We tend to think of high blood pressure as a problem that affects the heart. But in chronic thromboembolic pulmonary hypertension (CTEPH), the blood pressure in your lungs can rise, often to dangerous levels. While this relatively rare condition can be life threatening, there’s good news: CTEPH is the only type of pulmonary hypertension that can be completely resolved with surgery in some people.

CTEPH is Rare But Risky

High blood pressure in the blood vessels within your lungs is known as pulmonary hypertension. This problem can force the right side of your heart to work harder, which over time causes your heart to enlarge.

CTEPH is a form of pulmonary hypertension that can occur when a patient develops a chronic pulmonary embolism, where an embolism (clot) gets stuck in one of the lung’s blood vessels and turns into scar-like tissue. This can block blood flow within the lung, leading to symptoms such as chest pain, shortness of breath — especially during and after physical activity — and fatigue.

Pulmonary embolisms tend to form in people who have been sedentary for long periods of time due to illness, surgery, or even a long plane flight. Smoking, obesity, use of birth control pills, and conditions like heart disease and cancer can also increase your odds of experiencing a pulmonary embolism.

CTEPH Causes and Risk Factors

Having one or more pulmonary emboli during your lifetime is a major risk factor for developing CTEPH. You’re also more likely to develop CTEPH if you:

  • Have had your spleen removed
  • Have or have had cancer
  • Have a chronic inflammatory disease, such as inflammatory bowel disease
  • Have a family history of pulmonary hypertension
  • Are female
  • Are otherwise at high risk for developing blood clots

However, some people can develop CTEPH without having these or any other risk factors for the condition.

CTEPH Tests and Diagnosis

If your doctor suspects that you might have CTEPH, he or she may use one or more of the following tests to diagnose it:

  • Ventilation/Perfusion Scan or “V/Q Scan” – This test shows any areas in the lung that are not receiving the appropriate amount of blood flow due to blockage of the arteries by clots.
  • Echocardiogram – Also known as a heart sonogram, this painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning.
  • CT scan or CT angiogram – This test is to look for blood clots and to evaluate the condition of the rest of the lung besides the arteries.
  • Pulmonary angiography – X-rays are used to show blood flow through the lungs.
  • Heart catheterization – This test measures the pressure inside the arteries.

When properly treated, people with CTEPH can live as long as those without any history of pulmonary hypertension.

CTEPH Treatment Options

  • Pulmonary thromboendarterectomy (PTE) – Surgeons open the pulmonary arteries and remove the clot, restoring proper blood flow to the lungs. Depending on how advanced the disease is, this surgery can cure up to 90 percent of all patients.
  • Medications – In the 10 percent of people for whom PTE doesn’t work, medications can provide a secondary treatment option.
  • Lung transplant When surgery or medications fail to cure CTEPH, some people may become candidates for a lung transplant.

Your doctor can tell you more about these options and answer any questions you have about CTEPH.

To learn more about CTEPH or to schedule an appointment with a CTEPH specialist, call  412-648-6161, or toll-free at  1-877-PH4-UPMC, or email PHprogram@upmc.edu.