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

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


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:; No.: NCT00370214; URL:

Publication types

  • Comparative Study
  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Airway Obstruction / complications*
  • Airway Obstruction / diagnosis
  • Cardiac Catheterization / methods*
  • Diagnosis, Differential
  • Disease Progression
  • Early Diagnosis*
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / etiology
  • Male
  • Middle Aged
  • Prevalence
  • Prognosis
  • Pulmonary Wedge Pressure
  • Registries*
  • Retrospective Studies
  • Sleep Apnea Syndromes / complications*
  • Sleep Apnea Syndromes / diagnosis
  • Time Factors
  • United States / epidemiology

Associated data