What is PAH?

What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary arterial hypertension (PAH) is one specific type of PH. With PAH, the small arteries in the lungs become narrowed, thickened, or damaged, making it harder for blood to flow through the lungs. This causes the heart to work harder to pump blood through the lungs.

PAH can occur on its own, without another known medical condition. It can also develop in connection with other diseases or conditions, including certain connective tissue diseases, congenital heart conditions, liver disease, and some infections or drug exposures.

PAH can be difficult to diagnose because its early symptoms such as shortness of breath, fatigue, dizziness, and reduced ability to exercise can look like symptoms of more common conditions.

PAH is a serious and complex disease. Without appropriate treatment, it can worsen over time. However, treatments are available to help manage PAH, improve symptoms and quality of life, and slow the progression of the disease.

Early diagnosis and treatment can make a difference.

Pulmonary Hypertension (PH) vs Pulmonary Arterial Hypertension (PAH)

Pulmonary hypertension (PH) is a general term for high blood pressure in the blood vessels of the lungs. PH is not one single disease. There are several different types of PH, and they can have different causes and require different treatments. Think of PH as the overall name for a group of conditions. PAH is is one specific type of PH.

Why the Difference Matters

The symptoms of PH and PAH can be very similar. Shortness of breath, fatigue, dizziness, chest discomfort, and reduced ability to exercise are common symptoms. Because these symptoms can also occur with many more common conditions, PH and PAH can be difficult to recognize and diagnose.

Knowing which type of PH a person has is important because treatment depends on the cause. PAH has specific treatments that target the blood vessels in the lungs, while other forms of PH are generally treated by addressing the underlying condition.

PAH is a serious and complex disease that can worsen over time without appropriate treatment. While there is currently no cure for most forms of PAH, treatments can help manage the disease, improve symptoms and quality of life, and slow its progression.

Pulmonary Arterial Hypertension Symptoms

The symptoms of pulmonary arterial hypertension (PAH) can develop gradually and may be easy to overlook at first. Many people initially notice symptoms during physical activity, but symptoms can become more noticeable as PAH progresses.

Symptoms include:

Shortness of breath: Feeling out of breath, especially during physical activity.

Chest pain or pressure: Discomfort or pressure in the chest, particularly with activity.

Heart palpitations: Feeling like your heart is racing, pounding, fluttering, or skipping a beat.

Fatigue: Feeling unusually tired, weak, or lacking energy.

Swelling: Fluid buildup can cause swelling in the feet, ankles, legs, or abdomen.

Dizziness or fainting: Feeling lightheaded or losing consciousness, especially during physical activity.

These symptoms can have many possible causes and do not necessarily mean you have PAH. If you experience persistent or unexplained symptoms, talk with your healthcare provider. Early recognition and diagnosis can help you receive appropriate care sooner.

Pulmonary Arterial Hypertension Treatments

There are several treatments available for pulmonary arterial hypertension (PAH). Your healthcare team will choose a treatment plan based on your type and severity of PAH, your symptoms, and how your body responds to treatment. Some people may need more than one type of medication.

PAH treatments may include:

Oral medications: Medicines that you take by mouth, usually as a pill or tablet.

Inhaled medications: Medicines that you breathe directly into your lungs using an inhaler or other device.

Intravenous (IV) medications: Medicines delivered directly into a vein through a small catheter or tube. Some IV treatments may need to be given continuously using a portable pump.

Subcutaneous medications: Medicines delivered through a small needle placed just under the skin. These medications may also be given continuously using a portable pump.

Diuretics: Often called “water pills,” these medicines help your body get rid of extra fluid. They can help reduce swelling and may decrease some of the workload on your heart.

Treatment Is Individual

PAH affects people differently, so there is no single treatment that works for everyone. Your healthcare team may adjust your medications over time based on your symptoms, test results, and how well you are responding to treatment.

In addition to medication, your care team may recommend other treatments or lifestyle changes to help manage PAH and support your overall health.

Pulmonary Arterial Hypertension Risk Factors

Pulmonary arterial hypertension (PAH) can affect anyone, and in some people, the exact cause is unknown. However, certain health conditions, genetic factors, and exposures can increase the risk of developing PAH.

Risk factors and conditions associated with PAH include:

Connective tissue diseases: Conditions such as scleroderma, lupus, and rheumatoid arthritis can be associated with PAH.

Liver disease: Severe liver disease, including cirrhosis, can increase the risk of developing PAH.

HIV infection: People living with HIV have a higher risk of developing PAH.

Certain drugs and substances: Exposure to substances such as methamphetamine and cocaine has been associated with an increased risk of PAH.

Congenital heart disease: Certain heart conditions that are present at birth can increase the risk of developing PAH.

Family history and inherited gene changes: In some people, PAH runs in families and is linked to inherited changes in certain genes. This is known as heritable PAH.

Having a risk factor does not mean that you will develop PAH. If you have one of these conditions or other risk factors and experience symptoms such as unexplained shortness of breath, fatigue, dizziness, or chest discomfort, talk with your healthcare provider.

PAH Treatment is Personal

There is no single treatment plan that works for everyone with pulmonary arterial hypertension (PAH). Your healthcare team will consider several factors when deciding which treatments are right for you, including:

You as an individual: Your overall health, other medical conditions, lifestyle, and personal needs.

Your symptoms: How severe your symptoms are and how they affect your daily activities.

Your test results: Results from heart, lung, blood, and other tests help your healthcare team understand how PAH is affecting you.

Your support at home: Your ability to manage medications and treatments at home, along with the support available from family, caregivers, and your healthcare team.

Your response to treatment: Your doctor may adjust your treatment over time based on how well it is working and how your condition changes.

More than one treatment: Many people with PAH need a combination of medications that work in different ways. Using more than one treatment may provide greater benefit than a single medication for some patients.

PAH Is Serious but Treatment Can Help

PAH is a serious, potentially life-threatening disease. Without appropriate treatment, PAH can worsen over time and may lead to heart failure and death. However, treatments are available that can help manage the disease, improve symptoms and quality of life, and slow its progression.

Working closely with a PAH specialist and following your treatment plan can play an important role in managing the disease.