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Chronic Thromboembolic Pulmonary Hypertension — An Overview

Posted on September 13, 2021
Medically reviewed by
Steven C. Pugliese, M.D.
Article written by
Emily Wagner, M.S.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots. When the clots form in the lungs, they cause pressure to build in the lungs’ arteries. This pressure then leads to symptoms such as weakness, shortness of breath, and chest pain.

There are around 5,000 new cases of CTEPH diagnosed per year in the U.S. The condition can be difficult to diagnose, and the actual number of new cases may be much higher than what is estimated.

Causes of CTEPH

CTEPH is caused by blood clots that get stuck in the arteries of the lungs, known as the pulmonary arteries. These clots block blood flow through the arteries, causing the flow to back up and build pressure. Clots can also cause scar tissue and lesions in the arteries, which can narrow the blood vessels and further limit blood flow.

Risk Factors for CTEPH

Anyone can develop CTEPH, but there are certain factors that put you at a higher risk. If you have blood clots in the lungs, known as acute pulmonary embolisms (PEs), you are at a higher risk. Roughly 75 percent of people with CTEPH have had at least one blood clot before. However, you can still develop CTEPH without ever having a PE.

Risk factors for acute PEs include:

  • Being older
  • Having long periods of inactivity, such as recovering from surgery or illness, or sitting on long car trips or plane rides
  • Being obese
  • Having had previous surgeries, particularly joint replacement surgery
  • Smoking
  • Taking birth control that contains estrogen

Other underlying conditions and treatments that cause blood clots can also lead to CTEPH. These include blood diseases, cancers, and inflammatory diseases. Other risk factors are:

  • Being female
  • Having a disorder that interferes with blood clotting, like antiphospholipid and lupus anticoagulant syndromes
  • Having a family history of blood clots
  • Having Type A, Type B, or Type AB blood
  • Undergoing a spleen removal
  • Undergoing thyroid hormone replacement therapy

Symptoms of CTEPH

The main symptoms of CTEPH are shortness of breath (either while resting or exercising), fatigue, and weakness. Other symptoms include:

  • Fainting
  • Pounding or pain in the chest, known as palpitations
  • Swelling in the legs and feet (edema)
  • Coughing up blood, in rare cases

The symptoms of CTEPH are similar to other diseases that affect the heart and lungs, including asthma, chronic obstructive pulmonary disease (COPD), and emphysema. This can make the condition difficult to diagnose.

How Is CTEPH Diagnosed?

If your doctor thinks you might have CTEPH, they will order a number of tests to look at how your lungs and heart function. These include a CT scan, a lung ventilation-perfusion scan (also known as a lung V/Q scan), and other tests. A diagnosis of CTEPH is serious and needs to be addressed as quickly as possible.

CTEPH is diagnosed when there is a mean pulmonary artery pressure of 25 millimeters of mercury (written as "mm Hg," a medical unit used to describe pressure) or more after the presence of a PE.

Lung Ventilation-Perfusion Scan

A lung ventilation-perfusion scan lets doctors see how air and blood move through your lungs. There are two procedures for this scan: one for the ventilation portion of the scan, and another for the perfusion portion of the scan.

For the ventilation portion, you breathe in a gas mixture with a radioactive tracer. The tracer helps your doctor visualize the air moving through your lungs.

For the perfusion portion, a liquid is mixed with a radioactive tracer and injected into a vein to flow through your bloodstream. The tracer can be visualized using special X-rays. It will absorb in areas where there is a lot of blood flow, and it will not absorb in areas with little blood flow. The areas with little blood flow — where the tracer is not absorbed — most likely contain blood clots.

CT Scan

CT scans can also be used to visualize the lungs. This technique uses special X-rays to take detailed pictures of the chest area, including your heart and lungs, to look for blood clots.

Pulmonary Angiogram

A pulmonary angiogram is another test that helps doctors look at the blood flow through the lungs. A special dye is injected into arteries and the right side of your heart using an intravenous catheter (a thin, hollow tube). Using X-ray scans, a doctor will be able to look for blood clots.

