Diagnosing PAH*

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.

*Patient Indication and Important Safety Information


*What is Ventavis?

Ventavis is a prescription medicine used to treat adults with certain kinds of severe pulmonary arterial hypertension (PAH), a condition in which blood pressure is too high in the blood vessels between the heart and the lungs. Ventavis may improve your ability to exercise and your symptoms for a short time by lowering your blood pressure and opening up the blood vessels in your lungs.

  • In the key clinical study, Ventavis showed clinical improvement as defined by 3 specific measurements: ability to exercise as measured by the 6-minute walk test, symptoms (NYHA Functional Class), and decrease in the worsening of PAH symptoms.
  • Ventavis is a medication you breathe in through a special device called the I-neb Adaptive Aerosol Delivery (AAD) System.

The study showing Ventavis is effective included mainly patients with NYHA Functional Class III-IV PAH. In these patients, PAH was caused by unidentified or hereditary factors (65%) or connective tissue diseases (23%).

Ventavis has not been studied in children younger than 18 years old.

What is the most important information I should know about Ventavis?

Ventavis may not be right for you. Before taking Ventavis, tell your doctor about all of your medical conditions, including if you have liver or kidney problems; are pregnant, or plan to become pregnant; or are breast-feeding. Tell your doctor about all the medicines you take, including prescription and nonprescription medicines, vitamins, and herbal supplements. Especially tell your doctor if you take medicines used to treat high blood pressure or heart problems or medicines that lessen blood clotting (warfarin, Coumadin, Jantoven). Ventavis and other medicines may affect each other, causing side effects.

What are the possible side effects of Ventavis?

  • Ventavis may cause side effects, including feeling dizzy, lightheaded, and faint. If you have any of these side effects, you should stand up slowly when you get out of chairs or bed. Tell your doctor if your fainting gets worse during treatment with Ventavis. Your doctor may need to change your dose or treatment.
  • Do not drive a car or operate any tools or machines if dizziness or fainting from low blood pressure is a problem for you.
  • You may have trouble breathing after taking Ventavis, because it may cause the muscles around your airway to tighten (bronchospasm). Get emergency help right away if you have trouble breathing.
  • The most common side effects of Ventavis include red face (flushing), increased cough, low blood pressure, headaches, nausea, spasm of your jaw muscles that makes it hard to open your mouth, and fainting.

Talk to your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of Ventavis. For more information, ask your doctor or pharmacist.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Please see full Prescribing Information

For more information about Ventavis, please call 1-866-ACTELION (1-866-228-3546).