Learn About Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive form of pulmonary Hypertension (PH). It is caused by blood clots – some old, some new – that don’t dissolve in the lungs. This causes scar-like tissue that clogs up or narrow down the small blood vessels in the lungs.
- CTEPH is a rare disease.
- The leading symptom of CTEPH is shortness of breath – which can be difficult to diagnose since its a common symptom of many other conditions.
- Many patients with CTEPH can be cured with surgery. For patients who can’t have surgery, there are medications and other treatments that can help reduce symptoms and provide some relief.
How CTEPH Affects Your Body
Let’s start by understanding all the words that make up the name of this disease.
Chronic means a condition that lasts over a long period of time, like months or years.
Thromboembolic means that a blood clot (thrombus) forms in a blood vessel in the body and travels through your arms, legs, or a vein into blood vessels in the lungs, where it clogs the arteries. A blood clot in the lungs is called a pulmonary embolism (PE).
Pulmonary is a medical term that refers to the lungs.
Hypertension means high blood pressure, and, in this case, it refers to high blood pressure in your lungs.
Next, let’s review the way lungs contribute to the normal flow of blood. The heart is divided into two sides: the left and the right. Each side has an atrium (a receiving chamber) and a ventricle (a pumping chamber). Each side is responsible for pumping blood, but for different reasons. The left side of the heart pumps blood through arteries to supply oxygen and vital nutrients to cells and tissues throughout the body. Because the blood has to go a long distance from the heart, it needs a lot of pressure. When you get your blood pressure checked, it is measuring the pressure that pushes the blood throughout the body.
After the blood delivers the oxygen, it returns through veins right side of the heart and is pumped back to the lungs. The pump in the right side of the heart doesn’t need a lot of pressure since the heart and lung are so close together. This is called pulmonary pressure and it is measured differently from the blood pressure in the left side of the heart.
When the pumping pressure is too high in the left side of the heart, it is called hypertension or high blood pressure. Similarly, when the pumping pressure is too high in the right side of the heart, it is called pulmonary hypertension (PH).
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive form of PH. In CTEPH, repeated blood clots in the lung get stuck and clog the arteries, which turns into scar tissue over time. As the blood vessels get narrow and clogged, pulmonary pressure grows and PH results. This means the right side of the heart has to pump harder – meaning use more pressure – to get blood to pass through.
It also leads to a back-up of blood returning from the veins to the heart. As a result, the right side of the heart works too hard to keep up with the blood flow. This can weaken the heart, make it swell, and, over time, lead to heart failure. Because it takes longer for the blood to travel through the lungs, the oxygen level in the blood can get too low. Oxygen is important to cells and tissue and is needed for healthy brain function. Without treatment, patients with CTEPH will die within 5 years. That’s why diagnosing and treating CTEPH is so critical.
Measuring Blood Pressure
You’re probably familiar with getting your blood pressure checked with a blood pressure cuff. This measures the pressure in the left side of the heart and is recorded in millimeters of mercury (mm Hg). There are two parts to this blood pressure measure. The first number reflects the systolic measure, which measures the pressure when your heart beats. The second number is the diastolic measure, which measures the pressure when your heart rests between beats. A normal blood pressure level is 120 (systolic) over 80 (diastolic).
In the right side of the heart, the pressure measures the blood vessels in the lungs, not the heart, and is called pulmonary artery pressure (PAP). Normal pulmonary artery pressure is 8 to 20 mm Hg at rest. If this measure is greater than 25 mm Hg at rest or 30 mm Hg during physical activity, it is considered high and qualifies as pulmonary hypertension.
CTEPH Symptoms, Causes, and Risk Factors
What are the Symptoms of CTEPH?
It is hard to diagnose CTEPH because it’s symptoms are like those of other diseases, including:
- Chronic obstructive pulmonary disease (COPD)
- Obesity (overweight)
- Heart failure
Common symptoms include:
- Shortness of breath, especially with exercise or climbing stairs
- Fatigue (tiredness)
- Swelling of the legs
- Tightness in the chest or belly
- Palpitations (heart pounding in the chest)
In rare cases, patients may cough up blood.
What Causes CTEPH?
Some of the most common causes of CTEPH are:
- History of one or more blood clots in the lungs
- Elevated blood pressure at the time that a blood clot is diagnosed
- Having PH at least 6 months after a pulmonary embolism (blood clot of the lung) has been diagnosed
What Are the Risk Factors?
About 75% of patients diagnosed with CTEPH have had one or more blood clots in the lungs. Other risk factors include:
- Having a high risk for blood clots
- Chronic inflammatory diseases, such as osteomyelitis or inflammatory bowel disease
- Having your spleen removed
- Thyroid replacement therapy
- Family history of blood clots
- Being female
- Blood clotting (hypercoagulability) disorders
When to See Your Doctor
If you have had a blood clot in your lung, (a pulmonary embolism (PE)), and are still experiencing shortness of breath or other symptoms (difficulty with exertion or exercise) after 3 months of treatment with bloodthinners you should discuss your symptoms with your doctor. The symptoms could be caused by pulmonary hypertension (PH) or the old clots. Then you would need more tests to see what kind of PH you have and what therapy would be required.
