landscape in the Era of Elexacaftor/Tezacaftor/Ivacaftor: A Word of Caution

Background

Elexacaftor/tezacaftor/ivacaftor (ETI) has dramatically changed the landscape of cystic fibrosis (CF) care, including in those who require lung transplantation. The objectives of the study were to describe the cohort demographics and outcomes of primary lung transplant recipients before and after the availability of ETI.

Methods

This is a descriptive study of lung transplants performed at the Toronto Lung Transplant Program for CF during two time periods: 2019 (pre-ETI era) and 2021–2023 (post-ETI era). All subjects were referred from the Adult CF program at St. Michael's Hospital, Toronto. Data were obtained from chart review and the Toronto Lung Transplant database. The Kaplan–Meier method was used to estimate survival probability at 1 year post-transplant.

Results

There were 22 lung transplants performed in 2019 (19 [86.4%] primary and 3 [13.6%] re-transplants) compared to 11 lung transplants (8 [72.7%] primary and 3 [27.3%] re-transplants) in the post-ETI era. In primary transplant recipients, median age was 29.4 years (Range 18.6–67.6 years) in 2019 compared to 30.0 years (Range 19.1–64.0 years) in 2021–2023. In the post-ETI era, none of the individuals had a deltaF508 variant, compared to 84% in 2019. One-year survival probability was lower in the post-ETI era (62.5% vs. 84.2%, respectively).

Conclusion

Lung transplant recipients in the post-ETI era were more complex with high-risk characteristics and had worse post-transplant outcomes. This study highlights the importance of further investigation to better understand the impact of ETI on transplant referral patterns, recipient characteristics, and post-transplant outcomes in the CF population.