What Is Pulmonary Arterial Hypertension?

Written by Madeline Laguaite
Medically Reviewed by Shruthi N, MD on August 19, 2025
8 min read

Pulmonary arterial hypertension (PAH) is a type of pulmonary hypertension. It means that you have high blood pressure in the arteries that go from your heart to your lungs. But it’s different from having regular high blood pressure. 

With PAH, the tiny arteries in your lungs become narrow or blocked. It’s harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries. 

PAH is a progressive condition that gets worse as time goes on. After a while, the heart muscle gets weak. Eventually, it can lead to heart failure.

 

A few things can cause pulmonary arterial hypertension. 

Genes may play a role in why some people get it. When that’s the case, it’s called heritable pulmonary arterial hypertension. Of people that have PAH, about 15%-20% have this heritable type.

Any of these can lead to pulmonary arterial hypertension:

Who else gets pulmonary arterial hypertension?

It’s most common in women who are between 30 and 60 years old. People of African and Hispanic descent are more likely to be affected.

You may not notice any symptoms for a while. The main one is shortness of breath when you’re active. It usually starts slowly and gets worse as time goes on. You may notice that you can’t do some of the things you used to without feeling tired and out of breath.

Other symptoms include:

  • Chest pain
  • Fatigue
  • Your heart pounding or racing (heart palpitations)
  • Feeling dizzy
  • Passing out
  • Swelling in your feet, ankles, legs, neck, or belly
  • Blue or gray skin (depending on your skin tone)

If you have shortness of breath and see your doctor, they’ll ask you about your medical history. They may also ask you:

  • Do you smoke?
  • Does anyone in your family have heart or lung disease?
  • When did your symptoms start?
  • What makes your symptoms better or worse?
  • Do your symptoms ever go away?

Your doctor may order tests, including:

  • Echocardiogram , an ultrasound picture of your beating heart that can check blood pressure in your pulmonary arteries
  • CT scan, which can show enlarged pulmonary arteries or other problems in the lungs that could cause shortness of breath
  • Ventilation-perfusion scan (V/Q scan), which can help find blood clots that can cause high blood pressure in your lungs
  • EKG, which traces your heart’s activity and can show whether the right side of your heart is under strain, a warning sign of pulmonary hypertension
  • Chest X-ray, which can show if your arteries or heart are enlarged or help find other lung or heart conditions
  • Exercise testing, in which your doctor may want you to run on a treadmill or ride a stationary bike while you are hooked up to a monitor (this lets them see any changes in your oxygen levels, how your heart works, and lung pressure, among other things)
  • BNP test (B-type natriuretic peptide test), a type of blood test that can show signs of PAH by looking at how hard your heart is working
  • Breathing tests (lung function tests), which check for conditions such as chronic obstructive pulmonary disease (COPD) or asthma

Your doctor may also do blood tests to check for HIV and conditions such as scleroderma or lupus.

If these tests show that you might have pulmonary hypertension, your doctor will need to do a right heart catheterization to be sure. Here’s what happens during that test:

  1. Your doctor places a catheter into a large vein — most often the jugular vein in your neck or femoral vein in your leg. 
  2. Then, they thread it into the right side of your heart.
  3. A monitor records the pressure in the right side of your heart and in the pulmonary arteries.

You doctor may also inject medicines into the catheter to see if the pulmonary arteries are stiff. This is called a vasoreactivity test.

Right heart catheterization is safe. Your doctor will give you a sedative and use local anesthesia. You can usually go home the same day, although you’ll need someone to drive you home.

You may want to write down a list of questions before your appointment. That way, you can make sure you ask your doctor everything you want to. It can also help to have a friend or family member with you to help you take notes. 

Some possible questions are:

  • What’s the best treatment for me?
  • How often should I see a doctor for my condition?
  • Do I need to see a specialist?
  • What are signs that I need to go to the emergency room?
  • Do I need to limit the salt or fluids in my diet?
  • What kind of exercise is safe to do?
  • Are there any activities I should stay away from?
  • Should I get a pneumonia vaccine, a flu shot , or a COVID-19 vaccine?

Pulmonary arterial hypertension treatment differs from person to person, so your treatment plan will be specific to your needs. Ask your doctor what your options are and what to expect. PAH is rare, so your doctor may refer you to someone who specializes in pulmonary hypertension.

There are many types of medications for pulmonary arterial hypertension. Some you take by mouth, others you breathe in, and some you get through an IV. The goal of these medications is to balance the substances your lungs make. These include endothelin, nitric oxide, and prostacyclin. PAH medications can also help your lungs work better and make sure blood can move easier through your veins to you lungs.

