Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the small blood vessels of your lungs. It is caused by blood clots and scarring. CTEPH can happen to anyone, but it’s often curable.
CTEPH is a rare form of pulmonary hypertension. It occurs when there’s abnormally high pressure in your lung’s small blood vessels. Abnormally high pressures are a result of prior blood clots in your lungs.
CTEPH only happens to people with a history of blood clots in the lungs (pulmonary embolism). If you have high pressures without pulmonary embolisms, then you don’t have CTEPH. You have another condition.
There are approximately 5,000 new CTEPH cases per year in the U.S. This might be underestimated since the condition isn't always correctly diagnosed.
With pulmonary hypertension and CTEPH, pressure is too high on the right side of your heart. This leads to a backup of oxygen-poor blood. Your heart then works harder to push it out to your lungs. Blood also takes longer to travel through your lungs, causing a drop in oxygen levels.
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The condition occurs when tiny blood clots in lung blood vessels don’t dissolve. This causes scar-like tissue to develop, leading to blood vessel narrowing. The more severe the narrowing, the higher the pressure.
Chronic thromboembolic pulmonary hypertension risk factors include:
Chronic thromboembolic pulmonary hypertension symptoms include:
Your care may start with tests to check for signs of CTEPH. These include:
Additional studies are typically necessary. They help confirm a chronic thromboembolic pulmonary hypertension diagnosis and its severity. You may need:
A procedure is typically necessary. There are two options:
Riociguat tablets, a pulmonary hypertension medication, can bring some symptom relief. This drug is for people who can't have a procedure. It’s also for patients whose pulmonary hypertension continues after surgery.
CTEPH is a complication of many health conditions, some of which are out of your control. You can lower your risk by taking good care of your lungs. This includes avoiding tobacco or quitting smoking.
Pulmonary endarterectomy and pulmonary balloon angioplasty can potentially cure CTEPH. People who have one of these procedures typically have an excellent prognosis.
Despite this positive outlook, many patients struggle with anxiety and depression. This is especially true in people who aren't eligible for curative treatment. If you experience these issues, let your healthcare provider know. They can address your concerns and recommend therapies to help you feel better.
You'll be on blood thinners for the rest of your life. If you take warfarin, you might have to avoid certain foods. These include foods high in vitamin K, like soy and broccoli.
Light exercise, like walking, helps many people gain strength and feel their best. Your healthcare provider may recommend a medically supervised exercise program (pulmonary rehabilitation).
A note from Cleveland Clinic
Chronic thromboembolic hypertension is a form of pulmonary hypertension. It's a rare condition that causes high blood pressure in the small vessels of the lungs. CTEPH is potentially curable if you undergo a procedure. Many people who receive treatment for this condition have an excellent prognosis.
Last reviewed by a Cleveland Clinic medical professional on 07/29/2022.
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