The importance of an accurate PAH* diagnosis
PAH* is often not diagnosed early in the course of the disease. Often a diagnosis may be delayed for months or even years. Undiagnosed pulmonary arterial hypertension* patients who do not receive treatment may experience more rapid worsening of symptoms or physical condition than patients who are on a doctor-prescribed treatment plan.
PAH* is a rare disease that starts with symptoms that are easily confused with those of other conditions, such as asthma or other lung diseases. Thus, patients may be misdiagnosed and receive inappropriate treatment for their symptoms. That's why a series of tests may be performed to rule out other conditions and confirm PAH*.
Making a diagnosis
Once PAH* symptoms are detected, there is an array of diagnostic testing tools that your healthcare team may want to use. For an accurate diagnosis to be made, certain tests may be performed either to rule out other conditions and/or to confirm PAH*. If PAH* is suspected, your doctor may perform the following PAH tests:
- Electrocardiogram (ECG)
- Chest X-ray
- Doppler Echocardiogram
- Pulmonary function test
The definitive test to make a diagnosis is a right-heart catheterization. This test may also evaluate the severity of PAH* by measuring pressure and blood flow changes in the pulmonary arteries and right side of the heart.3 A thin tube with a special tip is inserted into a vein in the neck or groin and threaded into the heart and the pulmonary artery, where it measures pressure.3
Your healthcare provider might order other kinds of tests to learn more about your condition or to rule out other diseases.
Classifying your symptoms
Once a PAH* diagnosis has been made, your healthcare provider may determine your Functional Class. The Functional Class system categorizes the severity of your disease according to your symptoms. Functional Class describes how symptoms, such as shortness of breath, chest pain, or lightheadedness, limit your physical activity.
The 6-Minute Walk Test
The 6-Minute Walk Test is a tool to evaluate exercise capacity. For the test, you walk as far as possible in 6 minutes. Before, during, and after the test, you will be asked to report how short of breath you are. Your healthcare provider may ask you to take the 6-MWT before you begin and several times during your treatment.