Survival in pulmonary arterial hypertension patients awaiting lung transplantation

Mardi Gomberg-Maitland, Cherylanne Glassner-Kolmin, Sydeaka Watson, Robert Frantz, Myung Park, Adaani Frost, Raymond L. Benza, Fernando Torres

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1179-1186
Number of pages8
JournalJournal of Heart and Lung Transplantation
Volume32
Issue number12
DOIs
StatePublished - Dec 2013

Fingerprint

Lung Transplantation
Pulmonary Hypertension
Survival
Lung
Transplants
Cardiac Output
Registries
Disease Progression
Transplantation
Hemodynamics
Confidence Intervals
Oxygen

Keywords

  • Lung allocation score
  • Lung transplant
  • Pulmonary arterial hypertension
  • Survival prediction model
  • Waitlist

ASJC Scopus subject areas

  • Transplantation
  • Cardiology and Cardiovascular Medicine
  • Pulmonary and Respiratory Medicine
  • Surgery

Cite this

Gomberg-Maitland, M., Glassner-Kolmin, C., Watson, S., Frantz, R., Park, M., Frost, A., ... Torres, F. (2013). Survival in pulmonary arterial hypertension patients awaiting lung transplantation. Journal of Heart and Lung Transplantation, 32(12), 1179-1186. https://doi.org/10.1016/j.healun.2013.08.016

Survival in pulmonary arterial hypertension patients awaiting lung transplantation. / Gomberg-Maitland, Mardi; Glassner-Kolmin, Cherylanne; Watson, Sydeaka; Frantz, Robert; Park, Myung; Frost, Adaani; Benza, Raymond L.; Torres, Fernando.

In: Journal of Heart and Lung Transplantation, Vol. 32, No. 12, 12.2013, p. 1179-1186.

Research output: Contribution to journalArticle

Gomberg-Maitland, M, Glassner-Kolmin, C, Watson, S, Frantz, R, Park, M, Frost, A, Benza, RL & Torres, F 2013, 'Survival in pulmonary arterial hypertension patients awaiting lung transplantation', Journal of Heart and Lung Transplantation, vol. 32, no. 12, pp. 1179-1186. https://doi.org/10.1016/j.healun.2013.08.016
Gomberg-Maitland, Mardi ; Glassner-Kolmin, Cherylanne ; Watson, Sydeaka ; Frantz, Robert ; Park, Myung ; Frost, Adaani ; Benza, Raymond L. ; Torres, Fernando. / Survival in pulmonary arterial hypertension patients awaiting lung transplantation. In: Journal of Heart and Lung Transplantation. 2013 ; Vol. 32, No. 12. pp. 1179-1186.
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abstract = "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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AU - Benza, Raymond L.

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N2 - 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.

AB - 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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