Echocardiogram

An echocardiogram uses sound waves similar to an ultrasound to create pictures of the heart. This lets your doctor see your heart pumping and the blood flow in real time. To diagnose CTEPH, your doctor will perform a transthoracic echocardiogram that specifically looks at the chest and heart.

Right Heart Catheterization

Your doctor will perform a right heart catheterization to check the pressure in your lungs and heart directly. A catheter is inserted into the pulmonary artery on the right side of your heart. As the catheter moves through the artery, it takes pressure and blood flow measurements.

Pulmonary Function Tests

Pulmonary function tests measure how well your lungs function. They measure several things, such as how much air your lungs can hold, how well air flows through them, and more.

Pulmonary function tests are noninvasive and can be done using special instruments that only require you to breathe in and out. One example is spirometry, where you breathe into a mouthpiece connected to a machine that measures your lung function.

Treatments for CTEPH

If you receive a CTEPH diagnosis, your doctor will discuss your treatment options with you. These can range from surgery to noninvasive methods like medication. Your treatment plan will depend on your exact case of CTEPH, your overall health, and any underlying conditions you may have.

Fortunately, it is possible to cure CTEPH if it is diagnosed early and treated properly.

Pulmonary Thromboendarterectomy

A pulmonary thromboendarterectomy (PTE), also known as a pulmonary endarterectomy (PEA), is a surgery used to treat CTEPH. According to the American Thoracic Society, the PTE “is currently the recommended and only effective treatment for patients with CTEPH.”

While it is the preferred treatment for people with CTEPH, a PTE is not recommended for everyone. For those who are not candidates, there are other options that are less intensive than surgery, such as anticoagulation therapy.

Balloon Pulmonary Angioplasty

A balloon pulmonary angioplasty (BPA) is a less invasive method for treating CTEPH. People who cannot undergo surgery for a PTE may be eligible for a BPA.

During a BPA, a catheter is inserted through a vein in your thigh and guided up to the pulmonary arteries. Once the catheter reaches the artery, a small balloon attached to the catheter is blown up, causing the artery to expand. This will help blood flow through the artery more easily and can relieve some of the increased pressure.

Anticoagulant Therapy

There is currently only one medication approved for the treatment of CTEPH. Adempas (riociguat) is an anticoagulant, which prevents blood clots from forming. This medication is given to people who cannot have surgery or to those who have high blood pressure that continues after surgery.

Lung Transplantation

A lung transplant is a treatment option for people who are not candidates for other surgeries, such as a PTE or BPA. With this procedure, your lungs are removed and another set of lungs from a donor are placed inside of you.

Prognosis With CTEPH

While it is a rare condition, CTEPH has many treatment methods that can help people live longer. However, if CTEPH is not treated and continues to progress, the life expectancy for people with CTEPH is one to three years.

Surgery offers the best chance at living a healthier and longer life with CTEPH. One study found that a PTE improves three-year survival rates to 89 percent, compared to only 71 percent for people who did not have surgery.

As research continues to find new medications and other treatments, the life expectancy and prognosis for those living with CTEPH may improve.

Talk With Others Who Understand

On myPHteam, more than 37,800 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Are you or a loved one living with CTEPH? Share your experience in the comments below, or start a conversation by posting on your Activities page.

All updates must be accompanied by text or a picture.
Steven C. Pugliese, M.D. is affiliated with the Hospital of the University of Pennsylvania in Philadelphia, serving as the director of the pulmonary embolism response team, co-director of the comprehensive pulmonary embolism program, and an assistant professor of clinical medicine. Review provided by VeriMed Healthcare Network. Learn more about him here.
Emily Wagner, M.S. holds a Master of Science in biomedical sciences with a focus in pharmacology. She is passionate about immunology, cancer biology, and molecular biology. Learn more about her here.

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