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Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Program

The Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Program is dedicating to identifying, diagnosing, and treating patients with CTEPH disease. Because many people with chronic thromboembolic pulmonary hypertension don't know they have it, one of our main goals is to identify patients who have a higher risk (chance) of developing CTEPH.

We are one of the only comprehensive CTEPH treatment programs in the country. We serve patients throughout the Mountain West and are the only CTEPH program within 800 miles of Salt Lake City.

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What Is Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease that causes pressure to build in the arteries inside your lungs. About four percent of patients with pulmonary embolism will develop CTEPH within two years of a blockage.

CTEPH is a serious disease that becomes worse over time. If left untreated, CTEPH can cause fatigue, chest pain, and shortness of breath. In later stages, CTEPH can cause fainting and heart failure symptoms.

Because CTEPH can cause heart attack-like symptoms, patients with CTEPH have poor health and low quality of life. Surgery is the best option for treating CTEPH.

How Common is CTEPH?

CTEPH affects 1 in 1000 people in the United States. Anyone can develop this disease.

About four percent of people who have pulmonary embolism will develop CTEPH. Pulmonary embolism is when a blood clot develops inside a blood vessel and then travels to a lung artery and blocks blood flow. But as many as half of all people with CTEPH have no history of pulmonary embolism.

Cardiology experts know that CTEPH is underdiagnosed. Fortunately, CT scans that look for pulmonary embolism are slowly helping cardiologists find and diagnose more patients with CTEPH.

Hard to Diagnose

Because CTEPH is relatively rare, many providers and patients have never heard of this disease. This makes CTEPH hard to diagnose. Even more challenging, many patients with CTEPH have no symptoms. Other patients have symptoms that are easily mistaken for other common illnesses.

Many patients with CTEPH have no history of pulmonary embolism, which is one of the risk factors. Patients with deep venous thrombosis (DVT) can also develop CTEPH. But many patients with CTEPH do not have DVT.

Cardiologists are working to better identify and diagnose people with pulmonary embolism. Cardiologists hope that by identifying people with pulmonary embolism, they will also be able to identify patients who have a higher chance of developing CTEPH.

What Are the Symptoms of CTEPH?

CTEPH's early symptoms can be easily confused with many other diseases. Many people with CTEPH do not have any symptoms for a long time.

Early Stage Symptoms 

In the early stages of CTEPH, symptoms can easily be confused for other common problems. Symptoms include: 

  • fatigue,
  • chest pain, and
  • shortness of breath (especially during exercise).

These common symptoms make it hard for both patients and doctors to suspect CTEPH.

Late Stage Symptoms

If a patient has CTEPH for many years, symptoms are more severe and life-threatening. Symptoms can include signs of heart failure, including:

  • fainting
  • edema (when your body swells and retains fluids), and
  • a blue color in the fingers and toes.

Treatment for CTEPH

Surgery is the best treatment option for CTEPH. Patients who have surgery have better health outcomes than patients who do not. Over 89 percent of patients who have surgery are still alive three years after being diagnosed.

For patients who do not have surgery, just over 70 percent are alive three years after being diagnosed. Patients with CTEPH have the best chance of becoming healthy again if they have surgery.

Our cardiology specialists will perform many tests, including imaging and evaluation, to determine if you are a good candidate for surgery.

If you are not a strong candidate for surgery, our doctors will discuss non-surgical medical treatments including drugs and lab procedures.

Treating CTEPH With Surgery 

To treat CTEPH, cardiovascular surgeons perform a type of surgery called pulmonary endarterectomy or PEA. The goals of PEA surgery are to:

  • decrease heart failure symptoms like edema,
  • decrease shortness of breath, and
  • increase survival rates.

PEA surgery decreases pulmonary vascular resistance. Decreasing this resistance helps push and circulate blood through your lungs. Surgery also improves ventilation and perfusion matching, which help regulate the amount of air and blood delivered to your lungs.

PEA is a complex, demanding surgery. Surgeons who perform PEA must have specialist training and sophisticated care management skills.

Even though CTEPH surgery is complex, it is very successful. The survival rate for PEA is over 95 percent.

What Happens During PEA Surgery?

During a PEA procedure, surgeons clear all thromboembolic material that is blocking blood flow in your pulmonary arteries. Surgeons open your chest through your sternum. The surgery lasts eight to 10 hours.

Surgeons first make small incisions in the left and right pulmonary arteries one at a time. Surgeons then carefully remove the old blood clots that are compressed (or squeezed) against the blood vessel wall. These blood clots often have more fibrous connective tissue than normal. Surgeons carefully dissect these old blood clots.

Cooling Your Body

The blood vessels in your lungs deliver blood to your brain. Your surgical team must make sure they can safely operate on these important blood vessels. To do this, surgeons use a technique called deep hypothermic circulatory arrest so they can safely operate on the large blood vessels inside the lungs that deliver blood to your brain.

During hypothermic circulatory arrest, your body will be cooled to 20 degrees Celsius. The surgical team will place you on a heart-lung bypass machine to protect your body's organs and support your brain. Your heart will also stop temporarily, but your surgical team will carefully and safely monitor and control your heart's activity.

After Surgery

After surgery, patients have a risk of developing the same complications as traditional heart surgery. Patients also have additional risks like neurological problems and reperfusion lung injury. This injury causes tissue damage in your lungs when blood returns to your lungs after they have not received oxygen for a while.

You will need to stay in the intensive care unit so hospital staff can monitor for signs of these unique complications.

How Many People Have PEA Surgery?

Because CTEPH is underdiagnosed, medical centers in the US perform fewer PEA surgeries than medical centers in Europe. Cardiologists perform almost twice as many PEA procedures in Europe each year.

The US has few comprehensive CTEPH treatment centers. Many patients with CTEPH do not receive the referrals they need.

Treating CTEPH Without Surgery

Surgery is the most successful way to treat CTEPH disease. But patients have a few different options for treating CTEPH without surgery.

Riociguat is an oral drug that makes it easier for you to exercise, improving your overall quality of life. Riociguat is best for patients who cannot have PEA surgery, or for patients who have reoccurring pulmonary hypertension after PEA surgery.

Balloon Pulmonary Angioplasty

Balloon pulmonary angioplasty is a non-surgical alternative to PEA surgery. Patients have this procedure in outpatient cardiology labs. Cardiologists use small balloons to dilate (or open) blocked blood vessels. 

Patients will need many sessions to open blocked blood vessels. Most patients need an average of five sessions.

Balloon pulmonary angioplasty may be best for frail and elderly patients with CTEPH.

Multidisciplinary Care

We rely on the expertise of doctors in many specialties, including imaging, anesthesiology, cardiovascular medicine, pulmonary medicine, and cardiothoracic surgery. Our multidisciplinary collaboration helps patients with CTEPH live longer and have a better quality of life.

Contact Us

For more information, call us 801-585-7676, option 1 or option 4.

For Our Patients

Treating CTEPH Without an Organ Transplant

CTEPH is a serious lung condition that can arise after an individual suffers a pulmonary embolism. Luckily for patients, there is a surgery available that can treat CTEPH without a full lung transplant. Heart surgeon Dr. Craig Selzman explains the steps involved in the six-hour "roto-rooter operation.

Listen/Read (9 min.)