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What is CTEPH?

CTEPH is a type of pulmonary hypertension (PH). It is caused by repeated blood clots or blood clots that don’t dissolve in the arteries of your lungs. These blood clots are called pulmonary emboli or PE.
Half of the patients with CTEPH don’t have symptoms or have never been diagnosed with pulmonary emboli. It is not well understood why some people are able to dissolve the pulmonary emboli inside their pulmonary arteries and others go on to develop chronic clot and scar tissue, even while taking blood thinners. 
Here is a breakdown of the name:
Chronic - a condition that last a long time (months to years)
Thromboembolic - blood clots (thrombi) travel from your arms and or legs through the veins into the blood vessels of the lungs and block the pulmonary arteries
Pulmonary - related to the lungs
Hypertension - high blood pressure inside blood vessels

What is CTEPH? | Dr. Marc de Perrot

What happens when you have CTEPH?

Diagram of heart and lungs pointing to each part; right and left lungs, Right Atrium, Left Atrium, Right Ventricle, and Left Ventricle

Diagram of heart and lungs pointing to each part; right and left lungs, Right Atrium, Left Atrium, Right Ventricle, and Left Ventricle
  • The blood that flows through your lungs contains blood clots that travel from veins in legs or arms. These blood clots get stuck in the pulmonary arteries, causing them to become blocked or narrowed.
  • Over time, these clots become scar tissue (also called webs) inside your pulmonary arteries.
  • This scar tissue restricts the blood flow through your pulmonary arteries. This causes the pressure inside your lung vessels to increase (pulmonary hypertension).
  • The right side of your heart has to work harder to move blood through your narrowed pulmonary arteries.
  • Over time, blood from your pulmonary arteries backs up in the right side of your heart.
  • As the blood backs up, your right ventricle becomes larger, and its muscle becomes thicker (right-sided heart failure). This leads to swelling in your abdomen and ankles from water retention (fluid build-up).
  • Without treatment, average life expectancy for CTEPH patients is five years.

What causes CTEPH?

Some common causes of CTEPH include:
  • A history of one or more blood clots in the lungs
  • High blood pressure at the time a blood clot is diagnosed
  • Having PH for at least 6 months after a PE (a blood clot in the lung) has been diagnosed

Symptoms of CTEPH?

CTEPH can be difficult to diagnose because it has symptoms similar to other diseases, including:
  • Asthma
  • Emphysema
  • Chronic obstructive pulmonary disease (COPD)
  • Obesity (overweight)
  • Heart failure

Common symptoms of CTEPH include:
  • Shortness of breath, especially during exercise or when climbing stairs
  • Fatigue (tiredness)
  • Swelling of the legs
  • Dizziness
  • Tightness in the chest or belly
  • Fainting
  • Palpitations (heart pounding in the chest)

What are the risk factors for CTEPH?

About 75% of patients diagnosed with CTEPH have had one or more blood clots in the lungs. Other risk factors include:
  • Having a high risk for blood clots
  • Chronic inflammatory diseases, such as osteomyelitis or inflammatory bowel disease
  • Having your spleen removed
  • Thyroid replacement therapy
  • Cancer
  • Family history of blood clots
  • Being female
  • Blood clotting (called hypercoagulability) disorders

Who should be referred to a CTEPH clinic?

Anybody with shortness of breath and an abnormal ventilation-perfusion scan (V/Q scan) should be referred to a CTEPH clinic.

A V/Q scan is a medical test that looks at how air and blood move through your lungs. This test can be done in any hospital. The result of this test is often the first step to finding out if you have CTEPH. 

CTEPH Guidelines for patients

Whether you're newly diagnosed or managing your condition, these guidelines serve as a valuable resource to help navigate your journey with CTEPH. Click the link below to access the comprehensive document containing guidelines designed to support you in understanding and managing CTEPH effectively.

Click here to open/download the "CTEPH Guidelines for patients" document.