Familial primary pulmonary arterial hypertension (PAH), is also known as pulmonary hypertension. It is a hereditary type of hypertension in the lungs that affects your pulmonary blood vessels. These blood vessels convey blood from the lower right of your heart (right ventricle) into your lungs.

As the weight in your pulmonary blood vessels and capillaries increase, your heart must work harder to siphon blood to your lungs. After some time, this tires out your heart muscle. In the long run, it can prompt heart problems and death.

There is no known solution for PAH, yet treatment alternatives are there. If you have PAH, treatment may help calm your side effects, bring down your risks of having complications, and save you from death.


In the beginning stages of PAH, you probably won’t have any detectable symptoms. As the condition deteriorates, the symptoms will manifest themselves. These symptoms include:

  • Trouble breathing
  • Fatigue
  • Dizziness
  • Fainting
  • Weight on chest
  • Chest pain
  • Fast heartbeat
  • Heart palpitations
  • Pale blue color to your lips or skin
  • Swelling of your lower legs


If your doctor presumes you may have PAH, he or she will probably arrange at least one test to evaluate your respiratory blood vessels and heart.

Tests for diagnosing PAH may include:

  • Electrocardiogram
  • Echocardiogram
  • Chest X-ray
  • CT scan or MRI
  • Right heart catheterization
  • Blood tests to check for conditions related to PAH or other conditions


Right now, there’s no known treatment for PAH, yet treatment can ease symptoms, diminish the danger of complexities, and prolong life.

To help deal with your condition, your doctor may recommend at least one of the following medicines:

  • Prostacyclin
  • Soluble guanylate cyclase stimulators
  • Endothelin receptor antagonists
  • Anticoagulants

If you have coronary artery disease or heart failure, your doctor may suggest a heart transplant.


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