Chronic thromboembolic pulmonary hypertension (CTEPH) is increased blood pressure in the arteries in the lungs due to long-term blood clots. It is possible to treat the condition with surgery.

The condition occurs due to a chronic or long-term blood clot in the lungs called a pulmonary embolism. The blood clot or embolism obstructs the blood flow in the lungs, which increases the pressure in the arteries in the lungs.

Read on to learn more about CTEPH. This article discusses symptoms, causes, treatment options, and more.

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In the early stages of CTEPH, there may be no symptoms.

However, as the condition progresses, symptoms become similar to other types of pulmonary hypertension.

Typical symptoms include:

Learn about symptoms of pulmonary hypertension.

Researchers do not know exactly why some people with a blood clot develop CTEPH.

The condition most often follows pulmonary embolism. About 75% of people with CTEPH have had one or more blood clots in the lungs. This means that conditions that increase the risk of a pulmonary embolism also increase the risk of CTEPH.

CTEPH can also occur in people that do not have a known history of blood clots. A 2016 article reports that a small number of people with CTEPH do not have a history of pulmonary embolism.

Risk factors for developing CTEPH include:

  • long-term inflammatory conditions
  • having a spleen removed
  • family history of blood clots
  • thyroid replacement therapy
  • blood clotting conditions
  • cancer

Certain factors can also increase the chances of developing a pulmonary embolism, including:

In most cases, if there is an early diagnosis, it is possible to treat CTEPH with surgery.

Currently, the recommended treatment for CTEPH is pulmonary thromboendarterectomy (PTE). PTE is a surgical procedure that involves removing blood clots from the vessels in the lungs.

Not everyone with CTEPH is a candidate for surgery. More than 30% of people do not qualify for surgery.

If surgery is not an option, treatment can involve medical therapy to dilate the pulmonary arteries and balloon pulmonary angioplasty (BPA). BPA involves inflating a small balloon in the artery and temporarily inflating it.

Learn about recovering from a blood clot in the lungs.

Usually, the path to diagnosis of CTEPH involves a two-step process. The first part of the diagnostic process involves evaluating for pulmonary hypertension. The second step involves determining if blood clots cause elevated pulmonary pressure.

Possible diagnostic tests orders to confirm a diagnosis include:

  • Echocardiogram: An echocardiogram involves using sound waves to determine if the pressure on the right side of the heart is high.
  • Cardiac catheterization: This procedure allows doctors to measure the pressure in the pulmonary artery.
  • Ventilation-perfusion scan: This scan helps the doctor determine how efficiently air and blood move through the lungs.
  • Computed tomography pulmonary angiography: A CT pulmonary angiography creates images of the pulmonary arteries. These pictures help determine the extent and location of blood clots in the lungs.

CTEPH is a rare condition with an overall incidence of about 3–30 in every 30 million people in the general population.

It affects about 0.4–4.8% of all people with a pulmonary embolism.

Chronic thromboembolic pulmonary hypertension (CTEPH) refers to increased blood pressure in the arteries in the lungs. It often develops due to long-term blood clots in the lungs.

Symptoms may include trouble breathing, fatigue, and light-headedness. The preferred treatment involves surgery to remove the blood clots. Other treatments may be suitable if a person is unable to undergo surgery.

As early diagnosis can help improve the efficacy of surgery, it is best for a person with concerns about CTEPH to contact their doctor as early as possible.