Did you know that pulmonary arterial hypertension (PAH) affects not only your lungs, but your heart as well? People with this lung disease are at a higher risk of developing heart failure — especially right-sided heart failure (also called cor pulmonale).

In this article, we’ll discuss what heart failure is and how PAH can lead to it. We’ll also cover three tips for recognizing when your PAH has progressed to heart failure. With this information, you can seek treatment sooner and better manage your disease.

What Is Heart Failure in PAH?

Your heart muscle beats continuously, day and night, to carry oxygen and nutrients throughout your body. When this muscle becomes weak, it can no longer pump blood properly. Doctors diagnose heart failure when a person’s heart can’t meet their body’s needs.

The heart muscle has two sides — the left and the right. Each side contains an upper chamber (atrium) and a lower chamber (ventricle). Blood flows into your right atrium and then into the ventricle, where it’s pumped into the pulmonary arteries. People with PAH have high blood pressure in these arteries caused by thickening and narrowing of the arteries.

Once inside the lungs, blood is replenished with oxygen. This oxygen-rich blood then flows back toward your heart. The last stop is the left ventricle, which pumps the blood out to the rest of the body. Heart failure occurs when one of the chambers of your heart can’t pump blood well anymore.

How Does PAH Cause Heart Failure?

There are two main types of heart failure: left-sided heart failure and right-sided heart failure.

People with PAH may develop right-sided heart failure (right heart failure) as their disease progresses. Remember, blood flows from your right ventricle through the pulmonary arteries. In PAH, these arteries are narrowed or blocked, which stops blood from flowing through.

Think of trying to blow a pea through a straw. You’re trying to push the pea through a narrow tube by blowing on the straw as hard as you can. If you try too hard, you’ll get tired pretty quickly.

Your right ventricle is trying to do the same thing by pushing blood through a narrow or blocked artery. The heart muscle becomes weaker over time from all the extra effort. Eventually, you may develop right heart failure, also called right ventricular failure (RV failure).

Right heart failure may be the most common cause of death in people with PAH. Studies show that 44 percent to 73 percent of PAH deaths are due to right heart failure or cardiac arrest.

Symptoms of Heart Failure With PAH

Heart failure limits the amount of blood your heart can pump in one minute — known as your cardiac output. When your heart can’t move blood around your body, your blood begins pooling in different areas.

Symptoms of right heart failure develop due to fluid buildup and can include:

• Edema (swelling in the legs, feet, and ankles)
• Ascites (fluid buildup in the abdomen), which may cause bloating and nausea
• Shortness of breath and coughing
• Palpitations (rapid or pounding heartbeat)
• Chest discomfort or pain

Some of your PAH symptoms may also overlap with heart failure symptoms. One myPHteam member shared their experience, “I’ve overworked and stressed my heart to the point of right-sided heart failure. I had chest pain and dizziness, most likely because of heart failure. I also had symptoms of PAH, such as shortness of breath, fast heart rate, and high blood pressure.”

Diagnosing and Treating Heart Failure With PAH

If your doctor thinks you may have heart failure, they’ll run tests to check your heart function. Examples include:

• Echocardiogram — An ultrasound that looks at blood flow through the heart
• Electrocardiogram (ECG or EKG) — A test that monitors your heart’s electrical activity
• Right heart catheterization — A test that measures blood pressure and cardiac output
• Six-minute walk test — A test that measures your exercise capacity (how far you can walk in six minutes)
• Blood tests — Checks for high levels of B-type natriuretic peptides (BNP) as a sign of heart failure

Treating Right Heart Failure With PAH

Doctors typically treat right heart failure by treating its underlying cause. If PAH is the underlying cause, then doctors will focus on treating your PAH. Some PAH medications are also useful for treating right heart failure.

Diuretics help remove extra fluid from the body to reduce swelling and take pressure off the heart muscle. Other PAH medications relax and widen the blood vessels. This helps lower the blood pressure in your pulmonary arteries. PAH treatment usually involves a combination of:

• Endothelin receptor antagonists, like bosentan (Tracleer) and macitentan (Opsumit)
• Phosphodiesterase-5 (PDE5) inhibitors like sildenafil (Revatio) and tadalafil (Adcirca)
• Prostacyclin analogs, like selexipag (Uptravi) and treprostinil (Remodulin, Tyvaso)

Read more about specific medications in this list of treatments for PH.

For people with right-sided heart failure caused by something else, such as chronic obstructive pulmonary disease (COPD), doctors will focus on treating the lung disease with medications and supplemental oxygen.

Recognizing Disease Progression to Heart Failure With PAH

Some myPHteam members have reached out to others about watching their PAH symptoms. “How do you know if you’re having a PAH flare or possibly congestive heart failure? … My calves are really swollen today.”

Below, we’ll cover three tips for recognizing PAH progression and signs of heart failure. We’ll also let you know when it’s a good idea to reach out to your doctor or get emergency medical treatment.

1. Check for New Signs of Swelling

When living with PAH, it’s a good idea to regularly check your body for new swelling. Take a close look at your feet, ankles, legs, and arms. If you notice mild swelling, you should reach out to your doctor to check in. They’ll continue monitoring you and change your medications as needed.

More severe swelling with pain or discomfort is a sign that you may be progressing to heart failure. Other signs include abdominal pain due to ascites and loss of appetite. These are all signs that you need urgent medical attention, especially if you have other heart failure symptoms. The American Heart Association has made a handy guide to reference.

2. Monitor Your Weight

Fluid buildup due to right-sided heart failure can cause weight gain. You’ll want to weigh yourself regularly to make sure you keep a consistent weight.

If you experience a sudden weight gain of 2 to 3 pounds within 24 hours or 5 pounds in one week, let your doctor know. This may be a sign that your heart failure is progressing. Weight gain is just one of many symptoms used to track heart failure progression. Keep an eye out for other signs of worsening, like coughing, shortness of breath, and new swelling.

3. Take Note of When You Become Short of Breath

Some shortness of breath with PAH is normal, especially if you’re up and moving. If you notice you’re becoming more short of breath over time with activity, check in with your doctor.

Some myPHteam members have taken action after experiencing shortness of breath. One shared, “I’m still having slight chest pains and shortness of breath. My pulmonary specialist feels my PAH is advancing so I’m just waiting on a right-side heart catheter.”

If you can’t catch your breath while you’re sitting at rest, you likely need medical attention. This is a serious sign of worsening PAH and heart failure. Some people also experience shortness of breath while lying down due to fluid buildup. This can become so uncomfortable that it’s hard to get a good night’s sleep. If you’re having trouble sleeping at night, it’s time to talk to your doctor.

Find Your Team

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 54,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Are you living with pulmonary arterial hypertension and heart failure? What signs did you notice that prompted you to get medical treatment? Share your experience in the comments below, or start a conversation on your Activities page.