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What does this image mean?  Article about primary pulmonary hypertension; high blood pressure; symptoms of primary pulmonary hypertension; management of primary pulmonary hypertensionPrimary Pulmonary Hypertension - A Rare Disease with Many Names

Primary pulmonary hypertension is not a very common disease, but many more people are affected by primary pulmonary hypertension than you may be aware.

Primary pulmonary hypertension is defined as an abnormally high blood pressure in the arteries of your lungs. The word "primary" in this term means that no other diseases have been identified of the lungs or heart that is causing high blood pressure.

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Pulmonary Hypertension Has Many Names

To name just a few, the following terms also refer to pulmonary hypertension:

  • Pulmonary arterial hypertension
  • Sporadic primary pulmonary hypertension
  • Familial primary pulmonary hypertension

What Are Some of the Causes and Incidences of Primary Pulmonary Hypertension?

The cause of primary pulmonary hypertension is unknown.  It is believed that some cases are caused by genetics (something you are born with).

Pulmonary hypertension is the result of greater resistance to blood flow. As a result of the increased workload caused by this resistance, the right side of the heart becomes enlarged and overworks the heart.  Eventually, progressive heart failure may develop.

It is a fairly rare condition that tends to affect more women than men.

A Short Summary of Primary Pulmonary Hypertension Symptoms

  • Shortness of breath upon exertion
  • Chest pain
  • Weakness
  • Fatigue
  • Fainting
  • Light-headedness during exercise
  • Dizziness
  • Leg swelling (edema) 
Medical Testing For This Disease

If you are experiencing the above-listed symptoms, you should visit a medical professional.  A physical examination may reveal an increase in the size of the veins in the neck, normal lungs, a heart murmur, enlargement of the liver, and swelling (edema) due to fluid retention in the bodies' tissues. Your doctor might order the following tests:

  • Chest X-ray
  • Echocardiogram
  • Pulmonary function tests
  • Nuclear lung scan
  • CT scan of the chest
  • Cardiac catheterization
  • Pulmonary arteriogram
Management of Primary Pulmonary Hypertension Disease 

There is no known cure for primary pulmonary hypertension.  The main goal of treatment is to control the symptoms. Oral medications, such as calcium channel blockers and diuretics, appear to be promising treatments. More severe cases may need IV treatments like epoprostenol. Recently, new drugs that block the abnormal constriction of blood vessels have become available and show promise in treating pulmonary hypertension. As the disease moves forward in its progression, oxygen may be needed. As a last resort, screened candidates may require a lung or heart-lung transplantation.

Pulmonary Hypertension Association is an example of a good patient and caregiver support.

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