GoodRx Guide
Pulmonary Arterial Hypertension
Comprehensive information for you or a loved one — including treatment options and discounts on popular medications.
What is pulmonary arterial hypertension?
Pulmonary arterial hypertension (PAH) is a rare and serious medical condition that affects about 1% of people worldwide. When you have PAH, the small arteries in your lungs become narrow or stiff, making it harder for your blood to flow through your lungs. This causes pressure to build up in the lungs and forces your heart to work harder. Over time, that increased strain on the heart can lead to heart failure and cause symptoms like shortness of breath, chest pain, and lightheadedness.
Unlike many conditions that affect the lungs, like asthma or chronic obstructive pulmonary disease (COPD), PAH isn’t caused by smoking. And even though it has hypertension in the name, pulmonary arterial hypertension isn’t the same as regular high blood pressure.
There are several types of PAH, including idiopathic (no known cause), heritable (genetic), and PAH that’s linked to other conditions like connective tissue diseases or HIV.
Causes
There are several different causes of PAH, but many cases of PAH are termed “idiopathic.” This means that while doctors can diagnose you with it, they can’t tell exactly what caused it.
Where a cause is identified, most are out of your control. Put another way, PAH isn’t caused by lifestyle alone: Most people with it never smoked and lived otherwise healthy lives.
PAH can be caused by:
Congenital heart disease (heart disease you are born with)
Autoimmune diseases, like scleroderma or lupus
Medications, such as chemotherapy medications like bleomycin and etoposide, appetite suppressants like aminorex and benfluorex, and immunosuppressants like leflunomide and cyclosporin.
PAH is more common in women than men, and it’s typically diagnosed between ages 30 and 60.
Symptoms
The early symptoms of PAH are often easy to miss. They can be easily mistaken for signs of aging, stress, or being out of shape.
As symptoms progress, they become more noticeable. For example:
Feeling short of breath with activity and, eventually, at rest
Fatigue
Dizziness
Chest pain
Fainting
Swelling in the legs or ankles
A bluish hue to the lips or fingertips
It’s worth noting that the symptoms you feel may show up before the signs (what medical professionals can measure). So if something feels off, speak up to your care team so that they can look into it further.
Stages
Healthcare professionals use the WHO Functional Class system to stage PAH. This classification system isn’t based on anything that can be measured, but describes how PAH makes you feel and how it affects your day-to-day life. There are four groups:
Class I: You have a diagnosis of PAH, but have no symptoms with normal activity. You can do everything you normally do without feeling limited.
Class II: You have mild symptoms with daily activity. Walking up stairs might feel tougher than it used to be, for example, but you’re fine at rest.
Class III: Symptoms like shortness of breath or fatigue kick in with even light activity, like getting dressed or walking across a room. Rest feels OK, but activity is hard.
Class IV: Symptoms are there even when you’re resting. Any effort makes them worse. And you have signs of heart failure, like swelling or fainting.
Diagnosis
Diagnosing PAH takes time. PAH isn’t a condition that can be diagnosed right away on your first visit to the doctor. Most people start with vague symptoms and are seen by their primary care provider first.
A common sign of PAH that medical professionals look for is low oxygen levels. But you’ll probably also get sent for tests like blood work, chest X-rays, lung function tests, and an echocardiogram (a scan of the heart).
From there, if your primary care provider suspects PAH, they’ll refer you to a cardiologist (heart specialist) or pulmonologist (lung specialist). The only way to confirm PAH is with a test called a right heart catheterization — this measures the pressure inside your heart and lungs directly.
Diagnosis can take weeks or months. If you suspect PAH, you can ask to be evaluated at a pulmonary hypertension center.
Medications
There’s no cure for PAH yet, but medications can relieve your symptoms and slow their progression. Prescription options include:
Endothelin receptor antagonists (to relax the arteries in your lungs)
Phosphodiesterase-5 inhibitors (these also open up the arteries in your lungs)
Prostacyclin analogs (to improve blood flow in your lungs)
Vasodilators (which open blood vessels throughout your body)
Blood thinners (to prevent blood clots)
Digoxin (to help your heart beat regularly)
Water pills (to remove extra fluid from your body)
You might need a combination of these medications tailored to your needs. There are no approved over-the-counter (OTC) treatments for PAH, and some OTC medications — like decongestants — can actually make it worse. Always check with your care team before starting new medications.
