Chronic thromboembolic pulmonary hypertension awareness
Informed by recognized medical guidance
Overview
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare condition where old blood clots (clumps of blood) in the lungs do not dissolve and cause high blood pressure in the arteries (blood vessels) that carry blood from your heart to your lungs. This makes the right side of your heart work harder than normal.
Key facts
- CTEPH is a complication that can happen after a pulmonary embolism (a blood clot in the lung).
- It is treatable, and some people can be cured with surgery.
- Early diagnosis is important to prevent long-term damage to the heart.
No, CTEPH is rare. It affects only a small number of people who have had a pulmonary embolism.
CTEPH can affect anyone, but it is more common in older adults, people with certain blood clotting disorders, and those who have had a large or repeated blood clots in the lungs.
Symptoms
- Sudden, severe shortness of breath
- Sharp chest pain that does not go away
- Fainting or collapsing
- Coughing up a large amount of blood
- ⚠Worsening swelling in your legs or abdomen (belly)
- ⚠Persistent coughing up of blood
- ⚠Severe dizziness that does not go away
Common symptoms
- Shortness of breath, especially with activity
- Feeling very tired
- Chest pain or tightness
- Swelling in your legs or ankles (oedema)
- Dizziness or fainting
- Coughing up blood (small amounts)
Symptoms in children
- Children may show signs as being less active than usual, not wanting to play, or seeming out of breath quickly.
Symptoms in older adults
- Older adults may notice increasing fatigue, confusion, or worsening swelling in the legs.
Causes
Main causes
- The most common cause is blood clots in the lungs (pulmonary embolism) that do not fully dissolve and scar the lung arteries.
- These scars narrow the arteries, raising blood pressure in the lungs.
Risk factors
- Having had a pulmonary embolism or deep vein thrombosis (blood clot in a leg)
- Certain blood clotting disorders (like antiphospholipid syndrome)
- Splenectomy (surgery to remove the spleen)
- Chronic inflammatory conditions
- Certain cancers or cancer treatments
- Long-term use of a central venous catheter (a tube placed in a large vein)
When to see a doctor
See a doctor urgently if:
- If you have sudden or severe shortness of breath, chest pain, or faint, call your local emergency number immediately.
- If you are coughing up blood, even a small amount, seek urgent medical care.
Book a routine appointment if:
- If you have had a pulmonary embolism and continue to feel short of breath or tired for weeks or months, see your doctor.
- If you develop new swelling in your legs or ankles, make an appointment.
Diagnosis
Doctors use a series of tests to see if CTEPH is present. They will ask about your symptoms, medical history (especially blood clots), and perform a physical exam. Then they will order imaging tests of your lungs and heart.
Tests that may be done
- Echocardiogram (ultrasound of the heart) to check the heart's size and how well it pumps blood
- Ventilation-perfusion (V/Q) scan to look for blood flow problems in the lungs
- CT scan of the chest to find blood clots and see the lung arteries
- Right heart catheterisation (a thin tube placed in a vein to measure pressure inside the heart and lungs directly)
What to expect at your appointment
Diagnosis can take some time because the symptoms are similar to other conditions. You may see a specialist called a pulmonary hypertension doctor. The tests are usually safe but may require a hospital visit.
Treatment
Treatment for CTEPH aims to reduce pressure in the lung arteries and improve your quality of life. The best option depends on where the clots are and how severe the condition is. Your care team will create a plan just for you.
Self-care at home
- Keep all your medical appointments and take any medications exactly as prescribed.
- Stay as active as you can, but rest when you feel tired.
- Talk to your doctor before taking any over-the-counter medicines or supplements.
- Avoid smoking and limit alcohol.
Medical treatments
Treatments include medicines that help relax blood vessels and lower blood pressure in the lungs (called pulmonary vasodilators) and blood-thinning medicines to prevent new clots. Some people may need oxygen therapy if their oxygen levels are low. In some cases, a procedure called balloon pulmonary angioplasty can open narrowed arteries. Your doctor will discuss which medicines or procedures are safe for you.
When is surgery considered?
The main surgical treatment is pulmonary endarterectomy (PEA), where a surgeon removes the old clots and scar tissue from the lung arteries. Not everyone is a candidate for this surgery, but it can be very effective. Your specialist will explain if this might be an option for you.
Living with this condition
Living with CTEPH means managing your energy and symptoms. You may need to pace yourself and plan rest breaks. Regular check-ups with your doctor are important to monitor your condition.
Lifestyle tips
- Eat a balanced diet with plenty of fruits, vegetables, and whole grains.
- Maintain a healthy weight to reduce strain on your heart.
- Avoid heavy lifting and activities that make you very short of breath.
- Learn to recognise when you are overdoing it and take breaks.
Diet and exercise
A heart-healthy diet low in salt can help manage fluid retention. Light exercise like walking or stationary cycling, as tolerated, can help your fitness. Always check with your doctor before starting a new exercise programme.
Mental health and emotional wellbeing
Living with a chronic condition like CTEPH can cause anxiety, fear, or sadness. It is normal to feel this way. If you are struggling, talk to your doctor or a mental health professional. In a crisis, reach out to a helpline or your local emergency number.
Prevention
CTEPH cannot always be prevented, but treating a pulmonary embolism quickly and properly may lower the risk. If you have had a blood clot, taking blood thinners as prescribed and staying active can help prevent new clots.
Complications
If left untreated
- The right side of the heart can become enlarged and weak (right heart failure), leading to fluid buildup in the body.
- Irregular heart rhythms (arrhythmias) may develop.
- The condition can worsen over time and become life-threatening.
Long-term outlook
With proper treatment, many people with CTEPH feel better and have a good quality of life. The outlook is especially good for those who can have surgery. Even without surgery, medical treatments can help manage symptoms. Your doctor will work with you to find the best plan for your situation.
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Always verify with your doctor
Health guidelines vary by country and region. The information in this article is based on international clinical guidelines but may not reflect the specific guidelines, medications, or practices in your country. Always discuss your health concerns with your own doctor or healthcare provider, and refer to your local national health guidelines where available.
Important notice This information is for educational purposes only. It does not replace professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare provider about your specific situation. If you are experiencing a medical emergency, call your local emergency services immediately.
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Sources and guidance
This article is educational and is prepared with reference to recognized health information and clinical guidance sources where available. Specific source links may vary by topic.
Last updated: July 17, 2026
Educational note: This information is for education only and is not a diagnosis.
Use it to support, not replace, advice from a licensed clinician.
If symptoms are severe, worsening, or urgent, call your local emergency number or seek emergency care.