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

Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry.

Author information

1
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Intermountain Medical Center, Murray, UT; Department of Internal Medicine, Pulmonary Division, University of Utah, Salt Lake City, UT.
2
Department of Medicine, Pulmonary and Critical Care Division, University of California, San Francisco, CA.
3
Department of Medicine, Pulmonary, Allergy and Critical Care Division, University of Pennsylvania, Philadelphia, PA.
4
Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN.
5
Pulmonary, Allergy, Sleep, and Critical Care Medicine, Boston University Medical Center, Boston, MA.
6
Section of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine, Houston, TX.
7
Department of Internal Medicine, Pulmonary Division, University of Utah, Salt Lake City, UT.
8
Statistical Analysis, ICON Clinical Research, San Francisco, CA.
9
Clinical Operations, Actelion Pharmaceuticals US, Inc, South San Francisco, CA.
10
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Intermountain Medical Center, Murray, UT; Department of Internal Medicine, Pulmonary Division, University of Utah, Salt Lake City, UT. Electronic address: greg.elliott@imail.org.

Abstract

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.

PMID:
21393391
PMCID:
PMC3198486
DOI:
10.1378/chest.10-1166
[Indexed for MEDLINE]
Free PMC Article
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