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Original Research: PULMONARY VASCULAR DISEASE |

Integration of Clinical and Hemodynamic Parameters in the Prediction of Long-term Survival in Patients With Pulmonary Arterial Hypertension

Garvan C. Kane, MD, PhD, FCCP; Hilal Maradit-Kremers, MD; Josh P. Slusser, BS; Chris G. Scott, MS; Robert P. Frantz, MD; Michael D. McGoon, MD, FCCP
Author and Funding Information

From the Pulmonary Hypertension Clinic (Drs Kane, Frantz, and McGoon), Division of Cardiovascular Diseases, Department of Medicine; and the Department of Health Sciences Research (Dr Maradit-Kremers and Messrs Slusser and Scott), Mayo Clinic, Rochester, MN.

Correspondence to: Garvan Kane, MD, PhD, FCCP, Division of Cardiovascular Diseases, Department of Medicine, Gonda 5, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: kane.garvan@mayo.edu


For editorial comment see page 1263

Funding/Support: This project was supported by an unrestricted research grant from Pfizer Inc.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;139(6):1285-1293. doi:10.1378/chest.10-1293
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Background:  Current management guidelines for pulmonary arterial hypertension (PAH) recommend a treatment choice based primarily on World Health Organization (WHO) functional class. This study was designed to assess how the incorporation of readily obtained clinical and test-based information may significantly improve the prediction of outcomes over functional class alone.

Methods:  Clinical and hemodynamic variables were assessed in 484 consecutive patients presenting with WHO group 1 PAH. The primary outcome measure was time to all-cause mortality over 5 years from the index presentation (data available in all). Follow-up was censored at the time of lung or heart/lung transplant in 21 patients or at 5 years. Predictors of mortality were assessed sequentially using Cox models, with the step-wise incorporation of clinical variables, echocardiographic, and catheterization findings. Results were further compared with the REVEAL (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) prediction score.

Results:  Overall median survival was 237 weeks (95% CI, 196-266), corresponding to 1-year, 3-year, and 5-year survival rates of 81.1% (77.0, 84.7), 61.1% (56.5, 65.3), and 47.9% (43.2, 52.4), respectively. The prediction of mortality was improved incrementally by incorporating clinical and echocardiographic measures with a concordance index (c-index) of 0.84 compared with that of 0.60 with functional class alone. The REVEAL prediction score was validated independently in this cohort to predict both 1-year and 5-year mortality. It had a prediction c-index of 0.71.

Conclusions:  The integration of routine PAH clinical (predominantly noninvasive) parameters predicts long-term outcome better than functional class and, hence, should be incorporated into medical management decisions.

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