What is chronic thromboembolic pulmonary hypertension (CTEPH)?
Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the arteries in your lungs (pulmonary arteries). There are about 5,000 people who are diagnosed with CTEPH every year in the United States. But, some people with CTEPH do not get the right diagnosis.
Who is at risk of getting chronic thromboembolic pulmonary hypertension?
Anyone can have CTEPH, although about 75% of patients with CTEPH have a history of blood clots in their lungs (pulmonary embolisms [PEs]).
What causes chronic thromboembolic pulmonary hypertension?
Chronic thromboembolic pulmonary hypertension is caused by clots and scar tissue from the clots in the pulmonary arteries. These cause blockages in the arteries that make it harder for blood to flow through. This increases the pressure in the arteries.
Risk factors for a PE include:
- Being still or inactive for a long period of time, including being laid up to due illness or not moving enough when traveling.
- Surgery, especially joint replacement surgery.
- Getting older.
- Obesity.
- Taking birth control pills that contain estrogen.
- Pregnancy and the period of time right after pregnancy.
- Smoking.
- Diseases like cancer, blood diseases and inflammatory diseases.
Your risk of CTEPH is higher if you:
- Have an unexplained or large PE.
- Do not have a spleen.
- Are on thyroid replacement therapy.
- Have a blood clotting disorder, such as lupus anticoagulant or antiphospholipid syndrome.
- Have an infected pacemaker.
- Do not have Type O blood.
What are the symptoms of chronic thromboembolic pulmonary hypertension?
Symptoms of a PE are:
- Being short of breath, especially when you exercise.
- Feeling tired or weak.
- Pain in your chest or heart palpitations (can feel like a pounding heartbeat or skipped beats/ fluttering).
- Swelling in your legs.
- A blue tint in your fingers/toes (cyanosis).
- Feeling lightheaded or fainting.
How is chronic thromboembolic pulmonary hypertension diagnosed?
If your doctor thinks you may have CTEPH, you will have an exam and may need tests, such as:
- Lung ventilation-perfusion scan (also called a lung V/Q scan): This type of test uses an X-ray to take pictures of what is happening in your lungs. During the ventilation part of the test, you will breathe in a little bit of a radioisotope gas-oxygen mixture. During the perfusion part of the test, you will get a small amount of a radioisotope (contrast material) through an IV. The pictures will show any blockages due to clots and how well blood and air move through your lungs. You will likely have a chest X-ray before or after this test.
- Echocardiogram or transthoracic echocardiogram: An ultrasound of your heart that helps determine the pressure in your pulmonary arteries.
- Computed tomography (CT) scan: A test that uses contrast material and an X-ray to show blood clots in your pulmonary arteries.
- Right-heart catheterization: This is the most accurate test to measure the pressure inside your pulmonary arteries. A long, thin tube called a catheter is inserted through a small incision in your neck or groin. The catheter is used to measure the blood pressure on the right side of your heart and lungs.
- Pulmonary angiogram: Dye is injected through a catheter in your neck or groin. Pictures are taken, and the dye shows blockages in your pulmonary arteries.
- Pulmonary function tests: These tests measure lung damage and how well your lungs are working.
What treatments are available for patients with chronic thromboembolic pulmonary hypertension?
There are several treatment options for patients with CTEPH. Your doctor will talk to you about your options, give you detailed information about each one and help you choose the one that is best for you.
Surgery
Surgery can clear the clots if they can be reached and if you are able to have surgery safely. The procedure is called a pulmonary thromboendarterectomy (PTE). It is the only cure for CTEPH.
If you are not able to have a PTE, your doctor may recommend other treatments, such as:
- Medication: Riociguat (Adempas) is a medication to treat patients with CTEPH who cannot have surgery or patients who have a PTE and still have CTEPH.
- Percutaneous balloon pulmonary angioplasty: This procedure helps improve blood flow and breathing. A catheter with a tiny balloon on the end is used to clear the blockages in the pulmonary arteries.
- Double lung transplant: Your doctor may recommend this procedure to replace both of your lungs if you are not able to have other treatments for CTEPH.
Your doctor may also talk to you about getting an inferior vena cava filter to keep clots from moving upward from your legs.
How does chronic thromboembolic pulmonary hypertension affect my life?
Your doctor will talk to you about lifestyle and other changes that are helpful if you have CTEPH. These include:
- Taking a blood thinner for the rest of your life to help prevent blood clots.
- Not smoking.
- Reaching and staying at a healthy weight.
- Taking all medications exactly as prescribed.
- Eating a low-sodium diet and other changes to your diet, as needed.
- Choosing the best types of exercise and intensity level for you.
