Chest
Volume 140, Issue 1, July 2011, Pages 19-26
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Original Research
Pulmonary Vascular Disease
Delay in Recognition of Pulmonary Arterial Hypertension: Factors Identified From the REVEAL Registry

https://doi.org/10.1378/chest.10-1166Get rights and content

Background

Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder. Despite the emergence of effective therapy, PAH is commonly at an advanced stage when recognized. Factors associated with a prolonged symptomatic period before the recognition of PAH have not been fully evaluated.

Methods

The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) enrolled 2,967 US adult patients with PAH from March 2006 to September 2007. Patients were considered to have delayed disease recognition if > 2 years elapsed between symptom onset and the patient receiving a PAH diagnosis, starting on PAH-specific therapy, or receiving a diagnosis by right-sided heart catheterization.

Results

In 21.1% of patients, symptoms were experienced for > 2 years before PAH was recognized. Patients with onset of PAH symptoms before age 36 years showed the highest likelihood of delayed disease recognition (OR, 3.07; 95% CI, 2.03–4.66). History of obstructive airways disease (OR, 1.93; 95% CI, 1.5–2.47) and sleep apnea (OR, 1.72; 95% CI, 1.33–2.22) were independently associated with delayed PAH recognition. Six-minute walk distance < 250 m (OR, 1.91; 95% CI, 1.16–3.13), right atrial pressure < 10 mm Hg (OR, 1.77; 95% CI, 1.26–2.48), and pulmonary vascular resistance < 10 Wood units (OR, 1.28; 95% CI, 1.02–1.60) were also associated with delayed disease recognition, but sex, race/ethnicity, and geographic region showed no association.

Conclusions

One in five patients in the REVEAL Registry who were diagnosed with PAH reported symptoms for > 2 years before their disease was recognized. Younger individuals and patients with histories of common respiratory disorders were most likely to experience delayed PAH recognition.

Section snippets

Design Overview

We conducted a cohort study among patients enrolled in the REVEAL Registry between March 1, 2006, and September 30, 2007. The study was approved by the institutional review board at each participating center (e-Appendix 1). Subjects provided written informed consent for collection of baseline and follow-up data. The design of the REVEAL Registry has been described in detail previously.20 Briefly, patients with PAH who were either previously diagnosed or newly diagnosed (defined as having a

Results

Of the 2,967 patients with PAH enrolled in the REVEAL Registry (Fig 1), we excluded 200 patients younger than 19 years at the time of diagnostic RHC. A total of 242 patients (8.2% of the enrolled population) had a PCWP or LVEDP > 15 to ≤ 18 mm Hg. A total of 2,525 patients met the conventional hemodynamic criteria for PAH. Thirty-two of these patients were excluded because of a missing date of PAH symptom onset. The final study population consisted of 2,493 patients, of whom 526 (21.1%) had

Discussion

Hemodynamic measurements are required to make a diagnosis of PAH.24 However, the REVEAL Registry is designed to capture “real-world” clinical practice where patients, ultimately having a valid diagnosis of PAH, may be told of a diagnosis of PAH, or started on PAH-specific therapy prior to the performance of the RHC that qualified them for the REVEAL Registry. This may have been due to an incomplete assessment of PAH, but occasionally the hemodynamic parameters required for REVEAL Registry entry

Acknowledgments

Author contributions: Dr Brown: contributed to the study design; collection, analysis and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Dr Chen: contributed to the study design; collection, analysis and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Dr Halpern: contributed to the study design; collection, analysis and interpretation of data; drafting and

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    Funding/Support: The REVEAL Registry is sponsored by Actelion Pharmaceuticals US, 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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