The types of medications you may take for pulmonary arterial hypertension include:

  • Biologics to help stop your blood vessel walls from getting thicker
  • Combination medications that work together to help your blood vessels stay wide
  • Endothelin receptor antagonists (ERAs) to help your heart pump blood better and stop your blood vessels from narrowing, such as ambrisentan, bosentan, and macitentan
  • Nitric oxides to help improve blood flow, including phosphodiesterase inhibitors (PDE5 inhibitors) such as sildenafil and tadalafil
  • Prostaglandins to help your blood vessels relax and widen, including prostacyclin analogues, prostacyclin mimetics, and selective IP receptor agonists

Other pulmonary arterial hypertension treatments

Your doctor may also recommend other treatments for pulmonary arterial hypertension. These work alongside treatments such as medication to manage your symptoms. These include:

Pulmonary rehabilitation. This includes education about your condition so you can have the skills you need to manage your symptoms. You may learn how to safely be more active without feeling tired and out of breath. It can also boost your overall level of fitness. Pulmonary rehab is often a group setting, so you can meet other people who have PAH or similar conditions.

Other medications. Your doctor may suggest other kinds of medications, too. These include blood thinners to avoid clots and water pills (diuretics) to get rid of extra fluid. Oxygen therapy can help your body get the oxygen it needs.

Surgery. In some cases, your doctor may mention surgery, such as a lung or heart transplant. Other procedures, such as an atrial septostomy, can ease pressure on your heart.

One of the best things you can do for yourself is to stay active, even if you have shortness of breath. Regular exercise, such as taking a walk, will help you breathe better and live better. Talk to your doctor first to find out what kind of exercise is best for you and how much you should do. Some people may need to use oxygen when they exercise.

Get plenty of rest, too. Pulmonary arterial hypertension makes you tired, so get a good night’s sleep, and take naps when you need to.

Just like anyone else, it’s good for you to eat a nutritious diet with lots of fruits, vegetables, and whole grains. Limit how much salt you take, and try to avoid saturated fat and trans fat. That’s important for your overall health.

Avoid things that put too much strain on your heart, including:

  • Weightlifting 
  • Traveling to places in high altitudes 
  • Spending time in saunas or hot tubs

The Pulmonary Hypertension Association offers in-depth information on everything from medications to tips on making daily tasks easier. It also has an active online support community.

Pulmonary arterial hypertension is a condition that gets worse over time. But treatments can help your symptoms so you can live better. It may take some planning, but many people who have it find ways to do the things they love, just as they did before their diagnosis.

Remember that each person is different, and there are good treatments available. Work with your doctor to find what’s right for you.

Can pulmonary arterial hypertension be reversed?

No, pulmonary arterial hypertension can’t be reversed. There’s no cure for pulmonary arterial hypertension, but the earlier you get a diagnosis, the easier it is to live with.

What’s the life expectancy of someone with pulmonary arterial hypertension?

How long you can live with pulmonary arterial hypertension depends on many factors. On treatment, the average median life expectancy of someone with PAH is about six years after diagnosis. 

What are some of the long-term complications of PAH?

Some of the long-term problems of pulmonary hypertension, including PAH, are:

  • Blood clots 
  • Bleeding in your lungs
  • Changes in your heartbeat (arrhythmias)
  • Enlargement of the right side of your heart (cor pulmonale)
  • Heart failure

Pulmonary hypertension can also affect pregnancy. It can be dangerous for you and the fetus.

Pulmonary arterial hypertension is a type of pulmonary hypertension that gets worse over time. With PAH, you have high blood pressure in the arteries that go from your heart to your lungs. You may have symptoms such as shortness of breath, swelling, and chest pain, among other signs. Even though there’s no cure, you can manage PAH symptoms with treatment, including medication and pulmonary rehab.

Here are some of the most commonly asked questions about pulmonary arterial hypertension.

How is PAH different from regular high blood pressure?

Regular high blood pressure happens throughout your body, whereas pulmonary arterial hypertension is high blood pressure in your lungs. With regular high blood pressure (hypertension), your body’s arteries narrow. With PAH, the arteries in your lungs narrow, which makes your heart work harder.

What causes idiopathic pulmonary arterial hypertension (IPAH)?

Sometimes, doctors can’t find a reason for high blood pressure in the lungs. In that case, the condition is called idiopathic pulmonary hypertension.

Is pulmonary arterial hypertension a disability?

Yes, pulmonary hypertension, including PAH, is considered a disability. Experts recommend applying for disability benefits as soon as possible because the approval process can take months.