Chest
Volume 139, Issue 6, June 2011, Pages 1285-1293
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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

https://doi.org/10.1378/chest.10-1293Get rights and content

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.

Section snippets

Study Population and PAH Definition

All patients agreed to the use of their data for research purposes, and the study was approved by the Mayo Clinic institutional review board (Committee Expedited Review B-IRB No. 06-005447). The study included all adult patients (≥ 18 years) who fulfilled the contemporary diagnostic criteria for WHO group 1 pulmonary hypertension (PH) (mean pulmonary arterial pressure ≥ 25 mm Hg occurring in the setting of increases in precapillary pulmonary resistance) and were first seen (January 1, 1995, to

Clinical Characteristics

Clinical, laboratory, and hemodynamic characteristics at the time of diagnosis of the 484 individual subjects with WHO group 1 PH are shown in Table 1. A total of 272 subjects (56%) had idiopathic, familial, or anorexigenic PAH, 114 (24%) had PAH in the setting of connective tissue disease, and the remainder were associated with congenital systemic to pulmonary shunts (n = 45), portal hypertension (n = 51), and HIV (n = 2).

The majority of patients (75%) were women. The mean age was 52 ± 15

Discussion

The present study expands the available information of survival patterns in a large cohort of patients with PAH and provides new information on which clinical variables provide incremental prediction of survival. The emphasis of this article is on examining the usefulness of integrating the intrinsic characteristics (demographics and measurable clinical parameters) into the estimation of future risk of death in PAH. Our findings indicate that clinical parameters have the highest impact on

Conclusions

Our findings suggest that routine PAH clinical parameters can significantly enhance prediction of survival over functional class alone. Patients with PAH who are functional class II-III but have a REVEAL risk score > 8 have a risk equivalent to those patients presenting in functional class IV. We propose that these patients be considered for aggressive treatment protocols such as first-line parenteral prostacyclin analog therapy and suggest that this approach be tested in future studies.

Acknowledgments

Author contributions: Dr Kane had access to the data and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Dr Kane: contributed to study concept and design, analysis and interpretation of the data, and drafting of the manuscript.

Dr Maradit-Kremers: contributed to study concept and design, analysis and interpretation of the data, statistical expertise, and critical revision of the manuscript.

Mr Slusser: contributed to study concept and design, analysis and

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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).

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