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)
  • 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

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.