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Original Research: PULMONARY VASCULAR DISEASE |

Delay in Recognition of Pulmonary Arterial HypertensionDelay Recognizing Pulmonary Arterial Hypertension: Factors Identified From the REVEAL Registry

Lynette M. Brown, MD, PhD, FCCP; Hubert Chen, MD, FCCP; Scott Halpern, MD, PhD; Darren Taichman, MD, PhD, FCCP; Michael D. McGoon, MD, FCCP; Harrison W. Farber, MD, FCCP; Adaani E. Frost, MD, FCCP; Theodore G. Liou, MD, FCCP; Michelle Turner, MS; Kathy Feldkircher, PhD; Dave P. Miller, MS; C. Gregory Elliott, MD, FCCP
Author and Funding Information

From the Department of Medicine, Division of Pulmonary and Critical Care Medicine (Drs Brown and Elliott), Intermountain Medical Center, Murray, UT; the Department of Internal Medicine, Pulmonary Division (Drs Brown, Liou, and Elliott), University of Utah, Salt Lake City, UT; the Department of Medicine, Pulmonary and Critical Care Division (Dr Chen), University of California, San Francisco, CA; the Department of Medicine, Pulmonary, Allergy and Critical Care Division (Drs Halpern and Taichman), University of Pennsylvania, Philadelphia, PA; the Division of Cardiovascular Diseases (Dr McGoon), Mayo Clinic, Rochester, MN; the Pulmonary, Allergy, Sleep, and Critical Care Medicine (Dr Farber), Boston University Medical Center, Boston, MA; the Section of Pulmonary, Critical Care, and Sleep Medicine (Dr Frost), Baylor College of Medicine, Houston, TX; Statistical Analysis (Ms Turner and Mr Miller), ICON Clinical Research, San Francisco, CA; and Clinical Operations (Dr Feldkircher), Actelion Pharmaceuticals US, Inc, South San Francisco, CA.

Correspondence to: C. Gregory Elliott, MD, FCCP, Department of Internal Medicine, Pulmonary Arterial Hypertension Center, Intermountain Medical Center, 5121 S Cottonwood St, Ste 307, PO Box 577000, Murray, UT 84157-7000; e-mail: greg.elliott@imail.org


For editorial comment see page 4

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).


© 2011 American College of Chest Physicians


Chest. 2011;140(1):19-26. doi:10.1378/chest.10-1166
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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.

Trial registry:  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov

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