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Pulmonary Arterial Hypertension

What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by high blood pressure (hypertension) in the arteries of the lungs (pulmonary artery) for no apparent reason. PAH is a chronic and life-changing disease that can lead to right heart failure if left untreated.

Symptoms

The symptoms for all types of pulmonary hypertension may be similar, and symptoms are usually more severe as the disease progresses. Symptoms of PAH may include:

  • Breathlessness
  • Chest pain (also called angina pectoris)
  • Dizziness
  • Fainting (also called syncope)
  • Loss of energy
  • Swelling of the arms, legs, ankles or abdomen (also called edema)
  • Dry Cough
  • Raynaud’s phenomenon (chalky white or dusky blue fingers that may be painful and can sometimes be provoked by the cold)

In advanced stages of pulmonary hypertension, minimal activity may produce some or all of these symptoms.

Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out and difficulty breathing at rest. The exact cause of PAH is unknown and although treatable, there is no known cure for the disease. PAH usually affects women between the ages of 30-60.

Individuals with PAH may go years without a diagnosis, either because their symptoms are mild, nonspecific, or only present during demanding exercise. However, it is important to treat PAH because without treatment high blood pressure in the lungs causes the right heart to work much harder, and over time, this heart muscle may weaken or fail.

Treatment

If you have severe pulmonary hypertension, your doctor may prescribe medications called calcium channel blockers. These medicines lower blood pressure in the lungs and the rest of the body.

If calcium channel blockers aren’t enough, your doctor may refer you to a specialized treatment center. You may need more targeted therapies that can open up your narrowed blood vessels. They may be pills, inhalers, or drugs that are given through an IV.

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