Life with Pulmonary Hypertension

Pulmonary hypertension, sometimes called pulmonary arterial hypertension (PAH), is high blood pressure in the arteries between your lungs and heart. Unlike regular chronic hypertension (high blood pressure), pulmonary hypertension is very rare. This life-threatening health condition makes it hard to breathe and can damage your heart.

It’s scary to learn you have a problem with your lungs or heart. A diagnosis of pulmonary hypertension is often life-changing, but there are treatments and ways to manage it. Keep reading to learn what living with pulmonary hypertension means for you or your loved ones.

What Is Pulmonary Hypertension?

Pulmonary hypertension causes the arteries in your lungs to become stiff and narrow. It reduces blood flow through the lungs and causes low oxygen levels in your blood. PH also causes the right side of your heart to work harder, which may increase your risk of heart failure.

Pulmonary arterial hypertension (PAH) is a type of pulmonary hypertension that may develop for unclear reasons, called idiopathic pulmonary arterial hypertension (iPAH). PAH can run in families because of a gene that gets passed on.

Other causes of PAH may include:

  • Chronic health conditions like autoimmune diseases that affect your connective tissue (such as scleroderma), cirrhosis, or HIV.
  • Using certain medicines or illegal drugs.

PH can also develop because of:

  • Some heart diseases, like heart failure, heart valve problems, or a heart defect you’re born with.
  • Lung diseases that reduce oxygen in your lungs, like COPD, emphysema, obstructive sleep apnea, or scarring in your lungs from pulmonary fibrosis.
  • Blood clots in your lungs (pulmonary emboli).

Managing any underlying health conditions is crucial when living with PAH and other types of pulmonary hypertension. Doing so can help slow the progression of the disease.

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Watch for Changing Symptoms

Pulmonary hypertension comes on slowly and may only cause mild symptoms at first. But symptoms often get worse as the condition progresses. It’s vital to monitor your symptoms and be on the lookout for any new ones, which can include:

  • Increasing fatigue.
  • Shortness of breath while exercising or at rest in more advanced stages.
  • Dizziness or fainting.
  • Blue lips or fingers
  • Swelling in your feet, legs, and eventually belly and neck.
  • An irregular or very fast heartbeat.
  • Chest pain.

Alert your doctor

Make sure you call your health care provider if any symptoms get worse. It might mean that your condition is worsening. Your doctor can screen, diagnose, and monitor pulmonary hypertension with these tests:

  • Exercise stress tests to see how your heart works during exercise.
  • Lung function tests to check your lung health.
  • Imaging tests like electrocardiogram (ECG) or echocardiogram to see how well your heart beats and pumps blood.
  • Cardiac catheterization to measure the blood pressure in your lungs and heart. A heart doctor inserts a catheter (small tube) into a vein in your neck, arm, or groin. They’ll thread it through the right side of the heart and into your pulmonary artery, where they can directly measure the pressure.

Treatments for People Living with PAH

Although there is no cure for PAH, various treatments can help manage your symptoms and slow the progression of this disease. These will improve your quality of life and help you live longer. Treatments have improved significantly over the past years, and life expectancy continues to increase.

Treatments can vary from person to person, so teaming with your pulmonary hypertension specialists is essential. They’ll monitor how well different treatments work and design the best plan for you. Most people need a combination of these therapies to manage symptoms:

Pulmonary rehabilitation

Pulmonary rehab includes education and exercises for your lungs to help you breathe easier. You may also have oxygen therapy from a portable oxygen tank.


Medication treatments can help:

  • Relax and open narrowed blood vessels so blood can pass through more easily.
  • Increase blood flow throughout the arteries in your lungs.
  • Relax the muscles in the walls of your blood vessels.
  • Strengthen and regulate your heartbeat.
  • Thin your blood to prevent blood clots.
  • Remove excess fluid that may build up in your lungs, legs, or abdomen.
  • Relieve shortness of breath.

It’s important to take any medications as directed and not skip doses.

Lifestyle changes

Lifestyle changes are also a vital part of PAH treatment and can help you feel better. Make sure you:

  • Quit smoking if you smoke cigarettes.
  • Maintain a healthy weight.
  • Eat more fruits and vegetables and cut back on fast foods and other sources of sodium in your diet.
  • Avoid hot tubs, saunas, and traveling to high altitudes, which can strain your heart and lungs. Ask your doctor if it’s safe for you to fly.
  • Exercise regularly but safely. A physical therapist can show you the safest forms of exercise for PAH.
  • Stay healthy and avoid respiratory infections by getting preventive care like a pneumonia vaccine, annual flu shot, and Covid-19 vaccine if appropriate.

Monitor Your Pulmonary Hypertension

With pulmonary hypertension, it’s essential to stay in close contact with your medical team. Your doctors may ask you to have periodic exercise stress tests, lung function tests, and imaging tests. These tell them how well your treatments are working.

If rehab, medication, and lifestyle changes don’t effectively manage your symptoms, your specialists may recommend surgery. In the most severe case, you may need a lung or heart-lung transplant.

Have a Support System

Living with pulmonary hypertension can feel overwhelming and stressful, with so much to consider. Don’t be afraid to ask for help when you need it — whether at home or at your medical appointments.

Keep a list of questions and ask a friend or family member to accompany you to medical appointments whenever possible. They can help you remember details you might forget. Also, support groups are invaluable because you can connect with and learn from others living with PAH.

And remember, having a team of medical experts to guide and support you can ease much of your stress. It allows you to focus on staying healthy and living a normal, happy life.

American Lung Association. Pulmonary Arterial Hypertension. LINK

Pulmonary Hypertension Association. Traveling with PH. LINK

About Pulmonary Hypertension

The UPMC Comprehensive Pulmonary Hypertension Program provides complete services for pulmonary hypertension (PH) and other lung diseases. We have specialists available for surgical and nonsurgical treatment of PH and related diseases like heart failure, HIV, and COPD. We have the largest multidisciplinary program in the U.S. and offer cutting-edge diagnostics and individualized treatment plans. We also are leaders in research for new treatments. Find a PH specialist near you.