Still breathless months after a clot? (CTEPH)
Most people feel better within a few months of a pulmonary embolism. If you don’t, old scarred clot in the lung arteries is one cause worth ruling out, because it’s treatable and often curable.
Quick answer: CTEPH is high blood pressure in the lungs caused by old clots that turned into scar tissue. About 3 in 100 people who survive a pulmonary embolism (PE) develop it. If you’re still breathless, or getting more breathless, 3 months or more after a PE, ask your GP about checking for it. The usual tests are an echocardiogram and a V/Q lung scan. Surgery can often cure it; balloon angioplasty and a medicine called riociguat help others, along with lifelong blood thinners.
CTEPH Awareness Day is 11 November (Pulmonary Hypertension Association), in the middle of Pulmonary Hypertension Awareness Month. The message: breathlessness that lingers months after a clot deserves a proper look.
What it is. Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the lungs caused by old clots that never fully dissolved. They turn into scar tissue that narrows the lung arteries, so the right side of the heart has to pump harder.
How common after a PE.
- About 3 in 100 people who survive a pulmonary embolism (PE) develop CTEPH (3.2% in a 2017 review of studies).
- Leftover clot is much commoner: 30% to 50% of people still have some a year after a PE, and most never get CTEPH.
- Many people with CTEPH never knew they had a clot, so it can be missed.
Symptoms. They creep up slowly, and there can be a “honeymoon” with few symptoms after the PE.
- Breathlessness on exertion that isn’t improving, or is getting worse
- Tiredness, and feeling you can’t do what you used to
- Chest discomfort or palpitations
- Later: dizziness or fainting, swollen ankles, legs or tummy, and bluish fingers or toes (signs the right heart is struggling)
Why 3 months matters
- Most people feel better within weeks to months of starting blood thinners.
- The 2022 European (ESC/ERS) pulmonary hypertension guidelines recommend checking for CTEPH in anyone with persistent or new breathlessness or exercise limitation after a PE (a strong, class I recommendation).
- Testing is usually done after at least 3 months of blood thinners, once the acute clot has had time to clear, or sooner if someone is getting worse.
The tests.
- Echocardiogram (heart ultrasound): estimates lung pressure and checks the right heart.
- V/Q scan (lung ventilation-perfusion scan): the guideline calls it the most effective way to rule out clot-related disease. A normal scan makes CTEPH very unlikely.
- A CT scan of the lung arteries can miss smaller, distant clots, so a normal CT doesn’t rule it out.
- If tests are abnormal: referral to a specialist pulmonary hypertension centre for a right heart catheter study and more imaging.
- Blood tests, such as BNP, and an exercise test may help too.
Treatment
CTEPH is the one type of pulmonary hypertension that can often be cured with surgery. Decisions are made by an expert CTEPH team.
- EveryoneLifelong anticoagulationBlood thinners to stop new clots.
- Works
- Recommended for everyone with CTEPH, for life
- Access
- GP and specialist
- Watch for
- Bleeding. People with antiphospholipid syndrome need warfarin rather than the newer tablets.
- First choicePulmonary endarterectomy (PEA)Open-heart surgery to peel the scarred clot out of the lung arteries.
- Works
- Potentially curative; about 90% are alive at 3 years in expert centres
- Access
- Specialist CTEPH surgical centres; roughly 60% to 70% of people are suitable
- Watch for
- Major surgery; some people have leftover pulmonary hypertension afterwards.
- If surgery isn’t possibleBalloon pulmonary angioplasty (BPA)A thin tube passed up to the lungs stretches narrowed arteries with a small balloon.
- Works
- Lowers lung pressure; for clots surgery can’t reach, or leftover pressure after surgery
- Access
- Specialist centres; usually several sessions
- Watch for
- Rare but serious complications, such as bleeding in the lung.
- MedicineRiociguatA tablet that relaxes and widens the lung arteries.
- Works
- Improves walking distance and lowers lung artery resistance (by about a third in the main trial)
- Access
- Specialist prescription, for inoperable CTEPH or pressure that persists after surgery
- Watch for
- Low blood pressure and dizziness. Not with nitrates or sildenafil-type medicines, and not in pregnancy.
Living with it
- Take your blood thinner every day, and tell every doctor, dentist and pharmacist you’re on it.
- Stay as active as your team advises; pulmonary rehab can help.
- Get flu, COVID-19 and pneumococcal vaccines.
- After treatment, expect yearly checks with an echo and exercise test.
- Patient groups such as the Pulmonary Hypertension Association offer support and information.
See your GP if
- You’re still breathless 3 months or more after a PE, or your breathing is getting worse, not better
- You get breathless doing things you managed easily before
- You’ve had a clot and notice swollen ankles, dizziness or chest discomfort on exertion
- You have unexplained breathlessness, even with no known clot
Get urgent help: call 000 (Australia) or your local emergency number for sudden breathlessness, chest pain, coughing up blood or fainting. These can mean a new clot.
Outlook. Without treatment, CTEPH tends to worsen. With surgery at an expert centre most people improve a lot, and BPA and medicine help many who can’t have surgery.
Common questions
How common is CTEPH after a pulmonary embolism?
About 3 in 100 people who survive a PE develop CTEPH. Some leftover clot is much commoner, but most people with leftover clot don’t get CTEPH.
Why should breathlessness 3 months after a PE be checked?
Most people improve within a few months on blood thinners. The 2022 ESC/ERS guidelines recommend checking for CTEPH in anyone with persistent or new breathlessness after a PE, usually after at least 3 months of treatment.
What tests check for CTEPH?
An echocardiogram estimates the pressure in the lungs, and a V/Q scan is the best test to rule out clot-related disease. Abnormal results lead to a specialist centre for more tests.
Can CTEPH be cured?
Often, yes. Pulmonary endarterectomy surgery can be curative for people whose clots are surgically reachable. Others can be helped by balloon pulmonary angioplasty and riociguat.
Do I need blood thinners for life with CTEPH?
Yes. Guidelines recommend lifelong anticoagulation for everyone with CTEPH, even after successful surgery.
Can you get CTEPH without a known clot?
Yes. Many people with CTEPH never knew they had a PE, so unexplained breathlessness should be checked.
When is CTEPH Awareness Day?
11 November, during Pulmonary Hypertension Awareness Month, run by the Pulmonary Hypertension Association.
Evidence and more information
- Humbert et al, Eur Heart J 2022: 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
- Ende-Verhaar et al, Eur Respir J 2017: Incidence of CTEPH after acute pulmonary embolism, a contemporary view of the published literature
- Taskar, J Postgrad Med 2023: Chronic thromboembolic pulmonary hypertension, time is of the essence
- American Thoracic Society: CTEPH patient information
- Royal Papworth Hospital: CTEPH patient information booklet
- Pulmonary Hypertension Association: CTEPH Awareness Day
maladywise.com/cteph/