Chronic thromboembolic pulmonary hypertension (CTEPH) is rare, serious and treatable. This form of pulmonary hypertension is classified by the World Health Organization as Group 4 and occurs when blood vessels in the lung have been blocked by a clot for an extended period of time.

Key Facts

  • CTEPH is a rare disease with up to 3,000 new cases diagnosed annually in the United States and occurs equally in men and women.
  • If you have been diagnosed with pulmonary hypertension, your healthcare provider should rule out CTEPH to make sure you are getting the correct treatment.
  • If you have a history of blood clots in your legs and/or your lungs and have new onset of shortness of breath, you should alert your healthcare provider immediately.

What Causes CTEPH

  • CTEPH is a rare and progressive form of pulmonary hypertension which can develop when chronic pulmonary embolism, or blood clots, form scar-like tissue in the lung’s arteries, blocking or narrowing the arteries.
  • You may be at increased risk for CTEPH if you:
    • Have a history of one or more blood clots in the lungs
    • Have high blood pressure at the time a blood clot is diagnosed
    • Have pulmonary hypertension for at least 6 months after a blood clot in the lung has been diagnosed

How CTEPH Affects Your Body

CTEPH results in blood clots getting stuck in the lungs, clogging the arteries, and causing the formation of scar-like tissue. As a result, the right side of your heart has to work harder. This increased work for the heart can result in heart failure. The oxygen level in the blood can also drop too low because of the increased time it takes for blood to travel through the lungs. This can result in damage to your cells, tissues, and organs which all need oxygen in order for you to be healthy. Without treatment, CTEPH can be fatal.

Page last updated: September 25, 2023

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