Results of a national study indicate that 20% of all patients with pulmonary arterial hypertension (PAH) suffer with the disease for more than 2 years before obtaining an accurate diagnosis and proper treatment.

“For a lot of patients, that means the treatment is more difficult and the damage is irreversible,” said lead author Lynnette Brown, MD, PhD, a pulmonologist and researcher from Intermountain Medical Center. “Finding out which patients are getting a delayed diagnosis is the first step in identifying them earlier, when treatment is easier and hopefully more effective.”

Among the nearly 3,000 adults with PAH who participated in the study, those with symptom onset before age 36 displayed the highest likelihood of delayed recognition (PAH diagnosis, start of PAH-specific therapy, or diagnosis by right-sided heart catheterization). History of obstructive airway disease and sleep apnea were also independently associated with a delayed recognition of PAH. Dr. Brown and colleagues also found that a 6-minute walk distance of less than 250 meters, right atrial pressure less than 10 mmHg, and pulmonary vascular resistance of less than 10 Wood units were associated with delayed recognition, whereas sex, race/ethnicity, and geographic region were not.

“We have a lot more medications available to fight pulmonary arterial hypertension, but we can’t use them all if we don’t get to patients early enough in the course of the disease,” said co-author, Gregory Elliott, MD, chairman of the department of medicine at Intermountain Medical Center. “If we can treat these patients sooner, we may find that we can improve survival.”

Drs. Elliot and Brown hope the study results provide guidance in diagnosing PAH. “If a young person comes in complaining of shortness of breath, it’s alright to suspect something common,” added Dr. Brown. “But if a patient is getting worse and not responding to treatment, it’s time to look for something else.”

PAH Study Abstract

Physicians Weekly wants to know…

• Do you think low rates of health insurance among young patients cause this population to be less likely to seek early treatment for PAH? Can that, in turn, cause delays in recognition?

• Do you think that PAH symptoms—shortness of breath, fatigue, swelling, and chest pain—are commonly mistaken for other common disorders, like asthma, and that this may result in misdiagnoses?

• What interventions would you recommend to achieve better rates of early diagnosis in patients with PAH?