ABOUT PH

CTEPH

Chronic thromboembolic pulmonary hypertension (CTEPH) is the result of multiple or recurrent blood clots in the lungs, also known as pulmonary emboli (PE). The clots and resulting scar tissue limit the blood flow to the arteries in the lungs, leading to pulmonary hypertension (PH), strain, and - eventually - failure of the right-side of the heart.

While all types of pulmonary hypertension share some common characteristics, there are certain differences that occur with CTEPH. It is not the same as other forms of PH, such as the more commonly known pulmonary arterial hypertension (PAH).

Chronic thromboembolic pulmonary hypertension is a type of pulmonary hypertension (WHO Group 4). It is caused by repeated blood clots or blood clots that don’t dissolve in the arteries of your lungs. These blood clots are called pulmonary emboli or PE.

Half of the patients with CTEPH don’t have symptoms or have never been diagnosed with pulmonary emboli. It is not well understood why some people are able to dissolve the pulmonary emboli inside their pulmonary arteries and others go on to develop chronic clot and scar tissue, even while taking blood thinners.

Here is a breakdown of the name:

Chronic - a condition that last a long time (months to years)
Thromboembolic - blood clots (thrombi) travel from your arms and or legs through the veins into the blood vessels of the lungs and block the pulmonary arteries
Pulmonary - related to the lungs
Hypertension - high blood pressure inside blood vessels

What is CTEPH?

The blood that flows through your lungs contains blood clots that travel from veins in legs or arms. These blood clots get stuck in the pulmonary arteries, causing them to become blocked or narrowed. Over time, these clots become scar tissue (also called webs) inside your pulmonary arteries.

This scar tissue restricts the blood flow through your pulmonary arteries. This causes the pressure inside your lung vessels to increase (pulmonary hypertension). The right side of your heart has to work harder to move blood through your narrowed pulmonary arteries.

Over time, blood from your pulmonary arteries backs up in the right side of your heart. As the blood backs up, your right ventricle becomes larger, and its muscle becomes thicker (right-sided heart failure). This leads to swelling in your abdomen and ankles from water retention (fluid build-up).

Without treatment, average life expectancy for CTEPH patients is five years.

What happens when you have CTEPH?

People diagnosed with chronic thromboembolic pulmonary hypertension often have:

A history of one or more blood clots in the lungs
High blood pressure at the time a blood clot is diagnosed
Pulmonary hypertension for at least 6 months after a pulmonary embolism (a blood clot in the lung) has been diagnosed

Common symptoms of CTEPH include:

Shortness of breath, especially during exercise or when climbing stairs
Fatigue (tiredness)
Swelling of the legs
Dizziness
Tightness in the chest or belly
Fainting
Palpitations (heart pounding in the chest)

CTEPH can be difficult to diagnose because it has symptoms similar to other diseases, including:

Asthma
Emphysema
Chronic obstructive pulmonary disease (COPD)
Obesity (overweight)
Heart failure

Symptoms of CTEPH?

About 75% of patients diagnosed with chronic thromboembolic pulmonary hypertension have had one or more blood clots in the lungs. Other risk factors include:

Having a high risk for blood clots
Chronic inflammatory diseases, such as osteomyelitis or inflammatory bowel disease
Having your spleen removed
Thyroid replacement therapy
Cancer
Family history of blood clots
Being female
Blood clotting disorders (hypercoagulability)

What are the risk factors for CTEPH?

Anybody with shortness of breath and an abnormal ventilation-perfusion scan (V/Q scan) should be referred to a CTEPH clinic.

A V/Q scan is a medical test that looks at how air and blood move through your lungs. This test can be done in any hospital. The result of this test is often the first step to finding out if you have CTEPH.

Who should be referred to a CTEPH clinic?

Chronic thromboembolic pulmonary hypertension is caused by blood clots that do not entirely dissolve following pulmonary embolism, leading to the formation of scars within the pulmonary arteries that impede blood flow into the lungs.

CTEPH is caused by blood clots that do not entirely dissolve following pulmonary embolism, leading to the formation of scars within the pulmonary arteries that impede blood flow into the lungs.

After pulmonary embolism, up to 4% of patients may develop CTEPH within two years.

How does a specialist know for sure whether or not I have CTEPH?

At a CTEPH clinic, you will do more medical tests. These tests may include:

Echocardiogram (ECHO) - an ultrasound of your heart
Electrocardiogram (EKG or ECG) - a recording of the electrical activity of your heart
CT scan of the chest - a special type of x-ray that looks at your lungs and blood vessels
Pulmonary angiogram - an intravenous (IV) dye is injected into your vein and travels to the vessels of your lungs. The dye helps us see blood flow and check for any blockages.
Femoral and carotid Doppler - an ultrasound of your leg and neck arteries
Pulmonary function test (PFT) - breathing tests
Right heart catheterization - a flexible catheter (tube) is put into your body through a vein in your groin area or wrist to measure the pressures of the right side of your heart and pressure in the pulmonary artery/lungs.
Left heart catheterization: a catheter is inserted through an artery, usually wrist or groin, and dye is injected to look for blockages in the arteries of the heart.
Exercise right heart catheterization - this is the same as a regular right heart catheterization except they will exercise you while laying back to see whether the pressure in your pulmonary artery/lungs increases with exercise. This is done when you do not have pulmonary hypertension at rest but still experience shortness of breath with any activity. This is currently only being done in Toronto where the CTEPH surgical centre is. Some of the referring provinces are working on offering this test, but it requires a cardiologist with specialty training to interpret and conduct.

These tests confirm whether you have CTEPH and help your health care team decide if surgery is the best treatment for you.

These screening and diagnosis guidelines will serve as a valuable resource to help you navigate your journey with CTEPH:

Download "Diagnosis of CTEPH: Patient Summary of the Canadian Thoracic Society Clinical Practice Guidelines”.

Visit our national directory of pulmonary hypertension centres

CTEPH Diagnosis

CTEPH is the only type of pulmonary hypertension that may be curable. Pulmonary thromboendarterectomy (PEA) is the treatment of choice for CTEPH. The pulmonary thromboendartectomy surgery leads to major clinical improvements due to improved hemodynamic parameters and oxygenation and reduction in dead space ventilation. The surgery can also be curative in many patients with resolution of pulmonary hypertension. Most patients experience significant functional recovery and can return to their baseline physical activity level without supplemental oxygen.

CTEPH patients are evaluated for PEA surgery at a CTEPH clinic. During your clinic visit, the thoracic surgeon and their team will check your health and look at all of your test results. This will help them decide if you are a candidate for pulmonary thromboendartectomy.

Learn more about the PEA surgery

If PEA surgery is not the best option for you, the CTEPH team will assess you to find other treatment options that may include:

The pulmonary hypertension medication riociguat (Adempas®) was approved in Canada in 2013 for the treatment of non-operative or residual post-operative CTEPH. Download an info sheet on riociguat for CTEPH
Balloon Pulmonary Angioplasty (BPA) is a procedure that is done by an interventional radiologist to dilate pulmonary arteries that are obstructed by the chronic scar tissue. Learn more about BPA.