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Shortness of breath is one of the most common symptoms of pulmonary arterial hypertension (PAH). If you have shortness of breath, you may feel like you’re not getting enough air or that you can’t catch your breath.
Feeling breathless can significantly affect your quality of life and ability to take care of yourself. “The shortness of breath and fatigue is disabling,” shared a myPHteam member who was diagnosed with pulmonary hypertension.
In this article, we’ll discuss how shortness of breath develops when you have PAH and how treatments can help.
Understanding why shortness of breath happens with PAH is essential for understanding how treatments can help.
PAH is a type of pulmonary hypertension that occurs when pulmonary arteries become thicker and narrower. Pulmonary arteries are the blood vessels that carry oxygen-poor blood from the heart to the lungs. If the pulmonary arteries get too thick and narrow, blood flow between the heart and lungs gets blocked, and blood pressure in the lungs rises.
That increased blood pressure means that the right side of the heart must work harder to pump blood to your lungs. The extra strain can eventually lead to heart failure.
Narrowed and thickened pulmonary arteries also make it more difficult for oxygen-poor blood to reach your lungs and gain oxygen. As a result, less oxygen-rich blood goes to the rest of your body. Symptoms of PAH may include:
It’s normal to become short of breath when you're exercising or at high altitudes, but people with PAH may experience this symptom even during normal daily activities. In the early stages of PAH, you may notice only mild shortness of breath when exercising or climbing stairs.
“I’m running so short of breath after riding the stationary bike for 30 minutes,” a myPHteam member said.
As PAH progresses, you may feel short of breath doing activities that didn’t cause this symptom before. “I have severe PAH. Just walking across a room gets me out of breath,” one member said. Another replied, “Shortness of breath on exertion is very common every day.”
PAH treatments usually work by dilating (opening or widening) pulmonary arteries by targeting pathways involved in PAH. Dilating the blood vessels in your lungs can help decrease the blood pressure in your pulmonary arteries and reduce the strain on your heart.
Treatments for PAH can help slow disease progression and improve your symptoms, including shortness of breath. You and your health care provider will develop a treatment plan based on the severity of your PAH and your specific needs.
Oral treatments come in the form of a pill that you take by mouth. Oral treatments for PAH help open the pulmonary arteries by either stimulating blood vessel dilation or preventing blood vessels from narrowing.
Endothelin receptor antagonists are a class of medications that prevent blood vessels from narrowing. These medications work by blocking endothelin, a substance that makes blood vessels constrict. Examples of endothelin receptor antagonists include:
Endothelin receptor antagonists can help people feel less short of breath during physical activity.
Phosphodiesterase inhibitors — also known as PDE-5 inhibitors — help the lungs make more vasodilators (substances that help open blood vessels). These medications can help widen your pulmonary arteries and allow more oxygen-poor blood to flow into your lungs and out to the rest of your body. Examples of PDE-5 inhibitors include:
PDE-5 inhibitors have been shown to improve the ability to exercise.
Other oral treatments for PAH aim to relax the blood vessels in your lungs so that your heart can more easily pump blood to your lungs. Examples include:
These medications can help slow disease progression and improve your ability to exercise.
Inhaled treatments are medications you take by drawing them directly into your lungs using a special device.
Inhaled treatments for PAH are laboratory-made versions of prostacyclins, substances naturally found in your body that help open blood vessels. Inhaled treatments help dilate your pulmonary arteries and reduce the strain on your heart. Examples of inhaled treatments for PAH include:
Using an inhaled treatment may allow you to be more physically active.
Intravenous (IV) and subcutaneous treatments are injected into your body. IV treatments go into your veins, and subcutaneous treatments are delivered under your skin.
Treprostinil (Remodulin) is a laboratory-made prostaglandin that can be given as an IV infusion or through a portable infusion pump. Taking treprostinil can improve both your ability to exercise and the shortness of breath caused by physical activity. Another IV drug, epoprostenol (Flolan, Veletri), has been shown to improve shortness of breath and fatigue (tiredness).
Sotatercept (Winrevair) is given as a subcutaneous injection every three weeks. This medication helps regulate the growth of blood vessel walls, which keeps PAH from getting worse and improves the ability to exercise.
Supportive treatments can help ease your symptoms, but they don’t treat the underlying cause of PAH.
Calcium channel blockers, such as nifedipine (Procardia) and diltiazem (Cardizem), can help lower the blood pressure in your lungs and entire body. However, calcium channel blockers don’t work for everyone with PAH, and research has found that these medications may not improve exercise ability.
Other supportive treatments may help treat or prevent complications of PAH, such as blood clots and swelling. However, they may not improve shortness of breath.
If your blood oxygen levels are low, your health care provider may prescribe oxygen therapy.
This supportive therapy may be helpful for some people with PAH. Supplemental oxygen can help you feel less breathless, be more active, and continue your daily activities.
Pulmonary rehabilitation is a program that teaches you how to exercise with less shortness of breath. A pulmonary rehabilitation class may offer disease education, supervised exercise, and breathing techniques, as well as provide nutrition advice and emotional support. Talk to your health care provider to find out if pulmonary rehabilitation may be able to improve your shortness of breath caused by PAH.
Lifestyle changes are actions you can take to help improve shortness of breath due to PAH, such as:
A myPHteam member also suggests remaining indoors in hot, humid weather: “I’m a little short of breath today. The temperature is 98 degrees and very humid. Days like this are better spent at home.”
Talk to your health care provider to learn more about lifestyle changes that may help with your shortness of breath.
On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 55,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.
What PAH treatment options have you tried? Which treatments have improved your shortness of breath? Share your experience in the comments below, or start a conversation by posting on your Activities page.
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Vedran Radonić, M.D., Ph.D.
completed medical school and his Ph.D. at the University of Zagreb, Croatia.
Learn more about him here.
Amanda Jacot, PharmD
earned a Bachelor of Science in biology from the University of Texas at Austin in 2009 and a Doctor of Pharmacy from the University of Texas College of Pharmacy in 2014.
Learn more about her here.
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