What Is PAH?

If you've recently been diagnosed with pulmonary arterial hypertension (PAH), you may be feeling confused and asking yourself, "What is PAH?" There's a lot to understand and the more you know, the better equipped you'll be to manage your condition. Managing your disease is a journey and we're here to help you start your journey on the right foot. Let's begin with a few basics:

PAH is a disease of the vessels that carry blood from the heart to the lungs. In PAH, there is a constant state of high blood pressure in the vessels of the lungs. It is a progressive disease—meaning that it gets worse over time. It is not common but it affects people of all ages and ethnic backgrounds. PAH can occur on its own, or it can be related to other causes.

Understanding the basics of PAH

Knowing how the cardiovascular system works will help you better understand PAH. The cardiovascular system includes your blood vessels and your heart. The vessels that carry blood from your heart to your lungs are called pulmonary arteries. When they are healthy, these vessels are large and flexible—this means they are very open and elastic, and blood can easily pass from the heart, through the pulmonary arteries, and into the lungs, ultimately moving oxygen out to the rest of the body.

How does the cardiovascular system work?

The vessels that carry blood from your heart to your lungs are called "pulmonary arteries." When they are healthy, these vessels are large and flexible—this means they are very open and elastic and blood can easily pass throughout the body.

In PAH, the pulmonary arteries tighten up and become stiff. This causes more resistance to the blood flowing through the arteries and causes the heart to work harder to push blood through the narrow artery. When this happens, less and less blood is able to flow to the lungs, and more and more symptoms begin to appear.3

Healthy pulmonary artery is open and elastic; blood flows through easily* Artery with early signs of resistance to blood flow* Artery with advanced narrowing and stiffening due to blood vessel wall thickening, scar tissue, and clotting4*

*Statement is based on laboratory studies. The clinical significance in humans is unknown.

PAH causes changes in the heart

In PAH, the heart gets bigger. The right ventricle has to work harder to pump blood against higher resistance in the arteries. As the heart grows, it starts to compress its left side. This makes it harder for the left side of the heart to do its job of circulating blood to the rest of the body, in turn causing more and more symptoms to begin to appear.

As this cycle continues, the strain on the right side of the heart may become too great, resulting in a condition known as "right-heart failure." This doesn't mean that the heart stops. But it does mean that the heart works less efficiently, because there is less blood flowing through the pulmonary artery.

What is PAH


VENTAVIS® (iloprost) Inhalation Solution 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.

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 should I tell my doctor before taking 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. Your doctor may need to give you a lower dose of VENTAVIS.
  • are pregnant, or plan to become pregnant. It is not known if VENTAVIS can harm your unborn baby. VENTAVIS should only be used during pregnancy if the benefit to you is worth the possible risk to your baby.
  • are breastfeeding. It is not known if VENTAVIS passes into your breast milk. You and your doctor should decide if you will take VENTAVIS or breastfeed.

Tell your doctor about all the medicines you take, including prescription and nonprescription medicines, vitamins, and herbal supplements.

VENTAVIS and other medicines may affect each other, causing side effects. VENTAVIS may affect the way other medicines work, and other medicines may affect how VENTAVIS works.

Especially tell your doctor if you take:

  • medicines used to treat high blood pressure or heart problems
  • medicines that lessen blood clotting (warfarin, Coumadin, Jantoven)

Know the medicines you take. Keep a list of your medicines and show it to your doctor and pharmacist when you get a new medicine.

How should I take VENTAVIS?

  • Take VENTAVIS exactly as your doctor tells you to take it. Your doctor may change your dose if needed.
  • You should not take VENTAVIS more than every 2 hours. The benefits of VENTAVIS may not last 2 hours, so you may adjust the times that you use it to cover planned activities.
  • Do not drink VENTAVIS.
  • Do not let VENTAVIS solution come into contact with your skin or eyes. If it does, rinse your skin or eyes with water right away.
  • Do not allow other people to be exposed to VENTAVIS while you are breathing it, especially babies.
  • If you take too much VENTAVIS, you may have a headache, red face, dizziness, nausea, vomiting and diarrhea. If this happens stop taking VENTAVIS. If your symptoms do not go away, call your doctor or get emergency help right away.

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 your 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.

Other important side effects of VENTAVIS include:

  • bleeding
  • red face (flushing)
  • increased cough
  • low blood pressure
  • headaches
  • nausea
  • spasm of your jaw muscles that makes it hard to open your mouth

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 FDA at 1-800-FDA-1088.

Please see full Prescribing Information and Patient Information.