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What is CTEPH?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, long-term disease caused by blockages in the blood vessels that deliver blood from the heart to the lungs.1 These blockages arise from a blood clot in the lungs – known as an acute pulmonary embolism.1 This causes an increase in the blood pressure in the pulmonary arteries (the vessels that deliver blood to the lungs), which means the heart must work harder to pump blood through the systemic circulation (the network of vessels that supply blood to the rest of the body). This might leave you feeling short of breath or more tired than usual during everyday tasks.2

 

To find out more about CTEPH, visit the “CTEPH basics” resources here.

 

What are the symptoms of CTEPH?

Symptoms of CTEPH vary from person to person, but the most common symptoms include feeling short of breath or more tired than usual during everyday activities.1,2 Some patients also experience dizziness, pains in their chest or symptoms of anxiety and low mood. You may find that routine tasks, such as household chores or grocery shopping, become more difficult over time: talk to your doctor if any of your symptoms, including your mental health, start to get worse.

 

To find out more about the symptoms of CTEPH and how they might affect your life, visit the “CTEPH – Symptoms & risk factors” page here, or check out our Patient stories page.

 

Is CTEPH hereditary?

Although some genetic factors have been linked with CTEPH, the condition is not considered to be hereditary – meaning it is unlikely to be passed from parent to child.1

However, you may be at greater risk of developing CTEPH if you have a family history of blood clots, as this may increase your risk of acute pulmonary embolism.3 In some patients, CTEPH may develop as a long-term complication of acute pulmonary embolism, meaning that genetic factors that increase your risk of blood clots may indirectly increase the risk of CTEPH.1

 

To find out more about the other risk factors for CTEPH, please visit our “CTEPH – Symptoms & risk factors” page here.

 

Why do people develop blood clots?

In some patients, CTEPH may develop as a long-term complication of a blood clot in the lungs – known as an acute pulmonary embolism.1 Although blood clots may develop for many reasons, some lifestyle factors can increase blood clot risk, such as smoking, obesity or the use of birth control pills.4 You may also be more likely to develop a blood clot if you have recently undergone surgery, are pregnant or have been diagnosed with some forms of cancer.4

Staying active and maintaining a healthy weight may decrease the risk of blood clots and improve your overall health.

 

For general advice on staying healthy – whether you have been diagnosed with CTEPH or not – please visit our “Diet & exercise tips” page here.

 

To find out more about the other risk factors for CTEPH, please visit our “CTEPH – Symptoms & risk factors” page here.

 

Is CTEPH curable?

CTEPH is potentially curable by a type of surgery called pulmonary endarterectomy (PEA) or pulmonary thromboendarterectomy (PTE). This surgery removes the blockages from the blood vessels in the lungs and may reduce symptoms for some patients.1,5 Although PEA is the recommended treatment for CTEPH, not everyone is a candidate for surgery.5,6 Even after PEA surgery, some patients may still experience CTEPH symptoms.7–9 Your healthcare team will determine if PEA surgery is right for you.

 

To find out more about PEA,  click on the links to visit the “CTEPH – Treatment” or “CTEPH – PEA surgery” webpages, or refer to “What is PEA?” below.

 

How is CTEPH diagnosed?

CTEPH can be difficult to diagnose as many of its symptoms are similar to those of other conditions, such as asthma or acute pulmonary embolism.10 If your doctor suspects you may have CTEPH, they may perform several tests to confirm your diagnosis.5 These might include non-invasive procedures like echocardiography (which uses sound waves to create a moving image of your heart) to more specialist procedures like ventilation/perfusion (V/Q) scanning and right heart catheterization (RHC).

Multiple tests are needed to confirm CTEPH diagnosis, and this process may take several months.6 You may be referred to a specialist CTEPH center, where a team of expert doctors, nurses, surgeons and other healthcare professionals will assess your symptoms.5

 

To find out more about how CTEPH is diagnosed, visit the “Diagnosis” resources here. For more information about the specific tests needed to diagnose CTEPH, please see “What is a V/Q scan?” and “What is right heart catheterization?” below.

