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Chronic Thromboembolic Pulmonary Hypertension (CTEPH)​​​​

What Is CTEPH?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition caused by blood clots in the lungs. 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.
  • Stroke when they travel to the brain.

What is a pulmonary embolism?

A blood clot in the lungs, 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 2-4 percent of those people. About 5,000 Americans are diagnosed with this condition each year.

CTEPH Symptoms and Diagnosis

Chronic thromboembolic pulmonary hypertension (CTEPH) symptoms 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.

CTEPH symptoms

The symptoms of CTEPH include:

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

CTEPH 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.

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

CTEPH diagnosis

Doctors diagnose chronic thromboembolic pulmonary hypertension (CTEPH) using common tests, including:

  • Ventilation/perfusion scan or "V/Q scan"
  • Echocardiogram
  • CT scan or CT angiogram
  • Right-heart catheterization
  • Pulmonary angiography
  • IVC filter placement
  • Coronary angiography or left heart catheterization
  • Carotid Doppler

CTEPH Treatment

UPMC's Comprehensive Pulmonary Hypertension Program is one of only five centers programs nationwide specializing in the treatment of CTEPH.

Because this disease is so rare and has similar symptoms to other cardiopulmonary conditions, diagnosing and treating CTEPH 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 the disease. When properly treated, the survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.

Treatment Options

Pulmonary Thromboendarterectomy (PTE)

Also called Pulmonary endarterectomy (PEA). In this complex surgical procedure performed with cardiopulmonary bypass, the patient’s body temperature is cooled and cessation of blood flow is sustained for a short period of time to allow for complete visualization and removal of the chronic pulmonary emboli. The goal is to restore normal blood flow to the lungs and prevent heart failure and death. This surgery can potentially cure CTEPH, leaving the patient without emboli and without the need for pulmonary vasodilator therapy. The success of the surgery depends on the combination of proper patient selection as well as surgical expertise and technique. PTE is considered safe with an experienced team.

Balloon Pulmonary Artery Angioplasty (BPA)

For patients with inoperable CTEPH or those with residual pulmonary hypertension after surgery, this minimallyinvasive procedure might be an option. UPMC is one of very few centers in the country offering this treatment. The technique for BPA is similar to a right-heart catheterization, where a catheter is introduced into the pulmonary artery with a balloon at its tip. The balloon is inflated in the area where the chronic blood clots are located, pushing the clots against the wall of the artery and restoring blood flow. BPA can complement surgery and/or medical therapy to improve the quality of life of CTEPH patients.

Medications

For patients who do not achieve a cure from PTE or are not surgical candidates, medications are a treatment option. 

Lung Transplantation 

Some patients may be 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 recognized center of excellence in transplantation. 

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