Primary Pulmonary Hypertension (PPH)
What is primary pulmonary hypertension?
Primary pulmonary hypertension
(PPH) is high blood pressure in the lungs. It is also known as idiopathic pulmonary
arterial hypertension. It’s a rare lung disorder in which the blood vessels in the lungs
narrow (constrict) and the pressure in the pulmonary artery rises far above normal
levels. The pulmonary arteries carry blood from your body to the lungs, where carbon
dioxide is traded for oxygen.
Pulmonary hypertension is a
serious, ongoing (chronic) disease. It can lead to heart failure if it’s not
What causes primary pulmonary hypertension?
Experts don't know what causes
primary pulmonary hypertension. Often there is no underlying heart or lung disease
causing the high blood pressure.
Some forms of pulmonary
hypertension are linked to a gene defect that can run in families. Researchers believe
this gene mutation makes the blood vessels more sensitive to certain factors. They
believe the blood vessels narrow when exposed to these factors.
What are the symptoms of primary pulmonary hypertension?
Each person's symptoms may vary.
Symptoms can develop so slowly that you can have it for years without knowing it.
Symptoms may include:
- Bluish lips and skin
- Chest pain (angina)
- Dry cough
- Extreme tiredness (fatigue)
- Feeling like your heart is fluttering or beating fast or hard (palpitations)
- Racing pulse
- Swelling in the ankles or legs
- Trouble breathing or shortness of breath, especially with activity
- Trouble getting enough air
These symptoms get worse as the disease progresses. More severe symptoms are a sign of more advanced disease. In advanced stages, you may:
- Have symptoms even when resting
- May become bedridden
The symptoms of PPH looks like
other conditions or health problems. This condition can be hard to diagnose. Many people
ignore symptoms because they think they are simply out of shape. If you or a family
member have any of these symptoms, talk with your healthcare provider for a
How is primary pulmonary hypertension diagnosed?
PPH is rarely discovered in a
routine health exam. In its later stages, the signs of the disease can be confused with
other conditions affecting the heart and lungs.
It may be diagnosed when other diseases are ruled out. Tests may include:
- Chest X-ray. A test that takes pictures of internal tissues, including the heart.
- ECG. This test records the strength
and timing of the electrical activity of the heart. It shows abnormal rhythms and can
sometimes find heart muscle damage. Small sensors are taped to your skin to pick up
the electrical activity.
- Echocardiogram. This test uses sound
waves to check the heart’s chambers and valves. The echo sound waves create a picture
on a screen as an ultrasound probe (transducer) is passed over the skin over the
heart. This test can show heart damage and enlargement.
- Pulmonary function tests. These are
diagnostic tests that help to measure the lungs’ ability to move air into and out of
the lungs. The tests are often done with special machines you breathe into.
- Perfusion lung scan. This is a type
of nuclear radiology test. A small amount of a radioactive substance is used to help
find changes in the arteries leading to the lungs and blood flow within the lungs.
This scan can also be used to check lung function.
- Cardiac catheterization of the right side
of the heart. In this test, the doctor passes a hollow tube through a large
tube (catheter) in the vein. He threads the tube through the right side of the heart
and into the pulmonary artery. This is the only test that directly measures the
pressure inside the pulmonary arteries.
- Blood tests. These can be used to
check the oxygen levels in the blood, assess liver and kidney function, and look for
other diseases. Certain blood tests can also help to check strain on the heart.
How is primary pulmonary hypertension treated?
There is no cure for primary
pulmonary hypertension. Treatment tries to manage symptoms and may include 1 or more of
- Anticoagulants. These are blood thinners used to make the blood less likely to clot and help it flow more freely.
- Diuretics. Called water pills,
these are used to decrease the amount of fluid in the body. They help reduce
swelling and the amount of work the heart has to do.
- Calcium channel blocking or vasodilator
medicines. These are used to improve the heart’s ability to pump
- Pulmonary artery specific therapy. This type of medicine targets the pulmonary arteries and tries to decrease the pressure of the blood flow through these arteries. These medicines include ambrisentan, sildenafil, and epoprostenol.
- Other medicines. These are used to
help lower blood pressure in the lungs. They also help the heart beat stronger and
pump more blood.
Some people also need supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult.
Lung or heart-lung transplant
This may be a choice for people with severe disease.
The condition can be made worse
by a number of factors. You can do certain things that will help you stay as healthy
as possible. To keep a healthy lifestyle with primary pulmonary hypertension:
- Don’t smoke.
- Try to stay away from high
altitudes and travel in nonpressurized airplane cabins. Consider using
supplemental oxygen during air travel.
- Before starting an exercise program, ask your healthcare provider about the type and amount of physical activity that is safe for you.
- Don’t participate in activities that can lead to dangerous symptoms, such as chest pain or dizziness.
- Be careful when using both
prescription and over-the-counter medicines. Ask your physician or pharmacist
which medicines are safe for a person with PPH.
- Pregnancy and childbirth can pose
serious dangers to women with this condition. Talk with your healthcare provider
if you are thinking about getting pregnant.
- Consider getting a pneumococcal pneumonia vaccine and yearly flu vaccines. Flu and pneumonia can be very dangerous for people with this condition.
Key points about primary pulmonary hypertension
Tips to help you get the most from a visit to your healthcare provider:
- Know the reason for your visit and what you want to happen.
- Before your visit, write down questions you want answered.
- Bring someone with you to help you ask questions and remember what your healthcare provider tells you.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.
- Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
- Ask if your condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if you do not take the medicine or have the test or procedure.
- If you have a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your healthcare provider if you have questions.
Online Medical Reviewer:
Alan J Blaivas DO
Online Medical Reviewer:
Daphne Pierce-Smith RN MSN CCRC
Online Medical Reviewer:
Marianne Fraser MSN RN
Date Last Reviewed:
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