 

My doctor says I might have CTEPH: how long will it take to know for sure?

It may take several months from your first visit to your doctor to confirm a diagnosis of CTEPH. This is because many of the symptoms of CTEPH are similar to those of other conditions, which need to be ruled out, and multiple tests are required to confirm you have CTEPH.6,10 You may feel frustrated or anxious during this time – talk to your doctor about your concerns and the support that may be available to you.

 

To find out more about diagnosis from a patient’s perspective, visit our Patient stories page.

 

What is a V/Q scan?

A V/Q, or ventilation/perfusion scan, is an important test required in the screening of CTEPH.5 Two tests make up this scan – a ventilation scan and a perfusion scan. During the ventilation scan, you will be asked to inhale a weak radioactive gas through a mask while lying under a scanner: this allows your doctor to understand how air flows through your lungs. For the perfusion scan, your doctor will inject a small amount of radioactive tracer into your vein. A scanner is then used to allow your doctor to see how well blood is flowing through your lungs, which can identify any blockages that indicate you may have CTEPH.

 

To hear a patient’s perspective on their CTEPH diagnosis journey, visit our Patient stories page.

 

What is right heart catheterization?

Right heart catheterization (RHC) is used to measure the pressures in your heart and blood vessels in the lungs, which may help your doctor understand whether you have CTEPH.5 During RHC, your doctor will insert a thin rubber tube – called a catheter – into a large vein in your neck or groin, before passing the catheter up to your heart. RHC takes place in a hospital under local anesthetic, but you can usually go home soon after the procedure.

 

To hear a patient’s perspective on their CTEPH diagnosis journey, visit our Patient stories page.

 

How might CTEPH affect my day-to-day life?

Living with CTEPH can impact on your life in many ways. You may find everyday tasks, such as household chores or grocery shopping, more tiring, and your ability to work outside your home may become limited. It’s important to remember that you have the support of family, friends and your doctor when you need it. If you feel able to work, you may wish to speak with your employer about adjusting your work schedule, such as working part-time or from home, if possible.

 

For more information about the support your doctor can provide, please see “What support is available to help me live with CTEPH?” below or visit our “Living with CTEPH” resource here.

 

What treatment options are available for patients with CTEPH?

Following your diagnosis, your healthcare team will carefully consider which treatment option is right for you.5

CTEPH is potentially curable by a type of surgery called pulmonary endarterectomy (PEA) or pulmonary thromboendarterectomy (PTE). PEA is the recommended treatment for CTEPH: however, not everyone is a suitable candidate and some patients still experience CTEPH symptoms even after PEA surgery.5–9

If your doctor decides your CTEPH is inoperable, or your symptoms return after PEA, talk to your healthcare team about the best options for you. As with many diseases, medical science is always looking for new treatments – your team will keep you up to date with any therapies that may be suitable for you.

 

To find out more about possible treatment options for CTEPH, visit our “Treatment” webpages here.

 

What is PEA surgery?

Pulmonary endarterectomy (PEA) surgery – sometimes also referred to as pulmonary thromboendarterectomy (PTE) – is the only potentially curative treatment for CTEPH and is therefore recommended for all eligible patients.5 PEA is a complex and invasive procedure which allows blockages to be removed from the arteries in the lungs.1 In many patients, PEA can reduce CTEPH symptoms and improve heart function.7

However, PEA is not suitable for every patient: up to 4 in 10 patients with CTEPH are not eligible for PEA.6 Even after PEA, as many as 1 in 3 patients experience CTEPH symptoms.7–9 If you have been diagnosed with CTEPH, your healthcare team will determine if PEA is right for you.

 

To find out more about PEA surgery, visit our “CTEPH – Treatment” page or check out our Patient stories page.

 

How is PEA operability determined?

