Long-term survival of patients with cystic fibrosis undergoing lung transplantation: a single center experience

SESSION TITLE: Transplantation Posters 3 SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2023 12:00 pm - 12:45 pm PURPOSE: Lung transplantation (LT) has been a gold standard treatment for end-stage lung disease due to Cystic Fibrosis (CF). In recent years, LT for CF is declining in many high-income countries due to the availability of CF transmembrane conductance regulator (CFTR) protein modulators. The aim of this study is to analyze the long-term outcomes of CF patients who underwent LT at a large academic medical center. METHODS: Data for this retrospective study was collected from our institutional database and United Network for Organ Sharing (UNOS) DonorNet following approval from the Institutional Review Board. The variables include donor and recipient sex, race, and age, recipient CF genotype, body mass index (BMI), albumin, pre-albumin, ischemic times, and the diagnoses of bronchiolitis obliterans syndrome (BOS) or restrictive allograft syndrome (RAS) from chronic lung allograft dysfunction (CLAD). The cohort was divided into two groups based on the development of the Lung Allocation Score (LAS): pre-LAS for 1994 - May 2005 and post-LAS for May 2005 - 2020. The Kaplan-Meier method was employed for time-to-event (CLAD, death). Cox regression was conducted to assess the impact of risk factors on hazard. Group comparisons were performed using T-test, Chi-square test, and Wilcoxon-rank sum test. RESULTS: 872 LTs were performed from 1990 to 2020, with 126 being bilateral LTs for CF patients. The majority were female patients (66; 55%) and of white race (119; 94%). The mean age at LT was 29.1 ± 10.1 years. Group 1 (pre-LAS) consisted of 46 while group 2 (post-LAS) had 80 patients. In the 75 patients with CF genotype data, the predominant mutation was homozygous delta F508 (19; 25%). 44 patients in group 2 had CLAD (24 BOS, 19 BOS/RAS, 1 RAS) at a median duration of 5.9 years (95%CI 3.5 - 8.0 yrs.), while CLAD data for group 1 was incomplete. The median survival in group 1 was 8.0 years (95%CI 4.6 -10.2 yrs.) and 6.3 years (95%CI 3.8 -14.4 yrs.) in group 2. Notably, the median survival time was statistically affected by the age of the recipient at LT (p=0.007) and displayed no statistical significance with the other variables analyzed. The actuarial survival of both groups 1 and 2 was plotted on a Kaplan-Meier graph. CONCLUSIONS: Lung transplantation extends life for CF patients with end-stage lung disease, but the median survival is still low. LT is still an option for CF patients with end-stage lung failure living in low- and mid-income countries where CFTR modulators are not freely available. Meanwhile, CLAD remains a significant cause of mortality. A larger cohort study is required to confirm the relationship between age at LT and median survival. CLINICAL IMPLICATIONS: Lung transplantation extends life for CF patients with end-stage lung disease, but the median survival is still low. LT is still an option for CF patients with end-stage lung failure living in low- and mid-income countries where CFTR modulators are not freely available. Meanwhile, CLAD remains a significant cause of mortality. A larger cohort study is required to confirm the relationship between age at LT and median survival. DISCLOSURES: No relevant relationships by Christopher Heid No disclosure on file for Maysoon Hussain Advisory relationship with Boehringer Ingelheim Please note: 2019 to current Added 04/13/2023 by Raksha Jain, source=Web Response, value=Honoraria Grant review relationship with Vertex Please note: 2021 to current Added 04/13/2023 by Raksha Jain, source=Web Response, value=Honoraria Advisory Committee Member relationship with Recode Please note: 2022 to current Added 04/13/2023 by Raksha Jain, source=Web Response, value=Consulting fee DSMB relationship with Armata Please note: 2022 to current Added 04/13/2023 by Raksha Jain, source=Web Response, value=Honoraria No relevant relationships by Vaidehi Kaza No relevant relationships by Stephany Kim No relevant relationships by John Murala No relevant relationships by Matthias Peltz No disclosure on file for Ananya Pillai No disclosure on file for Steve Ring No relevant relationships by Jewon Sohn No relevant relationships by Michael Wait No relevant relationships by Song Zhang