Significant Number of PAH Patients Going Undiagnosed, Untreated


One-fifth of all patients suffering from PAH go undiagnosed and thus untreated for over two years before their disease is correctly identified.

According to a recent national study, one-fifth of all patients suffering from pulmonary arterial hypertension (PAH) go undiagnosed and thus untreated for over two years before their disease is correctly identified.

"For a lot of patients, that means the treatment is more difficult and the damage is irreversible," said lead author of the study Lynnette Brown, MD, PhD, a pulmonologist and researcher at Intermountain Medical Centerin Salt Lake City. "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."

PAH is a scarcely seen, terminal disease that is characterized by tiny arteries in the lungs narrowing and becoming incapable to carry the same quantities of blood as healthy arteries. As a result, pressure builds up due to the heart working overtime in order to transport blood into the lungs. Ultimately, this can lead to heart failure.

While treatment possibilities have most certainly progressed, it is still critical for therapy to begin as soon as feasible, as with most diseases; the longer PAH goes untreated, the less effective the treatment is likely to be.

The study analyzed the medical records from the national Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) of 2,493 patients suffering from PAH. This number also included a majority of the 200 PAH patients who are being treated at Intermountain Medical Center or University Hospital, the only hospitals in the Intermountain area with the ability to effectively treat the deadly disease at an advanced stage.

The researchers found that over 21% of participants with PAH exhibited symptoms for more than two years prior to diagnosis and commencement of treatment.

Patients younger than thirty-six years old were most likely to receive a delayed diagnosis, as were patients who previously were diagnosed with a common respiratory disorder, such as obstructive airway disease or sleep apnea.

Brown and her fellow researchers were able to provide several possible reasons for delayed diagnosis. PAH usually presents with symptoms such as shortness of breath, fatigue, swelling, and chest pain, which can lead a physician to more than one common disorder, such as asthma.

Also, younger patients are characteristically more likely to have active lifestyles than older patients, and may only become aware of symptoms when they are subtler. Since these symptoms present as less severe, however, a physician may be less likely to proceed with testing which could diagnose PAH.

Brown’s final reasoning for delayed diagnosis in younger patients is the simple fact that young patients are a part of one of the largest groups of uninsured Americans, and therefore are less likely to seek medical treatment initially.

"We have a lot more medications available to fight pulmonary arterial hypertension” said Elliot, “but we can't use them all if we don't get to patients early enough in the course of the disease. If we can treat these patients sooner, we may find that we can improve survival.”

Researchers expressed the desire that the findings of this study will provide physicians with more effective guidance in terms of diagnosing pulmonary arterial hypertension.

"If a young person comes in complaining of shortness of breath, it’s alright to suspect something common,” stated Brown. “But if a patient is getting worse and not responding to treatment, it's time to look for something else.”

The study was published in the July issue of Chest, the journal of the American College of Chest Physicians.

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