Delay in Recognition of Pulmonary Arterial Hypertension

• DOI: 10.1378/chest.10-1166
• Publication Date: 2011-03-11T03:28:09Z
• Authors: Lynette M. Brown; Hubert Chen; Scott Halpern; Darren Taichman; Michael D. McGoon; Harrison W. Farber; Adaani E. Frost; Theodore G. Liou; Michelle Turner; Kathy Feldkircher; Dave P. Miller; C. Gregory Elliott
• Keywords: Adult; Male; Cardiac Catheterization; Time Factors; Hypertension, Pulmonary; Middle Aged; Prognosis; 3. Good health; Airway Obstruction; Diagnosis, Differential; Early Diagnosis; Sleep Apnea Syndromes; Disease Progression; Prevalence; Humans; Female; Pulmonary Wedge Pressure; Registries; Aged; Follow-Up Studies; Retrospective Studies

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