What is chronic thromboembolic pulmonary hypertension (CTEPH)?
The lungs and heart must exert pressure to move blood throughout the body. CTEPH is high blood pressure in the arteries in the pulmonary system (lungs). This is caused by clots in the blood vessels that last after at least three months on blood thinners. The clots typically leave scar tissue in the arteries.
How common is chronic thromboembolic pulmonary hypertension (CTEPH)?
The incidence of CTEPH in the US is estimated to be about 5,000 new cases per year. This is possibly low since CTEPH is not always diagnosed correctly.
Who is affected by chronic thromboembolic pulmonary hypertension (CTEPH)?
CTEPH can happen to anyone. Although CTEPH does develop from pulmonary embolisms (blood clots in the lungs, or PEs), a percentage of people with CTEPH have not had earlier PEs.
Risk factors for PEs include:
- Being still or inactive for a long period of time, including being laid up due to illness or not moving enough during trips by plane or automobiles.
- Having had surgery, especially joint replacement surgery.
- Getting older.
- Estrogen-containing birth control pills.
- Having certain diseases like cancer.
Symptoms and Causes
What causes chronic thromboembolic pulmonary hypertension (CTEPH)?
CTEPH is caused by the blood clots in the lungs that create scarring that in turn blocks the arteries in the lungs. Certain conditions, like cancer, blood diseases or inflammatory diseases are linked to a higher risk of CTEPH. Other risk factors for CTEPH include:
- Unprovoked large pulmonary embolisms, or PEs.
- Not having a spleen.
- Being on thyroid replacement therapy.
- Some blood clotting disorders, such as the lupus anticoagulant or antiphospholipid syndrome.
- Having an infected pacemaker.
- Having a blood type that is not Type O.
What are the symptoms of chronic thromboembolic pulmonary hypertension (CTEPH)?
- Being short of breath, especially during exercise.
- Feeling tired.
- Feeling weak.
- Feeling pain or pounding in the chest (the pounding is called palpitations).
- Swelling of the legs due to fluid retention (edema).
- Developing cyanosis (blue color to fingers and toes).
- Rarely, coughing up blood.
Diagnosis and Tests
How is chronic thromboembolic pulmonary hypertension (CTEPH) diagnosed?
If your healthcare provider suspects CTEPH, they will examine you and then may order one or more of the following tests:
- Lung ventilation-perfusion scan (also called lung V/Q scan): This type of test uses X-ray scanning equipment to take pictures of what is happening in your lungs. The ventilation portion calls for you to breathe in a little bit of a radioisotope gas-oxygen mixture. The perfusion portion calls for an injection of the radioisotope (basically contrast material) into your veins. The special X-rays can then determine if there is blockage due to a clot and how well blood and air moves in your lungs. A chest X-ray is usually done before or after a lung V/Q scan.
- Echocardiogram (sonogram of the heart; also called transthoracic echocardiogram or TTE): Sonography uses sound waves to make pictures of your heart and can estimate the pressure in the lung arteries. This painless test is often done early in the diagnosis process.
- Computed tomography (CT) scan: Contrast material is injected into your vein and X-ray pictures are taken. The pictures will show blood clots.
- Right-heart catheterization: This test, which uses a catheter placed in the neck or groin, measures blood pressure in the right side of the heart and lungs. This is the most accurate test for pulmonary hypertension.
- Angiography of lung (pulmonary angiogram): In tests of these kind, dye is injected into the arteries via a catheter put into a vein in the neck or groin. Pictures are taken. The dye highlights blockages in arteries of the lungs.
- Pulmonary function tests: These tests measure how well the lungs are working and the extent of damage to tissues.
Management and Treatment
What are the treatments for chronic thromboembolic pulmonary hypertension (CTEPH)?
If the clots are accessible, and you are able to withstand surgery, the treatment of choice is surgical pulmonary thromboendarterectomy (PTE), also called pulmonary endarterectomy (PEA). During this surgery, done through an incision (cut) in the breastbone, you are put on a heart-lung machine and cooled from 37 degrees Celsius to 18 degrees Celsius. After cooling happens, the circulation is stopped. This lets the surgeon look into the arteries of the lungs. Surgeons use special tools to carefully separate the clots from the normal wall of the artery. This delicate surgery should be done by a specialist team with experience. In these circumstances, the surgery is safe and can cure this disease.
If the you are not able to or do not want to have open surgery, treatment might include:
- Percutaneous balloon pulmonary angioplasty: This procedure calls for a small puncture. It uses small balloons and catheters (tubes) to break the scars in the arteries. It is usually done more than once. It might also be done in someone who has already had PTE. It has been shown to improve blood flow and breathing.
- Double lung transplant: This procedure replaces your lungs with donor lungs. If you are not a candidate for PTE surgery or balloon angioplasty, lung transplantation may be an option.
- Riociguat (Adempas®): This is the only drug approved to treat people with CTEPH who cannot have surgery, or for people with pulmonary hypertension that continues after surgery.
What are the complications/side effects of the treatments of chronic thromboembolic pulmonary hypertension (CTEPH)?
PTE surgery is delicate. If you have the surgery, you should expect to be in the hospital for about 10 days after surgery. The breastbone takes about two months to heal. You should be able to resume almost all activities by three months after PTE.
If you take Adempas® to treat CTEPH, you might have the following side effects:
Untreated, CTEPH gets worse and can be fatal.
What can you do to help relieve symptoms of chronic thromboembolic pulmonary hypertension (CTEPH)?
- Pay attention to your body, and do not overdo it. Low-intensity exercise (like walking) is recommended. Stop if you get dizzy or short of breath.
- Follow a low-salt diet as recommended by your healthcare provider.
- Follow any dietary needs that might go along with your blood-thinner medication.
- You should continue to take blood-thinners for the rest of your life.
How can you prevent chronic thromboembolic pulmonary hypertension (CTEPH)?
You can make efforts to combat certain risk factors. For instance, it helps to maintain a healthy weight and to avoid smoking. Take your medication (like blood-thinners) as directed. Always contact your healthcare provider if you notice that shortness of breath or fatigue is increasing.
Outlook / Prognosis
What is the prognosis (outlook) for patients who have chronic thromboembolic pulmonary hypertension (CTEPH)?
Pulmonary endarterectomy/pulmonary thromboendarterectomy (PEA/PTE) can cure CTEPH. The outlook is excellent. When surgery is performed by experienced teams, the risk of surgical death is less than 3%.
What should you know about living with chronic thromboembolic pulmonary hypertension (CTEPH)?
- You will have to be on blood-thinners for the rest of your life. Warfarin (Coumadin®) is preferred, but you might be able to switch to newer anticoagulants after a period of time.
- Your healthcare provider may suggest a low-salt diet. In addition, you might have to be careful with some foods and drugs as a result of taking blood-thinners.
- Your healthcare provider might suggest exercise, such as walking, as tolerated.
- Your doctor might decide to insert an inferior vena cava filter (IVC filter) into your abdomen to stop clots from moving upward from the legs.
When should you call the doctor if you have or suspect CTEPH?
Call the doctor or 911 if you:
- Have a fever.
- Have pain or other signs of infection, especially after surgery.
- Are suddenly unable to breathe well.
- Feel faint or actually do faint.
Are there any resources for a person with chronic thromboembolic pulmonary hypertension (CTEPH) and/or their families?
Resources for CTEPH include:
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