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 and improving your quality of life.

PAH is a rare but serious form of pulmonary hypertension (PH). With PAH, blood vessels in the lungs become damaged and constricted, causing hypertension (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:

• Dyspnea (shortness of breath)
• Hoarseness and cough
• Dizziness and fainting
• Chest pain or heart palpitations (fast-beating, fluttering, or pounding heart)
• Swelling, such as in hands and feet
• Extreme tiredness
• Discolored lips or fingers

Recognizing serious symptoms can help ensure that you get timely care and are monitored for disease progression. Regular risk assessment — including right heart catheterization, echocardiogram, and blood tests — can help manage serious risks associated with PAH symptoms. Even some common symptoms should not be ignored. Here are symptoms you should pay attention to and discuss with your cardiologist or pulmonologist.

1. Shortness of Breath

Shortness of breath or breathlessness often occurs with PAH, and it can be serious. In many cases, shortness of breath is the first noticeable symptom of PAH, and it may ultimately lead to a diagnosis if your doctor rules out more common lung diseases, such as asthma and chronic obstructive pulmonary disease (COPD).

During the course of PAH, if shortness of breath increases either during activity or at rest, it may indicate that your PAH is progressing.

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 take shortness of breath for granted. 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 assess you to determine whether you’d benefit from changing your treatment plan.

2. Dizziness and Fainting

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 get dizzy when your brain isn’t getting enough oxygenated blood due to limited function of the heart caused by PAH. People with PAH may experience dizziness during physical activity or even from remaining still.

“Falling over from being dizzy and light-headed,” one myPHteam member wrote. Another member said, “This has been a bad week for me. When I move I get dizzy, nauseous, 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:

• A change in medication
• Physical therapy
• Avoidance of physical exertion
• Cardiopulmonary rehabilitation

For some serious cases of fainting caused by PAH, a newer surgical procedure called an atrial septostomy can help, and a device called an atrial flow regulator can be placed in the heart to allow blood to bypass the high pressures in the lungs and flow directly into the left side of the heart. This procedure has been shown to provide relief from dizziness and fainting in some people.

Some people with PAH may experience a chronic cough — coughing that lasts at least eight weeks — that causes fainting. A team member described their experience with this symptom. “I have constant coughing attacks, and they always end with loss of consciousness,” they wrote. “I would get up and faint.”

If you have PAH and excessive coughing, it’s important to be evaluated for a coinciding condition such as COPD.

3. Chest Pain and Irregular Heartbeat

Chest pain is another 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, which increases blood flow and may resolve 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 heartbeat or arrhythmia, another cause of 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 are experiencing persistent and severe chest pain, be sure to let your doctor know.

4. Swelling in Your Legs or Abdomen

People with PAH may have edema (swelling) in their legs, abdomen, or other parts of the body. This swelling is caused by fluid retention and may 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 along with the following symptoms may indicate a blood clot and should be treated immediately:

• Cramping or pain
• A skin color change
• Warmth in the swollen area

Always report swelling to your health care provider, who may advise a diet low in sodium (salt) to reduce swelling or a plan for managing high weight. Diuretic medications are sometimes recommended to decrease fluid retention. A myPHteam described their experience with addressing edema: “Edema slowly 🐌 going away. I’m taking a diuretic. They are switching meds.”

5. Extreme Fatigue

The fatigue that commonly occurs with PAH can affect both physical and mental well-being. Because fatigue is a typical and expected symptom of PAH, its potential impact can be overlooked. However, clinicians are starting to recognize the importance of managing fatigue and determining the cause in people with PAH.

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:

• Worsening PAH
• Hypoxia (low blood oxygen levels)
• Side effects of medication
• Reduced physical activity and muscle strength
• A coinciding condition, such as sleep apnea (breathing that stops and starts while you sleep)

One myPHteam member shared how sleep apnea affects their quality of life: “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 management of fatigue. Physical therapy for appropriate exercise and physical activity can sometimes help reduce fatigue. In other cases, PAH may be getting worse, and your physician may make changes to your treatment plan.

6. Discolored Lips or Fingers

Low blood oxygen may lead 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 quickly evaluated. Also called cyanosis, discolored lips or fingers can be a sign of heart failure and an indication of worsening PAH.

Peripheral cyanosis, which involves hands, fingers, or toes, is seldom life-threatening. However, discoloration in 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.

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 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.