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

Background: Pulmonary arterial hypertension (PAM is a progressive and fatal disorder. Despite the emergence of effective therapy, PAM 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 PAM 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 PAM-specific therapy, or repeiving a diagnosis by right-sided heart catheterization. Results: In 21.1% of patients, symptoms were experienced for >2 years before PAM was recognized. Patients with onset of PAM 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 PAM recognition. Six-minute walk distance <250 in (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 PAM 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 PM! recognition. Trial registry: ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov CHEST 2011; 140(1)39-26