Primary Pulmonary Hypertension (PPH)

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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 treated.

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:

Blue color to lips and skin
Chest pain (angina)
Dizziness
Dry cough
Extreme tiredness (fatigue)
Fainting
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 diagnosis.

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 electrodes 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 a vein in the groin or the arm. They thread 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.
Chest CT scan. This is a type of imaging test that uses X-rays and a computer to make detailed pictures of the inside of your chest. These images are better than regular X-rays. They can give more details about injuries or diseases of the chest organs. This test can assess lung tissue and blood flow.

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 the following:

Medicines

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 blood.
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.
Other medicines. These are used to help lower blood pressure in the lungs. They also help the heart beat stronger and pump more blood.

Oxygen therapy

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.

Staying healthy

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

Primary pulmonary hypertension (PPH) is a rare lung disorder that causes high blood pressure in the lungs.
Experts don't know what causes it.
Symptoms can develop so slowly that you can have the condition for years without knowing it. And symptoms get worse as the disease progresses.
Pulmonary hypertension is a serious, ongoing (chronic) disease. It can lead to heart failure if it’s not treated.
There is no cure for the condition. Treatment is aimed at managing symptoms.

Next steps

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.

Medical Reviewer: Steven Kang MD

Medical Reviewer: Ronald Karlin MD

Medical Reviewer: Stacey Wojcik MBA BSN RN

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