Survival in pulmonary arterial hypertension patients awaiting lung transplantation
Open accessAbstract
Background Pulmonary arterial hypertension (PAH) is a progressive disease with lung transplantation as the only option for those patients refractory to medical therapy. Although several equations have been developed to predict PAH patient survival, it is unclear whether they can predict survival for patients awaiting transplantation.Methods Data were analyzed on 827 patients listed since 1991 on the Scientific Registry of Transplant Recipients. Overall survival and survival for patients listed prior to and after January 1, 2006 was estimated using the Kaplan-Meier (K-M) method and compared with predicted survival from the pulmonary hypertension connection (PHC) and lung allocation system (LAS) equations. A new equation using a novel model selection algorithm for correlated covariates and missing data was developed using clinical factors and variables in the LAS score. Model validation statistics were calculated and averaged across 500 bootstrap resamples within each of 5 imputation data sets. K-M with 95% confidence intervals and receiver-operator characteristic (ROC) curves assessed model performance.Results PHC predicted overall survival but underestimated and overestimated survival for those listed pre- and post-2006, respectively. The best model included baseline 6-minute walk distance (6MWD), invasive cardiac output and resting oxygen requirement (O2). Factors associated with 1-year waitlist survival included: resting O2 amount; invasive hemodynamics; 6MWD; and functional class. The new equation by ROC analysis outperformed the LAS and PHC equations.Conclusions Current prediction models overestimate survival for PAH patients listed for transplant in the LAS era. This new survival equation can help guide clinicians caring for PAH patients with progression of disease requiring transplant.
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In countries where lung allocation scores do not fully capture waitlist mortality for pulmonary arterial hypertension, early transplant consideration is particularly important.
“This issue is of particular importance in countries where a lung allocation system utilizes a score that does not fully capture the waitlist mortality for individuals with PAH (276–280).”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [278]
The Discussion (page unavailable) says the lung allocation score disadvantages PAH patients, that better assessment of transplant urgency could improve outcomes, and that PAH patients may be listed late. The paper examines the US allocation system, however; it does not establish the same concern for allocation systems in other countries. (LLM, conf=0.82)
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- Authors
- Gomberg-Maitland, M., Glassner-Kolmin, C., Watson, S., Frantz, R., Park, M., Frost, A., Benza, R. L., & Torres, F.
- Affiliations
- University of Chicago (US)
- Mayo Clinic (US)
- University of Maryland, Baltimore (US)
- Baylor College of Medicine (US)
- Allegheny General Hospital (US)
- The University of Texas Southwestern Medical Center (US)
- Venue
- The Journal of Heart and Lung Transplantation
- Publisher
- Elsevier BV
- Published
- 2013-12
- Volume
- 32
- Issue
- 12
- Pages
- 1179-1186
- ISSN
- 1053-2498 (ISSN-L)
- OpenAlex
- W2076842262
- Semantic Scholar
- db5254c94353dbcfcdacedae543c9cc50c014541
- Cited by
- 47
- References
- 28
- Citations by year
- 2026: 2 · 2025: 3 · 2024: 1 · 2023: 3 · 2022: 2 · 2021: 8 · 2020: 2 · 2019: 2 · 2018: 7 · 2017: 6 · 2016: 2 · 2015: 3 · 2014: 5 · 2013: 1
- FWCI
- 2.98
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- The Journal of Heart and Lung Transplantation (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Novartis
- Gilead Sciences
- Bayer
- GlaxoSmithKline
- Actelion Pharmaceuticals
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Keywords
- pulmonary arterial hypertension, lung transplantation, survival prediction, waitlist survival, 6-minute walk distance, invasive hemodynamics, functional class, prediction model, missing data imputation, model selection
- MeSH terms
- Cardiac Output, Female, Hemodynamics, Humans, Hypertension, Pulmonary, Male, Registries, Retrospective Studies, Waiting Lists, Models, Statistical, Survival Rate, Lung Transplantation, Walking, Kaplan-Meier Estimate, Familial Primary Pulmonary Hypertension
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