Pulmonary arterial hypertension (PAH) can be mild, moderate, or severe, and the treatment your doctor recommends will depend in part on the severity of your condition. If you have mild or moderate PAH, your doctor may start you on one or more medications. If your PAH is more severe, treatment may involve a combination of medications and additional therapies.

Although there's no cure for PAH, treatment can help manage symptoms, slow disease progression, and improve your quality of life. PAH is a rare lung disease — the American Lung Association estimates that each year, 500 to 1,000 people in the United States are newly diagnosed with PAH. Working with a pulmonologist, a cardiologist, or another PAH specialist can help ensure you’re getting the most up-to-date care.

“When I was diagnosed, I was in shock,” a myPHteam member said. “As I learned more about PAH, the treatments, and mortality rates, it became less frightening. Having a PAH specialist has made a difference, too. She assured me that my condition was diagnosed early and that with the right combination of medications, my prognosis was favorable.”

This article explores three ways the severity of PAH affects treatment options. Be sure to bring any questions you may have to your next appointment — your doctor can help you understand what your treatment plan means for you.

1. Pulmonary Arterial Pressure Measurements

Pulmonary arterial hypertension is a type of pulmonary hypertension (PH), a condition that causes high blood pressure in the arteries between the heart and lungs. Healthcare providers use different tests to measure how hard the heart is working and how well blood is flowing through these arteries. The results can help your doctor understand how severe your PAH is.

An echocardiogram — a routine, noninvasive procedure — is used to estimate pulmonary arterial pressure (or pulmonary blood pressure). In general, the higher your pulmonary arterial pressure, the harder your right heart is working to move blood into your lungs. Over time, this extra effort weakens the heart and may lead to right heart failure (when the heart isn’t pumping as well as it should). The pressure measurement gives your care team clues about how advanced your PAH might be.​

Your doctor may also recommend a right heart catheterization, which offers a more detailed picture of the hemodynamics (blood flow) within your heart and lungs. Doctors use this information with other measurements to determine the severity of PAH and what treatments may work best.

Two important readings from a right heart catheterization are:

• Mean pulmonary arterial pressure (mPAP) — Measures pressure in the pulmonary artery
• Pulmonary capillary wedge pressure (PAWP) — Estimates pressure in the heart’s left atrium

By comparing these two numbers, your doctor can tell whether high pressure in the lungs is due to narrowed pulmonary arteries (suggesting PAH) or from another cause, like heart failure on the left side of the heart. A high mPAP with a normal or low PAWP points to PAH. If both are elevated, left-sided heart disease may be involved.

“I have PAH and am doing pretty well,” a myPHteam member shared. “It’s considered mild.” Another member said, “I have PAH also, but with medication, I am doing much better.”

Combination Therapy

If you’re newly diagnosed with PAH, your doctor will likely recommend combination therapy, which is now the standard of care. Unlike monotherapy (relying on just one medication), combination therapy uses two or more drugs to ease symptoms and lower blood pressure by widening the blood vessels.

Combination therapy may include oral, inhaled, or IV medications, such as:

• Phosphodiesterase 5 (PDE5) inhibitors
• Soluble guanylate cyclase (sGC) stimulators
• Endothelin receptor antagonists
• Prostacyclin or prostanoid medications
• Activin signaling inhibitors

Your doctor might also suggest combination therapy if you’ve been taking a single drug to treat PAH but now need a different approach.

“I am preparing to be admitted into the hospital for a subcutaneous therapy treatment,” a myPHteam member said. “I’m very excited to get my PAH under control and start feeling better.”

2. Functional Class

When deciding which pulmonary arterial hypertension treatment to recommend, your healthcare provider will consider how much PAH affects your daily life. The World Health Organization (WHO) has defined four functional classes that describe the severity of PAH symptoms:

• Class 1 — You don’t have symptoms during physical activity or rest.
• Class 2 — You don’t have symptoms while resting, but you may feel uncomfortable or short of breath during activities like grocery shopping, climbing stairs, or doing chores.
• Class 3 — You may have PAH symptoms during light activity, and your daily activities are significantly limited by shortness of breath, fatigue, and possibly fainting.
• Class 4 — You have symptoms at rest, and any activity likely causes severe symptoms.

Supportive Therapies

If you often experience shortness of breath, lightheadedness, chest pain, fatigue, swollen feet and legs, and other symptoms that make daily life harder, your healthcare provider may recommend adding supportive treatments to combination therapy. Common supportive therapies include:

• Pulmonary vasodilators, which help open the pulmonary arteries
• Calcium channel blockers, which help lower blood pressure in the pulmonary arteries
• Diuretics, which help reduce extra body fluid and edema (swelling)
• Anticoagulants to help prevent blood clots
• Oxygen therapy to make breathing easier
• Pulmonary rehabilitation to help improve breathing
• Balloon atrial septostomy, a procedure that helps relieve pressure in the right side of the heart and may improve oxygen flow in the body
• Lung, heart, or heart-lung transplant, an option for the most advanced PAH

If you have several symptoms, tell your doctor which ones affect you the most. This helps them focus on what matters most to you and build a treatment plan that supports your quality of life.

“I go to a pulmonary rehabilitation facility twice a week to work out and am trying to keep my PAH and lungs stable.”
— A myPHteam member

“I go to a pulmonary rehabilitation facility twice a week to work out and am trying to keep my PAH and lungs stable,” a myPHteam member said.

“When I was diagnosed with PAH in 2007, I was told I would live maybe five to 10 years and not past 60, but I’m 65 now,” another member shared. “At 60, I went on oxygen. That scared me, but I feel so much better.”

3. PAH Disease Progression

Pulmonary arterial hypertension is a progressive disease, meaning it may get worse over time. However, symptoms may come and go, and you might have some good days mixed in with harder ones. Keep your healthcare provider updated about how you’re feeling so they can adjust your treatment plan if needed.

Pulmonary arterial hypertension is a progressive disease, meaning it may get worse over time.

To track your health, your doctor will monitor your heart health through regular tests, including:

• Echocardiograms to look at how well your heart is working
• Six-minute walk tests to see how far you can walk and determine your exercise capacity (how much exercise you can do)
• Right heart catheterization, usually done once a year, to assess whether your treatment is managing blood flow and pressure

Your healthcare provider will combine these test results with your WHO functional class to decide whether your treatment plan needs to change.

One myPHteam member shared, “I had so many tests done while they were trying to diagnose me.” Although all these tests feel like a lot, the results help guide your care and ensure you’re getting the most effective treatment possible.

Communicate Honestly With Your Healthcare Provider

Knowing what and how you feel, as well as how you’re responding to therapy, can help your doctor adjust your treatment plan or medication dosage. The severity of your symptoms and how they change or progress over time is important information for your doctor to consider when deciding on treatment of pulmonary arterial hypertension.

“I was diagnosed with PAH two years ago,” a myPHteam member said. “At first, I had a portable pump that I had to carry around and a PICC line in my upper right chest. My doctor later removed that and put me on an oral medication. … My doctor told me at my follow-up that everything looked good.” (PICC stands for peripherally inserted central catheter.)

There’s no one-size-fits-all treatment approach for PAH, and your protocol will likely change as your disease progresses. Your doctor may even suggest clinical trials (studies that test new treatments in people). These trials can help you try newer treatments sooner. Tell your doctor about any new or changing symptoms or side effects, and be honest about how you’re feeling. Don’t hesitate to bring up any questions.

Join the Conversation

On myPHteam, people share their experiences with pulmonary hypertension, get advice, and find support from others who understand.

How has your PAH treatment changed as it has become more severe? Let others know in the comments below.