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Symptoms of pulmonary arterial hypertension (PAH) can be serious and shouldn’t be ignored. Maintaining your treatment plan and reporting critical PAH symptoms to your doctor is key to slowing disease progression (worsening) and improving your quality of life.
PAH is a rare but serious form of pulmonary hypertension (PH). PAH causes blood vessels in the lungs to become damaged and narrowed, which compromises oxygen passing from the lungs to the blood and causes high blood pressure in the lungs. Stress on the right side of the heart, where the heart muscle must work harder to pump blood into the lungs, puts people with PAH at a high risk of heart failure and other life-threatening heart diseases. Although there is no cure for PAH, the condition can be managed with treatment, monitoring, and lifestyle changes.
Members of myPHteam often discuss and ask questions about PAH symptoms. One member wrote, “I think sometimes this group should write a book about PAH and what to expect, so we won’t be so surprised when we find out the symptoms we have been experiencing are symptoms of PAH that we didn’t know about.”
Symptoms of PAH include the following:
Recognizing serious symptoms can help ensure you get timely care and are monitored for disease worsening. Regular cardiology risk assessment tests — including right heart catheterization, echocardiogram, and blood tests — can help your doctor evaluate you for serious risks associated with PAH symptoms. Even some common symptoms shouldn’t be ignored.
Here are symptoms you should pay attention to and discuss with your cardiologist or pulmonologist.
Shortness of breath is often the first noticeable symptom of PAH in its early stages. Persistent breathlessness, particularly during physical activity or while at rest, may be a sign of rising blood pressure in the pulmonary arteries. As the blood vessels become increasingly damaged and narrowed, the heart has to work harder to pump blood through the lungs, leading to reduced oxygen exchange and respiratory difficulty.
During the course of PAH, increasing shortness of breath may indicate that your condition is getting worse.
One myPHteam member wrote, “My day started at 3 a.m. when I woke up gasping for breath, couldn’t breathe, wheezing, grabbing my chest with a sharp pain. Fear again, was it a heart attack or PAH?”
Do not ignore shortness of breath. It may later lead to a PAH diagnosis if your doctor rules out common lung diseases, such as asthma or chronic obstructive pulmonary disease (COPD). If you find it increasingly difficult to get enough air, you should notify your doctor as soon as possible. The sooner you do, the sooner they can determine whether you’d benefit from changing your treatment plan.
Feeling light-headed or dizzy can also be an early symptom of PAH. Syncope (fainting or suddenly losing consciousness) can occur with severe dizziness. You can get dizzy when your brain isn’t getting enough oxygen due to the limited function of the heart caused by PAH. People with PAH may experience dizziness during physical activity or even when remaining still.
“Falling over from being dizzy and lightheaded,” one myPHteam member wrote. Another member said, “This has been a bad week for me. When I move, I get dizzy, nauseous, and weak.”
Fainting can be a sign of advanced PAH, and you should be evaluated by your doctor if you experience severe dizziness or fainting. Fainting can be life-threatening if it occurs while you’re driving or in any situation where falling can be dangerous.
Your doctor may recommend the following to treat dizziness or fainting:
Coughing can be a complex symptom in pulmonary hypertension in adults, and it’s not always directly related to PAH. Chronic coughing may be caused by various underlying lung conditions such as COPD, pulmonary fibrosis, or other respiratory disorders.
For some individuals with PAH, a persistent cough can lead to complications. For instance, chronic coughing attacks might result in temporary loss of consciousness or fainting. A myPHteam member described their experience with coughing and fainting. “I have constant coughing attacks, and they always end with loss of consciousness,” they wrote. “I would get up and faint.”
If you have a persistent cough, especially one lasting more than eight weeks, it’s important to be evaluated for potential underlying lung conditions. Your healthcare provider can help determine its root cause and appropriate treatment.
A myPHteam member wrote, “Been up, off and on, all night coughing and coughing. I'm so tired.” Another chimed in, saying, “The hard coughing had me vomiting, which brought up a lot of mucus. I had about 3 coughing spells that did that.”
