What is primary pulmonary hypertension?

Primary pulmonary hypertension (PPH) is high blood pressure in the lungs. It’s a rare lung disorder in which the blood vessels in the lungs narrow 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, chronic disease that can lead to heart failure if it’s not treated.

What causes primary pulmonary hypertension?

The cause of primary pulmonary hypertension (PPH) is unknown. 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 and they constrict, or narrow, when exposed to these factors.

What are the symptoms of primary pulmonary hypertension?

The following are the most common symptoms for primary pulmonary hypertension (PPH). Symptoms can develop so slowly that you can have it for years without knowing it. However, each person may experience symptoms differently. Symptoms may include:

• Extreme tiredness (fatigue)
• Trouble breathing or shortness of breath, especially with activity
• Dry cough
• Dizziness
• Fainting
• Swelling in the ankles or legs
• Bluish lips and skin
• Chest pain (angina)
• Racing pulse
• Trouble getting enough air
• You feel like your heart is fluttering, or beating fast or hard (palpitations)

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 look like other conditions or medical problems, and PPH can be hard to diagnose. Consult a health care provider for a diagnosis.

How is primary pulmonary hypertension diagnosed?

Primary pulmonary hypertension (PPH) is rarely discovered in a routine medical exam. In its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.

PPH 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.
• Electrocardiogram (ECG). This test records the strength and timing of the electrical activity of the heart. It shows abnormal rhythms  and can sometimes detect heart muscle damage. Small sensors are taped to your skin to pick up the electrical activity.
• Echocardiogram (echo). 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 transducer is passed over the skin over the heart. Echo can show damage and enlargement of the heart.
• Pulmonary function tests (PFTs). These are diagnostic tests that help to measure the lungs' ability to move air into and out of the lungs. The tests are usually done with special machines you breathe into.
• Perfusion lung scan. A type of nuclear radiology test in which 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 assess the function of the lungs.
• Cardiac catheterization of the right side of the heart. With this procedure, X-rays are taken after a dye (contrast agent) is injected into the pulmonary artery to look for any narrowing, blockages, or other changes. 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, evaluate liver and kidney function, and look for other diseases. Certain blood tests can also help to assess strain on the heart.

How is primary pulmonary hypertension treated?

There is no cure for primary pulmonary hypertension (PPH). Treatment is aimed at managing symptoms and may include one or more of the following:

Medications

These include:

• Anticoagulants. These are blood thinners used to make the blood less likely to clot and help it flow more freely.
• Diuretics. These "water pills" are used to decrease the amount of fluid in the body, reduce swelling, and reduce the amount of work the heart has to do.
• Calcium channel blocking/vasodilator drugs. These are used to improve the heart's ability to pump blood.
• Other drugs. These are used to help lower blood pressure in the lungs and 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 an choice for people with severe PPH.

Key points about pulmonary hypertension

• Primary pulmonary hypertension (PPH) is a rare lung disorder that causes high blood pressure in the lungs.
• The cause of PPH is unknown.
• Symptoms of PPH can develop so slowly that you can have PPH for years without knowing it. And symptoms get worse as the disease progresses.
• There is no cure for PPH. Treatment is aimed at managing symptoms.

Next steps

Tips to help you get the most from a visit to your health care provider:

• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.