What is pulmonary arterial hypertension?
Pulmonary arterial hypertension (PAH) is high blood pressure in the arteries of the lungs (called pulmonary arteries). Pulmonary arteries carry blood from the heart into the lungs, where it picks up oxygen for delivery to the rest of the body.
The effects of PAH can make it difficult to perform certain daily activities, such as walking.
People with PAH may experience
- Dizziness and/or fainting (syncope)
- Shortness of breath (dyspnea)
- Chest pain (angina)
- Feeling tired or worn out (fatigue)
- Swollen ankles and legs (edema)
- Swollen abdomen
- Rapid, hard, or irregular heartbeats (palpitations)
PAH is a progressive disease
PAH worsens over time and can lead to serious complications. The physical symptoms you experience may not always reflect whether PAH is progressing or how it is affecting your heart. If you believe you have PAH, it's important to talk to your healthcare provider as early as possible so it can be determined if you have PAH and whether or not you need to begin treatment.
Your healthcare provider may perform some tests to initially diagnose your PAH
6-minute walk distance (6MWD)
Tracks how far you can walk in 6 minutes.
Uses sound waves to see how well your heart is beating and pumping blood.
Pulmonary function tests
Measure how well your lungs work.
Shows if your pulmonary arteries and right ventricle are enlarged.
Measures breathing and circulation in all areas of your lungs.
Rule out other diseases that may be causing your symptoms.
If PAH is suspected, a right heart catheterization may be performed to confirm the diagnosis
Right heart catheterization measures the pressure in your pulmonary arteries and shows how well your heart is pumping blood to the rest of your body.
Prostacyclin analogues are an FDA-approved treatment for PAH
In a person with PAH, the body produces low levels of prostacyclin. Prostacyclin is a natural substance that helps keep arteries in the lungs open and working properly. When the body does not produce enough prostacyclin, the pulmonary arteries become narrow. This causes the heart to have to work harder to do its job.
Prostacyclin analogues mimic the effects of the natural prostacyclin found in the body. Treatment with a prostacyclin analogue may make it easier for your heart to pump blood through your lungs. This can help improve your ability to exercise.
Experts have supported the use of prostacyclin-class therapy for over a decade.
Prostacyclin therapies can be taken 3 different ways
- Pump therapy—a small pump is used to administer medicine directly into your body (through your bloodstream or under your skin) by way of a thin tube called a catheter
- Inhaled therapy—a handheld device is used to inhale the medicine, delivering the medicine directly to your lungs
- Oral therapy—medicine is administered through a pill that you take by mouth