Survival Difference Between High-risk and Low-risk CFTR Genotypes After Lung Transplant

Overview

Journal J Heart Lung Transplant

Publisher Elsevier

Specialties Cardiology & Vascular Diseases
General Surgery
Pulmonary Medicine

Date 2022 Jul 21

PMID 35864004

Authors

Emily S Clausen

Jeremy M Weber

Megan L Neely

Kathleen J Ramos

Laurie D Snyder

Affiliations

Soon will be listed here.

Abstract

Background: While cystic fibrosis transmembrane conductance regulator (CFTR) genotypes are associated with clinical outcomes in cystic fibrosis patients, it is unknown if genotype impacts lung transplant outcomes. We sought to compare lung transplant survival and time to bronchiolitis obliterans syndrome (BOS) between high-risk, low-risk, and not yet classified CFTR genotypes.

Methods: We used merged data from the Organ Procurement and Transplantation Network (2005-2017) and United States Cystic Fibrosis Foundation Patient Registry (2005-2016). Cox Proportional Hazards models compared graft failure after lung transplant and time to BOS among high-risk, low-risk, and not yet classified risk CFTR genotype classes.

Results: Among 1,830 cystic fibrosis lung transplant recipients, median survival for those with low-risk, high-risk, and not yet classified genotype was 9.83, 6.25, and 5.75 years, respectively. Adjusted Cox models showed recipients with a low-risk genotype had 39% lower risk of death or re-transplant compared to those with high-risk genotype (adjusted HR 0.61, 95% CI = 0.40, 0.91). A subset of 1,585 lung transplant recipients were included in the BOS subgroup analysis. Adjusted analyses showed no significant difference of developing BOS among high-risk, low-risk, or not yet classified genotypes.

Conclusions: Lung transplant recipients with low-risk CFTR genotype have better survival after transplant compared to recipients with high-risk or not yet classified genotypes. Given these differences, future studies evaluating the mechanism by which CFTR genotype affects post-transplant survival could identify potential targets for intervention.

Citing Articles

Cystic fibrosis survival outcomes following second lung transplant: The north American experience.

Alshehri M, Ramos K, Sykes J, Ma X, Stanojevic S, Quon B Clin Transplant. 2023; 37(11):e15097.

PMID: 37563332 PMC: 10840857. DOI: 10.1111/ctr.15097.

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