Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension, or high blood pressure, affecting the lungs. It is caused by blood clots. This condition is difficult to diagnose, often leading to delayed or missed diagnoses. However, once diagnosed, there are treatment options available to patients at medical centers that specialize in this disease.
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.
CTEPH is caused by the blood clots in the lungs that create scarring that 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
The symptoms of CTEPH can include:
- Being short of breath, especially during exercise
- Feeling tired
- Being weak
- Swelling of the legs due to fluid retention (edema)
- Feeling pain or pounding in the chest (the pounding is called palpitation)
- Developing cyanosis (blue color to fingers and toes)
- Rarely, coughing up blood
Symptoms can start very mild and progress with time, and are similar to other diseases. This makes CTEPH difficult to diagnose, especially in its early stages.
If your healthcare provider suspects CTEPH, he or she 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 to tell 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.
The definitive treatment for CTEPH is a delicate surgery called a pulmonary thromboendarterectomy (PTE), also referred to as a pulmonary endarterectomy. This surgery is done while the heart is stopped, and a heart-lung bypass machine does the work of the heart and the lungs. The surgeon carefully removes the clots from the pulmonary arteries. This surgery is highly specialized, and should be done by medical centers with CTEPH expertise. PTE surgery is curative in over 90 percent of patients.
If the patient is not able to have the surgery, treatment might include:
- Percutaneous balloon pulmonary angioplasty: This procedure calls for a small incision to place small balloons to destroy the clots in the arteries. It is usually done more than once and might also be done in a patient who has already had PTE. It has been shown to improve blood flow and breathing.
- Double lung transplant: This procedure replaces a patient’s lungs with donor lungs.
- Riociguat: This is the only drug approved to treat patients with CTEPH who cannot have surgery, or for patients with pulmonary hypertension that continues after surgery.
If you have any problem, please ask your health care provider for help.
Keywords: chronic thromboembolic pulmonary hypertension; CTEPH.