Early Bird Gets the PAH Treatment

Pulmonary arterial hypertension treatment too often delayed

/ Author:  / Reviewed by: Joseph V. Madia, MD

(RxWiki News) The dated saying, “It’s never too late,” doesn’t always hold true, especially when it comes to your health. Timing is everything when diagnosing a serious illness like pulmonary arterial hypertension (PAH).

For many conditions including HIV, cancer and diabetes, early treatment minimizes the progression of the disease and maximizes the patient's chance of survival and quality of life. Add PAH to the list as well.

An estimated 20 percent of all patients with PAH suffer more than two years before receiving a proper diagnosis and appropriate treatment.

"See your doctor if you're short of breath and tired."

PAH is a rare, fatal disease that occurs when small arteries in the lungs narrow and are unable to carry sufficient amounts of blood. As the heart works harder to pump blood to the lungs, pressure builds, putting the patient at risk of heart failure.

Lead author Lynnette Brown, M.D., a pulmonologist and researcher at Intermountain Medical Center in Murray, Utah, reports that many patients are left with few treatment options when their diagnosis is delayed too long. During that time, the disease progresses and results in irreversible heart damage.

Co-author, Gregory Elliott, M.D., chairman of the Department of Medicine at Intermountain Medical Center, adds that the earliest possible diagnosis is critical. There are many more medications available to fight PAH, but they are useless if the disease has already progressed too far.

Patients who are diagnosed and treated early have significantly improved chances of survival.

Dr. Brown also recommends that physicians caring for young people whose shortness of breath and fatigue are not improving with standard treatment should consider testing for more serious diseases like PAH.

Patients younger than 36 years old are the most likely to receive a late diagnosis. One reason for delayed diagnosis is because some patients had been previously diagnosed with other respiratory conditions like sleep apnea or obstructive airway disease.

Dr. Brown identified three main reasons for late diagnosis:

  • Symptoms of PAH, including shortness of breath, swelling, chest pain and fatigue, are also symptoms of common disorders like asthma.

  • Younger people are more active than older patients and may notice symptoms when they are more subtle, which physicians are more likely to overlook.

  • Young people make up the largest group of uninsured Americans and are less likely to seek medical treatment.

This case review study examined the medical records of 2,493 PAH patients from a national registry and is published in the July 2011 issue of the journal Chest.

Reviewed by: 
Review Date: 
July 12, 2011
Last Updated:
July 15, 2011