Diagnosing and Treating CTEPH
Print out and take the following checklists to your appointment to facilitate better conversations with your health-care provider.
Questions About Your Diagnosis, Treatment, and Lifestyle
CTEPH Recovery Timeline
If your doctor thinks you might have CTEPH, you will need several tests. First, the tests need to prove that you have PH and second to prove you have CTEPH. A description of the tests used for diagnosing CTEPH are listed next.
Ventilation-perfusion scan (VQ scan): This test looks at how well air and blood are flowing into the lungs. The scan creates pictures that doctors can look at to see if you have blood clots in the lungs. It is recommended as the best screening test when CTEPH is suspected.
Echocardiogram: This test is an ultrasound scan of the heart. It doesn’t require any surgery. It looks at the areas of the heart to see how big they are and how well they function. An echocardiogram is a simple test that can detect some signs of PH in most patients with the disease. It does not directly measure pulmonary arterial pressure (PAP), but it gives doctors an idea of whether or not you have PH and how severe it is.
Pulmonary function tests: These are simple tests that measure how much air you can hold in your lungs and how much air moves in and out of your lungs. Pulmonary function tests help doctors know whether or not lung tissue is diseased.
Right-sided heart catheterization: If any of the tests suggest PH, your doctor will likely do a right-sided heart catheterization. This test will best show which of the five forms of PH you have. This test involves placing a small tube (catheter) into a large vein in the neck, arm, or groin. The tube goes from a large vein in the leg, neck, or arm to the right side of the heart, then out to the blood vessels that connect to the lung. The doctor measures the pressure in different areas of the heart and in lung blood vessels (pulmonary artery). These measurements can tell how severe your PH is and the cause of it.
Pulmonary angiogram. This test takes an x-ray of the blood vessels of the lungs. A dye is injected into the body and shows up on the x-rays. A pulmonary angiogram helps doctors find blood clots and blocked blood vessels in the lungs. This helps doctors identify the next treatment steps that might include surgery.
CT (computed tomography or CAT scan) pulmonary angiograph: This test is like a pulmonary angiogram, but instead of x-ray images, it shows cross-sectional (thin section) images of your lungs. This gives your doctor a better picture of what is happening in your lungs and where the blood clots are.
MRI (magnetic resonance imaging): An MRI uses magnets and radio waves to make a very detailed image. An MRI makes it easy for doctors to see which parts of your body are normal and which parts are not. In some patients, there may be evidence of chronic blood clots in lungs but without evidence of high blood pressure in lungs at rest and this condition is referred to as chronic thromboembolic disease (CTED).
Exercise tolerance test (also known as the 6-minute walk test): This test is used to compare your exercise capacity, oxygen levels and symptoms over time and to evaluate how these characteristics change over time and with therapy.
How is CTEPH Treated?
The best treatment for CTEPH is a surgical procedure called pulmonary thromboendarterectomy (PTE). It can also be called a pulmonary endarterectomy (PEA). PTE is a very delicate surgery and should only be performed in centers with experience in this procedure. If you are not believed to be a candidate for this procedure, it is worthwhile to seek a second opinion. First, the patient is put on a machine (heart-lung bypass) to provide blood flow to the other organs. After this is done, the heart is stopped while the surgeon removes all clots from the arteries in the lung. The good news is that PTE surgery can cause significant improvement in most patients and result in normalization of the pulmonary artery pressures in many patients. It is very important to maintain close follow up with your physician after this surgery to monitor for increases in the pulmonary artery pressure and determine the best treatment if there is any pulmonary hypertension following surgery.
If you qualify for and receive PTE:
- Make sure your doctor sends you to a medical center with experience in PTE surgery.
- You will be in the hospital for about 2 weeks.
- Your shortness of breath should be better right after surgery.
- You may have pain from the cut and stitches in your breastbone.
- It takes about 2 months for the bone to heal.
You should not drive, lift anything heavier than 10 pounds, or get the wound wet (no swimming or bathing).
If you can’t have PTE, there is a newer treatment called balloon pulmonary angioplasty (BPA). The doctor uses a tiny balloon that is blown up inside the lung artery. This helps open up the artery so it’s not so narrow. This treatment can lower pulmonary artery pressure and help reduce strain on the right side of the heart. There are also medicines for patients with CTEPH who can’t have surgery or who still have some PH after PTE surgery.
Surgical Management of CTEPH is the preferred approach. Medications should be used in patients that are not a candidate for surgery or who have persistent PH after surgery. Riociguat is the only PH specific medication that is approved by the FDA for this indication; although, other medicines may be used if necessary.
All patients with CTEPH, including those who successfully undergo PTE surgery or balloon pulmonary angioplasty, should take blood thinners (anticoagulants) for the rest of their lives to keep new blood clots from forming. You should talk to your doctor about the best medication for you to take. Things your doctor will talk about include:
- Risks of taking blood thinners
- Problems that may occur if you are taking other medications at the same time
If you have CTEPH there are some things you need to look at to make life easier. Many patients change their lifestyle to include exercise, nutrition, and support groups. You may also need to pay attention to CTEPH when you travel. Let’s look at each of these areas.