Chest pain is a common symptom of PAH, which often happens during physical exertion. “I’ve noticed when I do strenuous activities that the next day or two, I’m exhausted and have to rest more. I also have more chest pain,” a myPHteam member wrote.
Chest pain with PAH can also be a sign of a coinciding and serious condition, such as left main coronary artery (LMCA) compression, which can lead to a heart attack or other types of heart disease. LMCA compression can sometimes be treated with a stent — a tube that’s inserted into a blood vessel to keep it open. A stent can increase blood flow and may help stop symptoms of chest pain in some people with PAH.
A weakened heart muscle due to PAH can also lead to a higher risk of irregular heart rate or arrhythmia, which can cause discomfort in the chest. Arrhythmia can be treated, which may help slow disease progression. An irregular heartbeat is also a risk factor for heart attack and stroke.
If you’re experiencing ongoing or severe chest pain or any other discomfort in your chest, be sure to let your doctor know right away.
People with PAH may have edema (swelling) in their legs and feet. This may eventually cause swelling in other parts of the body, like the neck and stomach. Swelling may be caused by fluid retention and can be a sign of disease progression and right heart failure. Swelling can be a serious symptom and lead to further complications.
“I have a lot of swelling in my legs and hurt all over,” a myPHteam member wrote.
If you have PAH, it’s important to be evaluated regularly for fluid retention and swelling. This symptom may be caused by worsening disease, a side effect of a PAH medication, or another health condition or drug you take for a comorbidity (coinciding condition).
Swelling may indicate a blood clot and should be treated with blood thinners immediately, especially if swelling includes symptoms such as:
Always report swelling to your healthcare provider, who may advise a diet low in sodium (salt) to reduce swelling or a plan for managing higher body weight. Diuretic medications are sometimes recommended to decrease fluid retention. A myPHteam member described their experience with swelling: “Edema slowly going away. I’m taking a diuretic. They are switching meds.”
Another member shared, “My feet, ankles, and calves are so swollen that they are hard and painful. I am on water pills and elevating them, but the swelling has gotten worse over the past 2 weeks. My legs are so big I can't put on compression socks.”
The fatigue that often occurs with PAH can affect both your physical and mental well-being. Because fatigue is a common symptom of PAH, many people with PAH overlook it. However, doctors are starting to recognize the importance of understanding the cause of fatigue in people with PAH and managing it as well as possible.
One myPHteam member wrote, “I am reaching out wondering if my extreme fatigue is real or in my head. All I want to do is stay in bed. If I get up, I become painfully fatigued after doing very little.”
Severe or extreme fatigue may be due to factors such as:
One myPHteam member shared their experience with fatigue and problems with sleep: “Once I start moving about, I’m getting winded as usual, which creates more fatigue. Then, by the end of the day, I can’t get to sleep! So it has been a vicious cycle of getting winded, fatigue, and sleeplessness.”
If you have debilitating fatigue, talk with your doctor about the potential causes and how to manage it. Physical therapy and exercise can sometimes help reduce fatigue. In other cases, your PAH may be getting worse, and your healthcare provider may make changes to your treatment plan.
Low blood oxygen levels may cause your lips or fingers to look blue, white, or gray (depending on skin tone) in people with PAH. Abnormally low blood oxygen levels can be serious and should be evaluated as soon as possible. Also called cyanosis, discolored lips or fingers can be a sign of heart failure and an indication of worsening PAH.
Peripheral cyanosis, which involves the hands, fingers, or toes, typically isn’t life-threatening. However, discoloration in your mucous membranes, such as the lips or inside the mouth, can be a sign of a medical emergency. Be sure to contact your doctor right away if you have this symptom.
On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 57,000 members come together to ask questions, give advice, and share their stories with others who understand life with PH and PAH.
Do you have symptoms of PAH, such as shortness of breath, swelling, or fatigue? Have you discussed these symptoms with your doctor? Share your experience in the comments below, or start a conversation by posting on your Activities page.
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