Study May Lead to Improved Treatment for Lung Disease

One-fifth of all patients with pulmonary arterial hypertension suffer with the fatal disease for more than two years before being correctly diagnosed and properly treated, according to a new national study led by researchers at Intermountain Medical Center in Murray, Utah.

“For a lot of patients, that means the treatment is more difficult and the damage is irreversible,” said Lynnette Brown, MD, PhD, a pulmonologist and researcher at Intermountain Medical Center and lead author of the study, which is published this week in the July issue of Chest, the journal of the American College of Chest Physicians.

“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,” she said.

Pulmonary arterial hypertension (PAH) is a rare but fatal disease that occurs when small arteries in the lungs become narrowed and unable to carry as much blood as healthy arteries. Pressure builds as the heart works harder to move blood into the lungs. Eventually, the heart may fail.

Treatment options have improved, extending the lives of many patients. But treatment may be less effective for patients who receive a delayed diagnosis.

The study found that:

More than 21 percent of patients with PAH had symptoms for over two years before being diagnosed and beginning treatment.

Patients younger than 36 years old were most likely to receive a delayed diagnosis.

Patients who previously had been diagnosed with a common respiratory disorder such as obstructive airway disease or sleep apnea were also more likely to have a delayed diagnosis.

Dr. Brown and her colleagues gave several possible reasons for delayed diagnosis:

Symptoms — shortness of breath, fatigue, swelling, and chest pain — can also point to more common disorders, such as asthma.

Younger patients are typically more active than older patients and notice symptoms when they are subtler. But because the symptoms are less severe, physicians may be less likely to order testing to confirm PAH.

Younger patients make up one of the largest groups of uninsured Americans and therefore are less likely to seek early treatment.

Earlier diagnosis and treatment are vital, said Gregory Elliott, MD, chairman of the Department of Medicine at Intermountain Medical Center and another member of the research team.

“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. If we can treat these patients sooner, we may find that we can improve survival,” he said.

Researchers hope the study will give physicians more guidance in diagnosing pulmonary arterial hypertension.

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

The study examined the medical records of 2,493 PAH patients from the national REVEAL registry (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management), including many of the 200 patients who are being treated at Intermountain Medical Center or University Hospital, the only hospitals in the Intermountain region capable of treating the complicated disease at an advanced stage.

Co-authors were from the University of Utah; University of California, San Francisco; University of Pennsylvania; the Mayo Clinic; Boston University Medical Center; and Baylor College of Medicine.

Intermountain Medical Center serves as the flagship facility for the Intermountain Healthcare system.