Exercise and CTEPH
Exercise is a great way to rebuild, make you stronger and help you breath when you do activities like climbing stairs. Pulmonary rehabilitation (PR) is an organized exercise program paid for by Medicare. PR helps you control or reduce shortness of breath and feel stronger.
Pulmonary rehabilitation offers:
- Structured and monitored exercise activities
- Nutrition advice
- Help with how and when to take your medications
- Techniques to help you with your breathing problems
- Information about keeping and improving your body functions
- Help to quit smoking
- Support for depression and anxiety
- Help improve muscles that decrease shortness of breath
Because PR can improve life for many people with CTEPH, be sure ask your doctor about it.
Good nutrition – eating the right foods – can also help you better manage your CTEPH. Some people with CTEPH have trouble keeping weight on. When you lose weight, you lose muscle mass, which can weaken your strength and cause other physical limitations. Other people with CTEPH may be overweight, which adds strain to your heart and lungs. Talk to your doctor about good nutrition and a good weight for you to maintain.
Here are some helpful tips for maintaining a healthy weight:
- Eat several smaller meals throughout the day instead of three large ones.
- Ask your doctor about how much liquid you should drink each day so that you don’t get too much fluid.
- Eat slowly and chew food thoroughly.
- Make sure you are on a low salt diet.
Depression and Anxiety
Many people with CTEPH may feel depressed or anxious. This doesn’t mean that you are crazy or defective. Depression is the rule — not the exception — in patients with complex medical problems like CTEPH. It also can be hard to keep up with personal relationships and maintain your regular levels of activity when you are sick.
For some people, medication helps with depression, especially if the person wants to hurt themselves. Some treatments for depression and anxiety that don’t involve medicine are talk therapy, exercise, and meetings with a support group. You should never be afraid to talk to your doctors and other members of your care team about your feelings.
Your diagnosis of CTEPH can be difficult for the entire family. Try to remember that your family members may have feelings of depression and anxiety too. Support groups can help both you and your family.
Travel and CTEPH
It can be hard to travel with CTEPH, which is why it’s important that you make plans in advance. Some things to think about include:
- Review your travel plans with your doctors to see if you need special supplies, medication, and/or extra oxygen. Even if you don’t usually need oxygen, you may need it if you are traveling to a high-altitude.
- You need to know where to get medicine and oxygen on your trip.
- You should know in advance where you can receive care on your trip in case of emergency.
Some people with CTEPH may need to use extra oxygen to help them breathe better and improve their daily life. Oxygen is not addictive, so you don’t have to worry about how much you use. Oxygen can help:
- Make breathing easier
- Improve sleep, mood, and mental ability
- Your body work better during the day
There are three types of oxygen:
- Compressed gas oxygen, which comes in steel or aluminum containers in different sizes for home use and traveling
- Liquid oxygen, which changes liquid into oxygen that you can breathe
- Oxygen concentrators, electrical devices that remove nitrogen from the air, which makes oxygen more concentrated
Questions to ask If your doctor prescribes oxygen include:
- How do I make sure I receive the right amount of oxygen?
- When and how often should I use my oxygen container?
- Which type of oxygen equipment will be best for my lifestyle?
Traveling with Oxygen
Airlines have rules about using oxygen. You cannot travel on an airplane with an oxygen canister. You must use either an airline-supplied oxygen canister or an FAA-certified portable oxygen container. There may be other rules, so check with your airline and/or oxygen supplier at least 2 weeks before you plan to travel.
Lung Transplantation and CTEPH
Although lung transplant is rarely needed for CTEPH, this is still a possible option for some patients with an advanced disease when no other treatment options are working. Lung transplantation should be considered a last resort, because there are other complications associated with lung transplantation, such as infections, kidney failure, and rejection. Although long-term outcomes with lung transplantation are improving, they are still far from ideal. Therefore, it is often a very complex decision and needs to be made along with the family, caregivers, your CTEPH medical team, as well as a transplant team.
There are several organizations and programs to help patients with pulmonary hypertension and chronic thromboembolic pulmonary hypertension as well as their family members and caregivers. The Pulmonary Hypertension Association (www.phassociation.org) helps patients find medical specialists and facilities with expertise in CTEPH and provides access to support groups, including those for patients who have undergone PTE surgery.
Here are other websites that help PH and CTEPH patients get support:
- CTEPH.com http://www.cteph.com/Insights
- American Thoracic Society https://www.thoracic.org/patients/patient-resources/resources/cteph.pdf
- International CTEPH Association Resources https://www.cteph-info.com/en/patients/cteph-resources/overview/
- Cleveland Clinic https://my.clevelandclinic.org/health/articles/what-is-chronic-thromboembolic-pulmonary-hypertension-cteph
- phaware® global association (https://www.phaware.global/)
- phaware® MD (https://www.phawareMD.global/)
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Sandeep Sahay, MD, FCCP
Deborah Jo Levine, MD, MS, FCCP
Jay Peters, MD, FCCP
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