Other treatments
Nonmedication options can play a big role in treating PAH:
Oxygen therapy: If your blood oxygen levels are low, your care team may prescribe oxygen therapy, which delivers oxygen through a tube that sits right under your nose.
Pulmonary rehabilitation: This is a special type of exercise and education program that can improve stamina and symptoms.
Lung or heart-lung transplants: Transplants are reserved for very severe cases but can be an option when all other treatments have failed.
Lifestyle tweaks like avoiding high altitudes, staying active within your limits, and eating a heart-healthy, low-sodium diet can also help. Complementary treatments like yoga or meditation may support well-being, but they aren’t substitutes for medical therapy.
Living with
Living with PAH means managing a long-term condition, but it doesn’t have to define you. With the right care team and treatment plan, many people live full and fulfilling lives. Regular check-ins with your medical team, keeping up with your treatment plan, and knowing your limits are key. Mental health matters, too. Support groups, advocacy organizations, and therapy can help you feel less alone.
Cost can be a barrier, especially for specialty medications. But there are ways to save. GoodRx can help you compare prices and find discounts at local pharmacies. You can also ask a healthcare professional or pharmacist about options like patient assistance programs, manufacturer discount coupons, and insurance prior authorizations that can significantly lower your out-of-pocket costs. Don’t hesitate to speak up — lots of programs exist to help patients afford their medications.
Frequently asked questions
Pulmonary arterial hypertension (PAH) used to be considered a fatal condition with a short life expectancy, but that’s no longer the case. Thanks to earlier diagnosis and newer treatments, many people are living longer and with a better quality of life. That said, PAH is still a serious, progressive condition, and outcomes vary widely. Regular follow-ups with your medical team and a personalized treatment plan are key to improving your outlook.
No, this is a common misconception. Pulmonary arterial hypertension (PAH) is high blood pressure in just the arteries that supply your lungs, not the general blood pressure in your body. You can measure regular high blood pressure easily with a cuff that goes on your arm, but PAH has to be measured with a special procedure called a cardiac catheterization. Treatments and risks are also different, so it’s important not to confuse the two.
Yes, but with caution. Many people with PAH benefit from light to moderate physical activity — especially under medical supervision, like in a pulmonary rehab program. Exercise can actually improve your endurance and quality of life. But overdoing it can be risky. Always talk with your doctor or other healthcare professional before starting or changing your routine.
Right now, there’s no cure for PAH. But it can be managed. Current treatments focus on easing your symptoms, slowing disease progression, and improving your quality of life. In rare cases, a lung or heart-lung transplant may be considered. Ongoing research continues to bring hope for better long-term options.
References
American Lung Association. (n.d.). Pulmonary hypertension.
American Lung Association. (2025). PAH medication & treatment guide.
American Lung Association. (2025). Treating and managing PAH.
Frost, A. E., et al. (2023). Smoking history and pulmonary arterial hypertension: Demographics, onset, and outcomes. The Journal of Heart and Lung Transplantation.
Hlavaty, A., et al. (2022). Identifying new drugs associated with pulmonary arterial hypertension: A WHO pharmacovigilance database disproportionality analysis. British Journal of Clinical Pharmacology.
Ji, W., et al. (2013). Spironolactone attenuates bleomycin-induced pulmonary injury partially via modulating mononuclear phagocyte phenotype switching in circulating and alveolar compartments. PLoS One.
Machado, R. D., et al. (2009). Genetics and genomics of pulmonary arterial hypertension. Journal of the American College of Cardiology.
Manek, G., et al. (2024). Pulmonary hypertension. StatPearls.
Memon, H. A., et al. (2017). Pulmonary arterial hypertension in women. Methodist DeBakey Cardiovascular Journal.
Montani, D., et al. (2013). Drug-induced pulmonary arterial hypertension: A recent outbreak. European Respiratory Review.
National Heart, Lung, and Blood Institute. (2022). Pulmonary hypertension: Symptoms. National Institutes of Health.
National Heart, Lung, and Blood Institute. (2023). What is pulmonary hypertension? National Institutes of Health.
PHA Europe. (2020). Classification and WHO functional class.
Simonneau, G., et al. (2019). Haemodynamic definitions and updated clinical classification of pulmonary hypertension. European Respiratory Journal.
