Survival difference between high-risk and low-risk CFTR genotypes after lung transplant
Open accessAbstract
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.
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Our findings were consistent with the results of Clausen et al. following re-transplant.
“Our findings were consistent with these results following re-transplant.”
From Cystic fibrosis survival outcomes following second lung transplant: The north American experience (2023) · cites this paper as [8]
The Discussion reports better graft survival for recipients with low-risk than high-risk CFTR genotypes. However, the Methods (Study cohort) specify that the study included patients undergoing their first lung transplant; it does not report survival following re-transplant. The Results (Graft Failure Analysis) count re-transplant as part of a graft-failure outcome, not as the starting point for a survival analysis. (LLM, conf=0.86)
Experimental — accuracy not yet measuredPre-transplant factors known to be associated with mortality following first lung transplant include B. cepacia complex, year of transplant, and CFTR functional class, while male sex and age at transplant were of borderline significance.
“Pre-transplant factors known to be associated with mortality following first lung transplant include B. cepacia complex, year of transplant, and CFTR functional class while male sex and age at transplant were of borderline significance.7,8”
From Cystic fibrosis survival outcomes following second lung transplant: The north American experience (2023) · cites this paper as [8]
The Results (Graft Failure Analysis; page not provided) associate CFTR genotype classification and a history of Burkholderia cepacia complex with graft failure, defined as death or retransplant. They do not identify transplant year or male sex as associated factors. Nor do they describe age at transplant as borderline significant: age was significantly associated with graft failure at 35 years or younger, but not above 35. (LLM, conf=0.87)
Experimental — accuracy not yet measuredClausen et al. found that individuals with high-risk genotypes (functional class I-III) had worse survival following first lung transplant compared to those with at least one Class IV or V mutation.
“When looking at survival by genotype, Clausen et al. recently published a study showing that individuals with high-risk genotypes (categorized by functional class I-III) had worse survival following first lung transplant compared to those with at least one Class IV or V mutation.8”
From Cystic fibrosis survival outcomes following second lung transplant: The north American experience (2023) · cites this paper as [8]
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Cystic fibrosis survival outcomes following second lung transplant: The north American experience
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“7,8 Although risk factors for worse survival following re-transplant have been identified, the results are often reported overall and not by underlying disease state which limits our understanding of how CF-specific factors influence mortality in this population.”
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(2023) · The Journal of Heart and Lung Transplantation · doi:10.1016/s1053-2498(23)01801-6
- Authors
- Clausen, E. S., Weber, J. M., Neely, M. L., Ramos, K. J., & Snyder, L. D.
- Affiliations
- University of Pennsylvania (US)
- Duke University (US)
- University of Washington (US)
- Corresponding author
- Clausen, Emily S.
- Venue
- The Journal of Heart and Lung Transplantation
- Publisher
- Elsevier BV
- Published
- 2022-10
- Volume
- 41
- Issue
- 10
- Pages
- 1511-1519
- ISSN
- 1053-2498 (ISSN-L)
- PMC
- PMC10124119
- OpenAlex
- W4225264651
- Cited by
- 5
- References
- 32
- Citations by year
- 2025: 2 · 2024: 1 · 2023: 2
- FWCI
- 1.19
- Type
- journal article
- Language
- en
- Update notices
- erratum (2023-05-01)
- Open access
- bronze
- doi.org
- Licence
- Locations
- The Journal of Heart and Lung Transplantation (acceptedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- CHEST Foundation
- Cystic Fibrosis Foundation Therapeutics
- National Institutes of Health
- Cystic Fibrosis Foundation
- United Network for Organ Sharing
- Topics
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Surgery › Organ Transplantation Techniques and Outcomes
- Keywords
- cystic fibrosis, CFTR genotype, lung transplantation, post-transplant survival, bronchiolitis obliterans syndrome, graft failure, retransplantation
- MeSH terms
- Bronchiolitis Obliterans, Cystic Fibrosis, Genotype, Humans, Retrospective Studies, United States, Lung Transplantation, Cystic Fibrosis Transmembrane Conductance Regulator
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