Pulmonary arterial hypertension (PAH) is one form of pulmonary hypertension (high blood pressure in your pulmonary arteries). Symptoms may not appear until the condition has gotten worse. Your provider will run many tests to diagnose you and plan your treatment. PAH medications can improve your quality of life and help you live longer.
Pulmonary arterial hypertension (PAH) is a condition in which the small blood vessels in your lungs become narrow. As a result, blood can’t flow through your lungs as well as it should. This leads to high blood pressure in your pulmonary arteries. These are the arteries that carry oxygen-poor blood from your heart to your lungs.
Eventually, your heart gets weaker because it has to work harder to pump blood to your lungs. Without treatment, PAH can cause a cascade of problems in your heart, lungs and the rest of your body.
PAH is one form of pulmonary hypertension. This is a general diagnosis for high blood pressure in your pulmonary arteries from any cause.
Pulmonary arterial hypertension strains the right side of your heart, which pumps oxygen-poor blood to your lungs. This strain can lead to right-sided heart failure.
Plus, PAH slows down blood flow between your heart and lungs. This means less blood can enter your lungs to gain fresh oxygen. As a result, blood flow to the rest of your body also slows down. So, your organs and tissues can’t get enough oxygen.
Without treatment, PAH can be fatal.
Pulmonary arterial hypertension is a serious condition that can be life-threatening. An early diagnosis and swift treatment can help you live longer and have a better quality of life.
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Pulmonary arterial hypertension can affect adults at any age. It’s more common among women and people designated female at birth, who are usually diagnosed between age 30 and 60. Men over age 65 who develop PAH are more likely to have severe cases.
PAH can also affect infants. This condition is known as persistent pulmonary hypertension in the neonate (PPHN).
PAH isn’t as common as other forms of pulmonary hypertension, including those caused by underlying heart or lung disease. Each year, about 500 to 1,000 people are diagnosed with PAH in the U.S.
In Western countries, about 25 per 1 million people are living with PAH.
You may not feel any symptoms early on. Most people with PAH begin to notice symptoms as the condition progresses. These symptoms can include:
Without treatment, PAH symptoms continue to get worse over time. You may find it harder to do your usual activities without needing to catch your breath or rest.
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Damage to the lining of the blood vessels in your lungs leads to pulmonary arterial hypertension. It’s not always clear what causes this damage. When there’s no clear cause, you have what’s called “idiopathic” pulmonary arterial hypertension.
Other times, there is a clear cause. Known causes of PAH include associated medical conditions, genetic mutations and certain drugs.
Medical conditions that may lead to the development of PAH include:
Genetic mutations can also cause pulmonary arterial hypertension. Here’s what we know about genetics and PAH:
Certain drugs can cause PAH, including:
PAH can be hard to diagnose because its symptoms could be caused by many other conditions. Your provider will perform a physical exam and talk with you about your symptoms and medical history. You’ll likely need many tests (including imaging tests and blood tests) to find out if you have pulmonary hypertension and if so, which form.
Your provider may also refer you to a pulmonologist or cardiologist. These specialists will run specific tests to check your heart and lung function. They’ll determine what form of pulmonary hypertension you have (PAH or another form). They’ll also evaluate how far your condition has progressed.
PAH requires specific types of treatment that can’t be used for other forms of pulmonary hypertension. So, your care team must learn as much as possible about what’s happening in your lungs and your heart. This information helps them tailor treatment to your specific diagnosis and needs.
Your care team will use a combination of different tests to rule out other conditions and diagnose PAH.
If your provider suspects you have PAH based on your physical exam, the first test they’ll order for you is a transthoracic echocardiogram. This test evaluates the overall structure and function of your heart.
Other tests may include:
Talk with your provider about the tests you need and how to prepare for them.
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Providers diagnose pulmonary hypertension based on the blood pressure in your pulmonary arteries. The criteria for diagnosis is pulmonary arterial pressure higher than 20 mmHg while you’re at rest. A right heart catheterization measures this number.
Treatment for PAH focuses on slowing down its progression and giving you a better quality of life. There’s no one-size-fits-all approach to PAH treatment. Your provider will work with you to decide the best treatment for your specific needs.
Your individualized treatment plan for PAH may include:
A last resort option for some people with severe PAH is a lung transplant. This surgery may give you one or two new lungs. A heart-lung transplant gives you a new heart as well.
Medications that treat PAH come in several different forms:
The U.S. Food and Drug Administration (FDA) has approved the following drugs for people with PAH:
Common side effects of drugs that treat PAH include:
Specific drugs may have additional side effects. Talk with your provider about how to manage any side effects. In some cases, your provider may need to adjust your dosage.
Currently, medications can slow down PAH progression but not reverse the damage already done. However, researchers are working on promising new medications that could help reverse PAH. Such medications would repair damage to the endothelial cells that line your pulmonary arteries.
Talk with your provider to learn more about the latest research and clinical trials for PAH therapies.
Risk factors for developing PAH include:
If someone in your biological family has PAH, ask your provider about genetic testing. If you’re diagnosed with PAH, you may want to tell your family members so they can consider genetic testing.
Some risk factors for PAH (like genetic mutations) are beyond your control. However, avoiding street drugs can help lower your risk of PAH and many other health problems. Also, talk with your provider before taking any diet pills.
Depending on your risk factors, your provider may recommend preventive screenings for PAH.
Thanks to advances in treatment, people with PAH can live longer than ever before. Your life expectancy depends on many factors, including the severity of your condition and how early you’re diagnosed. Talk with your provider to learn your specific prognosis.
It’s important to keep up with your treatment and also closely follow your provider’s guidance. Steps you can take to improve your outlook include:
Follow your provider’s guidance for any lifestyle changes you should make. In general, some tips include:
Call your provider if you’re having problems with:
Go to the emergency department or call your local emergency number if you have:
A note from Cleveland Clinic
Pulmonary arterial hypertension is a life-changing diagnosis. If you or a loved one are newly diagnosed, take time to learn about the condition and what you can expect. Ask your provider for resources that explain how PAH works and how treatment can help.
One of the most important resources you can have is the support of other people in your life. Don’t try to manage your condition alone. If your family and friends don’t live close by, ask your provider about support groups and community resources.
If your loved one was diagnosed, do whatever you can to make them feel supported, and help them connect with others. Illnesses isolate us in some ways, but they can also bring us closer together. Use this time to form a community that can help you or your loved one navigate the path forward.
Last reviewed on 07/22/2022.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy