At present, pulmonary arterial hypertension (PAH) is difficult to diagnose via routine medical examinations. To detect PAH, medical professionals must first recognize the signs and symptoms of PAH. If your physician suspects you may suffer from PAH, your health care provider will order a series of preliminary diagnostic tests and examinations which may include:

Chest X-rays
Echocardiogram
Electrocardiogram
Blood tests
Pulmonary function tests
Nuclear Scan

The results of the preliminary tests will determine if your condition warrants a right-heart catheterization. Widely understood as the current “Gold Standard” in PAH diagnostics, the right-catheterization involves the insertion of a probe through the groin or neck into the right side of the heart to measure the pressure levels of the pulmonary arteries.

Upon diagnosis, today’s PAH patients have available more FDA approved therapies than ever before to manage and slow progression of the disease, allowing those suffering from PAH to live more prolonged and productive lives. Research has shown significant progress in the area of increased life spans for PAH patients who commence treatment in early stages of the disease. A comprehensive list of treatment options for PAH patients may be found here. http://www.phassociation.org/Treatments.

Early detection and diagnosis is paramount to saving and extending the lives of PAH patients. Unfortunately, PAH is often detected in advanced stages and near the end of a PAH patient’s average life expectancy. Did you know:

On average, PAH patients are consulted by 3 or more physicians before diagnosis

PAH patient’s generally incur 2.8 years between symptom onset and diagnosis via right heart catheterization

Left untreated, the average life expectancy of a PAH patient is approximately 3 years from symptom onset

74% of PAH patients are diagnosed in advance stages of the disease

Currently, 14 FDA approved therapies are available to manage and slow progression of the disease, the first of which was approved in 1995.

In the past 20 years, no improvement has been achieved in early detection rates of PAH

5 year survival rates are measured at 86% for PAH patients diagnosed in WHO Classes 1-2 with appropriate treatment. 5 year survival rates for PAH patients diagnosed in WHO Classes 3-4 with treatment decrease to approximately 50%

The Blue Lips Foundation is dedicated to changing the future of PAH through education of the gatekeepers of medicine for all ages as to the signs and symptoms of PAH, and to provide them with tools to screen and detect the disease in less invasive formats. Our goal is to make certain that every person who is inflicted with PAH is diagnosed early and efficiently. We believe this is the only method to keep patients alive long enough for PAH therapies to take hold and add quality years to their lives.