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 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 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. 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:
- Bluish lips and skin
- Chest pain (angina)
- Dry cough
- Extreme tiredness
- 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 primary pulmonary hypertension looks like other conditions or medical
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?
Primary pulmonary hypertension 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.
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 detect 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 transducer is passed over the
skin over the heart. Echo can show damage and enlargement of the heart.
- 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 usually done with special machines you breathe
- 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 check the function of the lungs.
- Cardiac catheterization of the right side of the heart. In this procedure, the doctor passes a hollow tube through a large 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
- 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 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 one or more of the following:
- Anticoagulants. These are blood thinners used to make the blood less likely to clot and help it flow
- 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/vasodilator drugs. 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. These medicines include ambrisentan, sildenafil,
- Other drugs. These are used to help lower blood pressure in the lungs and 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 maintain a healthy lifestyle with
primary pulmonary hypertension:
- Don’t smoke.
- Try to avoid 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 primary pulmonary hypertension.
- Pregnancy and childbirth can pose serious dangers to a women with this condition. Talk
with your healthcare provider if you are considering pregnancy.
- 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 pulmonary hypertension
- Primary pulmonary hypertension (PPH) is a rare lung disorder that causes high blood
pressure in the lungs.
- The cause of it is unknown.
- Symptoms can develop so slowly that you can have the condition for years without knowing
it. And symptoms get worse as the disease progresses.
- There is no cure for the condition. Treatment is aimed at managing symptoms.
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
- Know how you can contact your healthcare provider if you have questions.