One in five patients diagnosed with pulmonary arterial hypertension (PAH) in the United States report symptoms for more than 2 years before diagnosis.

PAH is a progressive and fatal disorder, and the disease is often advanced when recognized, especially in younger patients.

Researchers from Intermountain Medical Center, the University of California, University of Pennsylvania, Baylor College of Medicine, Boston University, and Mayo Clinic studied 2,967 adult patients enrolled in the Registry to Evaluate Early Long-term PAH Disease Management (REVEAL), for an 18-month period. In 21.1% of patients, symptoms were experienced for more than two years before PAH was recognized.

History of obstructive airways disease and sleep apnea were independently associated with delayed PAH recognition.  Despite progress in understanding the cellular and genetic basis of PAH, researchers found that the onset of PAH before age 36 years was associated with the greatest likelihood of delayed disease recognition, especially in individuals with histories of common respiratory disorders. This article is published in the July issue of CHEST, the peer-reviewed journal of the American College of Chest Physicians: Chest. 2011;140(1):19-26.

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