Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

CTEPH is a rare and progressive form of pulmonary hypertension caused when chronic pulmonary embolism, or blood clots, form scar-like tissue in the lung’s arteries, blocking or narrowing the arteries.

Learn About CTEPH

Chronic thromboembolic pulmonary hypertension (CTEPH) is rare, serious and treatable. This form of pulmonary hypertension is classified by the World Health Organization as Group 4 and occurs when blood vessels in the lung have been blocked by a clot for an extended period of time.

Find Out What the Experts Are Saying

Learn what our panel of experts recommended after convening an in-person PH Roundtable Meeting to review the updates to the ESC/ERS 2022 Diagnostic and Treatment Guidelines of Pulmonary Hypertension. View Guidance to the Guidelines 

Key Facts

• CTEPH is a rare disease with up to 3,000 new cases diagnosed annually in the United States and occurs equally in men and women.
• If you have been diagnosed with pulmonary hypertension, your healthcare provider should rule out CTEPH to make sure you are getting the correct treatment.
• If you have a history of blood clots in your legs and/or your lungs and have new onset of shortness of breath, you should alert your healthcare provider immediately.

What Causes CTEPH

• CTEPH is a rare and progressive form of pulmonary hypertension which can develop when chronic pulmonary embolism, or blood clots, form scar-like tissue in the lung’s arteries, blocking or narrowing the arteries.
• You may be at increased risk for CTEPH if you:
  • Have a history of one or more blood clots in the lungs
  • Have high blood pressure at the time a blood clot is diagnosed
  • Have pulmonary hypertension for at least 6 months after a blood clot in the lung has been diagnosed

How CTEPH Affects Your Body

CTEPH results in blood clots getting stuck in the lungs, clogging the arteries, and causing the formation of scar-like tissue. As a result, the right side of your heart has to work harder. This increased work for the heart can result in heart failure. The oxygen level in the blood can also drop too low because of the increased time it takes for blood to travel through the lungs. This can result in damage to your cells, tissues, and organs which all need oxygen in order for you to be healthy. Without treatment, CTEPH can be fatal.

Symptoms and Diagnosis of CTEPH - What Are the Symptoms of CTEPH?

The symptoms of CTEPH are frequently nonspecific and similar to many other cardiopulmonary diseases such as asthma, chronic obstructive pulmonary disease, or heart failure. The most common symptoms may include increased shortness of breath with exertion such as climbing stairs and an increased intolerance to exercise. Other symptoms may include:

• Swelling in your abdomen
• Swelling in your legs
• Dizziness or feeling lightheaded
• Heart pounding (palpitations)
• Fatigue
• Tightness in the chest or stomach
• Coughing up blood, also called hemoptysis.

When To See Your Healthcare Provider

CTEPH can be hard to diagnose due to presenting with symptoms similar to other diseases. It is important to see your healthcare provider when noticing unexplained shortness of breath and fatigue. Alert your healthcare provider if you have a history of blood clots. Prompt treatment for CTEPH is important in order to have the most positive health outcomes.

How CTEPH Is Diagnosed

Your healthcare provider will need to order several tests to confirm that you have CTEPH. In order to be diagnosed with CTEPH, you first need to have a diagnosis of pulmonary hypertension. 

• Ventilation-perfusion (V/Q) Scan: Measures how well air and blood flow into your lungs. Considered the best screening test for diagnosing CTEPH.
• Right Heart Catheterization: This test is invasive, so it is not usually performed unless other tests cannot produce a firm diagnosis. It involves inserting a catheter (small tube) into a large vein in either the neck, arm, or groin, and threading it through the right side of the heart and into the pulmonary artery. This allows measurement of the blood pressure in the lungs.
• Lung function tests (Breathing tests): Checks for diseases like asthma or COPD.
• Echocardiogram: Ultrasound of the heart to check the size and condition of the chambers of the heart. It can also be used to estimate the blood pressure in the pulmonary arteries.
• Pulmonary Angiogram: A dynamic X-ray of the blood vessels in your lungs using contrast injection.
• Computed Tomography (CT or CAT) Scan Pulmonary Angiograph: Diagnostic imaging that shows cross-section images of your lungs using contrast to outline your blood vessels.
• Magnetic Resonance Imaging (MRI): Non-invasive detailed imaging of your lungs that uses magnets and radio waves.
• 6-Minute Walk Test: Objectively measures how far you can walk and to see if your oxygen levels drop when you are physically active.

