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CTEPH - Chronic Thromboembolic Pulmonary Hypertension

Blood clots can cause serious health threats by blocking the flow of blood in the arteries, no matter where they develop or travel in the body. These include heart attacks when clots accumulate in the heart, or stroke when they travel to the brain.

A blood clot in the lungs is known as a pulmonary embolism, which can become lodged there and accumulate other clots. As blood backs up in the lungs and causes increased pressure, a rare but often treatable type of severe pulmonary hypertension known as chronic thromboembolic pulmonary hypertension (CTEPH) can develop.

While pulmonary emboli are quite common with 600,000 diagnosed each year, CTEPH is rare, affecting only two to four percent of those individuals. About 5,000 Americans are diagnosed with this condition each year.

Symptoms and Causes of CTEPH

The symptoms of chronic thromboembolic pulmonary hypertension are often similar to those of other cardiopulmonary conditions like pneumonia, heart failure or chronic obstructive pulmonary disease (COPD), so it often goes undiagnosed or is detected late in the advancement of the disease. The symptoms include:

  • Fatigue
  • Shortness of breath, especially with exercise
  • Chest pain
  • Swelling of the lower extremities or abdomen (edema)

This disease is typically caused by blood clots that develop in the lower extremities, which can be caused by long periods of inactivity, obesity, smoking or orthopaedic surgeries like joint replacement. In some cases, a period of only several hours of inactivity — such as a long flight — can cause a pulmonary embolism and trigger CTEPH.

This condition is more common in women and in those with a family history of blood clots.

The UPMC Treatment Approach

UPMC's Comprehensive Pulmonary Hypertension Program is one of only five centers programs nationwide specializing in treatment of CTEPH. Because this disease is so rare and has similar symptoms to other cardiopulmonary conditions, diagnosing and treating this disease requires the kind of multi-disciplinary expertise that is the hallmark of our physicians.

This disease is curable, and UPMC's experts are well-skilled in providing treatment options that are best suited for each person, even at advanced stages of disease. When properly treated, the survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.

Treatment options include:

  • Pulmonary endarterectomy (PEA) - In this surgical procedure, highly-skilled UPMC surgeons open the pulmonary arteries and remove the emboli, restoring proper blood flow to the lungs. Depending on how advanced the disease is, this surgery can cure up to 90% of all patients.
  • Medications - In the 10% of individuals who do not achieve a cure from PEA, medications can provide a secondary treatment option.
  • Lung transplantation - When surgery or medications fail to cure CTEPH, some people may become candidates for a lung transplant. Our physicians are leading experts in lung transplantation as part of the UPMC Thomas E. Starzl Transplantation Institute, a nationally regarded center of excellence in transplantation.

Schedule an Appointment

To schedule an appointment with the UPMC Comprehensive Pulmonary Hypertension Program team, call toll-free at
1-877-PH4-UPMC, or email PHprogram@upmc.edu.​​

Additional Resource

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