Although pulmonary endarterectomy (PEA) is the recommended treatment option for CTEPH, not everyone is a candidate for this potentially curative surgery.5,6 Your healthcare team will determine if CTEPH is right for you by considering several factors, including the location of blockages in your lungs.5 For successful PEA, the surgeon must be able to reach and remove the blockages in your arteries, which may not be possible if your blockages are within the small vessels deep within your lungs. Your team will also consider your overall health and any other conditions you may have: PEA is a highly invasive procedure with associated risks, so it is important to weigh up the possible benefits against the risk of major surgery.

 

To find out more about PEA operability assessments, visit our “CTEPH – PEA surgery” page, or check out our Patient stories page for a patient’s perspective.

 

What is the outlook for people with CTEPH?

The outlook for a person with CTEPH depends on several factors, including the severity of your condition, your overall health and lifestyle, and how well you respond to treatment. However, the introduction of new, effective treatments for pulmonary hypertension (PH) has improved long-term outcomes for patients with CTEPH. Pulmonary endarterectomy (PEA) surgery – sometimes referred to as pulmonary thromboendarterectomy (PTE) – is potentially curative for some eligible patients with CTEPH.5 For patients who are not eligible for PEA or experience CTEPH symptoms after surgery, medical science is exploring new treatment options that may be suitable for you. Talk to your doctor about the best options for your CTEPH.

 

To find out more about the support available for patients with CTEPH, check out our “Living with CTEPH” resources here.

 

What support is available to help me live with CTEPH?

Living with CTEPH may impact on your life in many ways, and you may feel overwhelmed following your diagnosis. However, there are many sources of support for patients with CTEPH; both online and at home. Patient organizations, such as PHA Europe, provide guidance and information for people living with CTEPH, including online communities to connect patients across the globe. Closer to home, your doctor can provide you with details of local support groups for patients with CTEPH. It is also important to remember that you have the support of friends, family and your doctor if you need it – ask for help if you are struggling to manage living with CTEPH.

 

To find out more about the support available for patients with CTEPH, check out our “Living with CTEPH” resources here.

 

Can lifestyle factors affect my CTEPH?

If you are living with CTEPH, it is important to eat a balanced diet and maintain a healthy weight. Although it may feel difficult to stay active, light exercise may improve your overall lifestyle – it’s important to start small and talk to your doctor before making any changes in your exercise program. Under your doctor’s guidance, you may be able to exercise more than you thought you could.

 

For more tips about living a healthy lifestyle with CTEPH, check out our “CTEPH – Diet & exercise” page here.

 

As a carer, how can I support someone living with CTEPH?

For patients living with CTEPH, the support of family and friends can make a real difference to their quality of life. As a caregiver, the most important thing you can do is provide emotional support: make sure the person you care for feels valued and listen to any concerns they may have. However, there are also some practical things you can do to support the well-being of the person you care for. This includes keeping an eye on their symptoms, making a note of any changes you may see and encouraging the person you care for to discuss these with their doctor at their next appointment.

Caring for someone with a condition like CTEPH can be challenging – make sure to take breaks from caring to focus on yourself from time to time and ask for further support if you need it.

 

If you are a patient and are worried about the impact of CTEPH on your caregivers, please visit our “Helping your caregiver help you” page here.

 

 

References:

 

1. Piazza G, Goldhaber SZ. N Engl J Med 2011;364:351–60. 2. American Heart Association 2014. What is pulmonary hypertension (accessed June 2020). 3. Dodson MW et al. Am J Respir Crit Care Med 2016;193:A1237. 4. American Heart Association 2020. Understand Your Risk for Excessive Blood Clotting (accessed June 2020). 5. Galiè N et al. Eur Respir J 2015;46:903–75. 6. Pepke-Zaba J et al. Circulation 2011;124:1973–81. 7. Mayer E et al. J Thorac Cardiovasc Surg 2011;141:702–10. 8. Condliffe R et al. Am J Respir Crit Care Med 2008;177:1122–7. 9. Freed DH et al. J Thorac Cardiovasc Surg 2011;141:383–7. 10. Gopalan D et al. Eur Respir Rev 2017;26:160108.