Treating and Managing CTEPH - How CTEPH Is Treated 

The primary treatment method for CTEPH is a surgical procedure known as pulmonary thromboendarterectomy (PTE), also referred to as pulmonary endarterectomy (PEA). This is considered the most effective treatment for CTEPH; however more than one-third of patients with CTEPH are not good candidates for this treatment option. 

Pulmonary Thromboendarterectomy (PTE) 

PTE is a surgical procedure that involves the removal of all of the clots from the arteries in your lungs. PTE results in significant improvement for patients that have been diagnosed with CTEPH and some patients may be cured. Not all patients with CTEPH are good candidates for this treatment intervention. Patients who have other medical conditions, or patients with distal lesions that are difficult to reach with surgery may not be candidates for this procedure. 

Balloon Pulmonary Angioplasty (BPA) 

If you are unable to tolerate PTE, your healthcare provider may recommend balloon pulmonary angioplasty (BPA). This procedure involves the use of tiny balloons that are inflated inside the pulmonary artery to widen it. This decreases the pulmonary arterial pressure so that the right side of your heart does not have to work so hard. 

Lung Transplant 

A lung transplant is rarely needed; however, it may be recommended for patients with advanced CTEPH when other treatment options are not working. Your healthcare provider will decide if a lung transplant is right for you. 

Medications 

Medication management may be recommended by your healthcare provider if you are not a candidate for surgery or if you continue to have pulmonary hypertension after having surgery. Medication therapy will help in opening, or dilating, your pulmonary arteries so that the heart does not have to work so hard. Your healthcare provider will usually recommend treatment with anticoagulants, even if you have had successful surgery. Anticoagulants, or blood thinners, will keep new blood clots from forming. 
Oxygen therapy may be prescribed if you are not getting enough oxygen. Your healthcare provider will decide if supplemental oxygen is right for you. 

For More Information:

• Pulmonary Hypertension Association
• 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Living with CTEPH: A Personal Story

Diagnosed in 2012 with a rare type of pulmonary hypertension, Dolores shares a new life purpose – raising awareness about the symptoms, diagnosis and treatment of CTEPH. Read the full story

Newly Diagnosed with CTEPH? - Take These Important First Steps

• If you have been diagnosed with CTEPH, learn more about what can cause CTEPH, what your treatment options are and how to best manage your disease.
• It will be important for you to work closely with your healthcare provider to determine what treatment course is right for you as soon as possible. Get an appropriate referral. Because CTEPH is such a rare disease, it is extremely valuable to get a referral to a specialist at an expert CTEPH center to ensure you are getting the most up-to-date treatment options. 

Take Care of Yourself 

• Pulmonary Rehabilitation: Ask your healthcare provider if pulmonary rehabilitation (PR) is right for you. PR provides a structured environment for learning what exercise is right for you, the important role of nutrition in your health, learning how to manage your medications, and more.
• Nutrition: Good nutrition is important in managing your CTEPH. Talk to your healthcare provider about what foods are good for you and what your ideal weight should be.
• Medications: You may be on new medications to help manage your CTEPH. It is important to make sure that you are taking your medications as prescribed in order to get the maximum benefit. If your healthcare provider has prescribed oxygen therapy, make sure you know how to correctly use your oxygen delivery device. 
• Exercise: Talk to your healthcare provider about what types of exercise are right for you.  It is important to strengthen your muscles and work on breathing techniques that can improve your quality of life.
• Healthy Lungs: Quitting tobacco smoking is important to your lung health.  You do not have to quit alone.  You can find programs, resources and support at Quit Smoking. Talk to your healthcare provider about what vaccinations you should have to best protect your lungs.
• Connect With Others: No person facing a chronic lung disease should go through it alone. There are online and in-person support groups available. Finding a support group early in your journey will provide you with more opportunities to get information about your disease, empower you to better manage your disease, and give you the support you need.
  • Living With Lung Disease (online support community) 
  • Better Breathers Clubs (in-person and virtual support groups)

Source: https://www.lung.org/lung-health-diseases/lung-disease-lookup/cteph