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Pulmonary Arterial Hypertension Symptoms, Causes and Risk Factors

What Are the Symptoms of Pulmonary Arterial Hypertension?

The symptoms of pulmonary arterial hypertension are similar to the symptoms often seen in more common diseases, such as asthma, emphysema or chronic obstructive pulmonary disease (COPD), and heart failure. Symptoms include:

  • Shortness of breath. At first with movement, but then at rest as the disease worsens.
  • Fatigue
  • Swelling of the feet, legs, belly and neck.
  • Chest pain, pounding of the heart and fainting are less common but can occur in very advanced disease.
  • Rarely, patients will cough up blood or have a change in their voice.

What Causes Pulmonary Arterial Hypertension?

PAH is characterized by progressive scarring of the tiny blood vessels going to the lungs. A number of diseases and conditions can cause this scarring including: connective tissue disorders like scleroderma and lupus; exposure to certain toxins and drugs, including methamphetamine and cocaine; infections, including HIV and schistosomiasis; cirrhosis of the liver; and congenital heart abnormalities. When the cause of PAH is unknown, it is called idiopathic pulmonary arterial hypertension.

What Are Risk Factors?

You are more likely to get pulmonary arterial hypertension if you are a young female, since the idiopathic form of the disease occurs more often in women of childbearing age than in men. There is an inherited form of PAH, so a family history of the disease may put you at increased risk.  Patients with diseases like lupus, scleroderma, cirrhosis of the liver, and HIV infection can develop it as well. Methamphetamine and cocaine use increase the risk for developing this disease.

When to See Your Doctor

If you have shortness of breath that does not improve with therapy for common diseases such as asthma and COPD; fainting or chest pain with exertion; unexplained swelling in your legs or abdomen and profound fatigue; you should contact your healthcare professional. If you have been diagnosed with PAH, you should find a specialist who deals with this disease.

    This content was developed in partnership with the CHEST Foundation, the philanthropic arm of the American College of Chest Physicians.

    Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel. Last reviewed August 4, 2016.

    Page Last Updated: March 13, 2018

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