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. In mild and moderate cases, they might prescribe one or more medications. For more severe cases, they might recommend surgery.

Although there is no cure for PAH, your doctor can help you manage symptoms, slow disease progression, and improve your quality of life with medications and procedures. PAH is a rare lung disease — the American Lung Association estimates that 500 to 1,000 new cases are diagnosed annually in the United States — so consider enlisting the help of a pulmonologist, a cardiologist, or another PAH specialist to ensure you receive the latest treatments.

“When I was diagnosed, I was in shock,” said one myPHteam member. “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.”

In this article, you’ll learn about three ways PAH severity impacts treatment. You can then bring your questions to your next appointment to discuss with your doctor.

1. Pulmonary Arterial Pressure Measurements

Pulmonary arterial hypertension is a form of pulmonary hypertension (PH), a type of high blood pressure that affects the arteries in the heart and lungs. Health care providers use several tests to measure blood pressure and the flow of blood within these arteries. These tests can help determine the severity of your PAH.

Doctors use an echocardiogram — a routine and noninvasive procedure — to estimate pulmonary arterial pressure or pulmonary blood pressure. In general, the higher your pulmonary arterial pressure, the harder your heart is working to pump blood from your heart to your lungs. Over time, this additional effort weakens your heart and can lead to heart failure. Pulmonary arterial pressure helps health care providers understand how advanced your PAH might be.

Health care professionals also use a test called right heart catheterization to get more information about the hemodynamics (blood flow) within your heart and lungs. Doctors use this information with other measurements to determine the severity of your PAH and what treatments may work best for you.

Two pressure measurements gathered 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

Doctors use the difference between mPAP and PAWP to understand how much pressure elevation in the lungs is due to abnormalities in the pulmonary arteries versus another issue, like left-sided heart disease. These measurements help them diagnose PAH and understand how severe it is.

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

Combination Therapy

If you’re newly diagnosed, your doctor will likely recommend combination therapy, which is now the standard of care for pulmonary arterial hypertension. Unlike monotherapy, which uses a single treatment or drug to treat PAH, combination therapy uses two or more medications to ease symptoms and lower blood pressure by widening the blood vessels.

Medications commonly used in combination therapy include oral, inhaled, and intravenous drugs such as:

• Phosphodiesterase-5 (PDE-5) inhibitors like sildenafil (Revatio) and tadalafil (Adcirca)
• Soluble guanylate cyclase (sGC) stimulators like riociguat (Adempas)
• Endothelin receptor antagonists (ERAs) like bosentan (Tracleer), ambrisentan (Letairis), and macitentan (Opsumit)
• Prostacyclin and prostanoid medications like treprostinil (Tyvaso, Orenitram), iloprost (Ventavis), epoprostenol (Flolan, Veletri), and selexipag (Uptravi)

You may also be a candidate for combination therapy if you’ve been taking a single drug to treat PAH and your doctor thinks it’s time to change or escalate your treatment.

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

2. Functional Class

Your health care provider will also consider the severity of your symptoms when deciding which pulmonary arterial hypertension treatment to recommend. The World Health Organization (WHO) has defined four functional classes to describe the severity of PAH based on symptoms:

• Class 1 — You experience no symptoms while exercising or resting.
• Class 2 — You experience no symptoms while resting but may feel uncomfortable or short of breath while grocery shopping, climbing stairs, or doing other daily activities.
• Class 3 — You may experience PAH symptoms while resting, and your daily activities are significantly limited by shortness of breath, fatigue, and possibly fainting.
• Class 4 — You experience symptoms while resting, and any physical activity likely leads to severe symptoms.

Supportive Therapies

If you regularly experience shortness of breath, lightheadedness, chest pain, fatigue, swelling in your feet and legs, and other symptoms that reduce your quality of life, your health care provider may recommend one or more supportive therapies in addition to combination therapy. These treatments 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 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 improves oxygen flow in the body
• Lung, heart, or heart-lung transplant (for most severe PAH cases only)

If you have multiple symptoms, tell your doctor which ones affect you the most. This will help them create a plan to address your most severe symptoms first and make you as comfortable as possible.

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

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

3. Disease Progression

Pulmonary arterial hypertension is a progressive disease, meaning it may worsen over time. However, you may experience periods of feeling better as your symptoms come and go. Tell your health care provider about your good and bad days so they can adjust your treatment plan if needed.

In addition to considering the feedback you provide, your doctor will likely monitor your heart health with regular echocardiograms and six-minute walk distance tests to measure your aerobic or exercise capacity. They may also recommend an annual right heart catheterization to assess your treatment response.

Knowing what and how you feel — and how you’re responding to therapy — can help your doctor adjust your treatment plan or medication dosage. The severity of your PAH symptoms and how they change or progress over time is important information for your doctor to consider when determining your treatment.

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

You may also be able to reduce the severity of your symptoms and maintain your health by making lifestyle changes, such as:

• Quitting cigarettes if you smoke
• Avoiding secondhand smoke
• Reducing or limiting sodium intake
• Following a heart-healthy diet by eating plenty of fruits and vegetables, controlling portion sizes, and limiting unhealthy fats
• Asking your doctor or PAH care team for help creating a safe exercise plan
• Avoiding activities that can strain your heart and lungs, like traveling to high-altitude locations or sitting in a hot tub

In addition, if you are considering pregnancy, talk with your doctor first.

There’s no one-size-fits-all treatment approach for PAH, and your protocol will likely change over time as your disease progresses. Your doctor may even recommend looking into clinical trials (studies that test how well new treatments work in people) to give you access to emerging therapies. Tell your doctor about any new or changing symptoms or side effects, be honest about how you are feeling, and if you have questions — ask.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 49,000 members come together to ask questions, give advice, and share their stories about life with PH and pulmonary arterial hypertension.

Do you have mild, moderate, or severe PAH? How has it affected your treatment? Share your thoughts in the comments below, or start a conversation with others on your Activities page.