Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Last Updated 11/02/2020
Authors:Sandeep Sahay, MD, FCCP; Deborah Jo Levine, MD, MS, FCCP; and Jay Peters, MD, FCCP
About Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease.
- The leading most common 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.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive form of pulmonary hypertension (PH). CTEPH is caused by blood clots that don’t dissolve in the lungs. These clots cause scar-like tissue that clogs up or narrows the small blood vessels in the lungs.
How CTEPH affects your body
To better understand CTEPH, you first need to understand the name:
- Chronic means a condition that lasts a long time, like months or years.
- Thromboembolic means that a blood clot, or 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—in this case, it refers to high blood pressure in your lungs.
Next, you need to know how your lungs contribute to the normal flow of blood in your body. Your 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 your 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, your health care provider is measuring the pressure that pushes your blood throughout your body.
After the blood delivers the oxygen, it returns through veins to the right side of your heart, which pumps the blood to your lungs. The pump in the right side of the heart doesn’t need a lot of pressure because the heart and lung are so close together. This pressure is called pulmonary pressure, and it is measured differently from the blood pressure in the left side of your 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).
CTEPH is a rare and progressive form of PH. In CTEPH, repeated blood clots in the lung get stuck and clog the arteries, which develop scars over time. As the blood vessels become narrower and more clogged, pulmonary pressure grows and PH results. This means that the right side of your heart has to pump harder—using more pressure—to get blood to pass through.
PH also leads to a back-up of blood returning from the veins to the heart. As a result, the right side of the heart has work too hard to keep up with the blood flow. This extra work can weaken the heart, making it swell and, over time, leading to heart failure. Because it takes longer for blood to travel through the lungs, the oxygen level in the blood can drop too low. Oxygen is important to cells and tissue, and your brain needs lots of oxygen to be healthy and function properly. Without treatment, patients with CTEPH will die within 5 years. That’s why diagnosing and treating CTEPH is critical.
Measuring blood pressure
If you’re like most people, you’ve had your blood pressure checked with a blood pressure cuff. This cuff measures the pressure in the left side of your heart in millimeters of mercury (or mm Hg). Your blood pressure measurement has two parts:
- Systolic. The larger number is the measure of the pressure when your heart beats.
- Diastolic. The smaller number is the measure of the pressure when your heart rests between beats.
A normal blood pressure level is 120 (systolic) over 80 (diastolic).
In the right side of your heart, the pressure measures the blood vessels in the lungs, not your heart. This measure is your pulmonary artery pressure (PAP). Normal pulmonary artery pressure is 8 to 20 mm Hg at rest. If this measure is higher than 25 mm Hg at rest or 30 mm Hg during physical activity, it is considered high and qualifies as PH.
What causes CTEPH?
Some common causes of CTEPH include:
- A history of one or more blood clots in the lungs
- High blood pressure at the time a blood clot is diagnosed
- Having PH for at least 6 months after a PE (a blood clot in the lung) has been diagnosed
Symptoms of CTEPH
CTEPH is difficult to diagnose because it has symptoms similar to other diseases, including:
Common symptoms of CTEPH include:
- Shortness of breath, especially during exercise or when climbing stairs
- Fatigue (tiredness)
- Swelling of the legs
- Tightness in the chest or belly
- Palpitations (heart pounding in the chest)
- In rare cases, coughing up blood
What are the risk factors for CTEPH?
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 (called hypercoagulability) disorders
If your health care provider thinks that you may have CTEPH, he or she will order several tests. These tests must first prove that you have PH, and then prove that you have CTEPH. The following tests are used to diagnose CTEPH:
- Ventilation-perfusion (V/Q) scan. This test measures how well air and blood flow into your lungs. The scan creates pictures that your health care team look at to see if you have blood clots in your lungs. A V/Q scan is the best screening test for diagnosing CTEPH.
- Echocardiogram. This test is a noninvasive ultrasound scan of your heart. It looks at the areas of your 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 doesn’t directly measure PAP, but it does give your health care team an idea of whether you have PH and how severe it is.
- Lung function tests. These simple tests measure how much air you can hold in your lungs and how much air moves in and out of your lungs. Lung function tests help your health care team know whether lung tissue is diseased.
- Right-sided heart catheterization. If any of the tests suggest PH, your provider will likely order a right-sided heart catheterization. This test best shows which of the five forms of PH you have. For this test, the doctor places a small tube (or catheter) into a large vein in your neck, arm, or groin, advances it to the right side of your heart, and then out into the blood vessels that connect to the lung. The doctor measures the pressure in different areas of the heart and your pulmonary artery. These measurements can help your provider know how severe your PH is and what has caused it.
- Pulmonary angiogram. This test takes an X-ray of the blood vessels in your lungs. A dye injected into your veins makes them show up on the X-rays. A pulmonary angiogram helps providers find blood clots and blocked blood vessels in the lungs, which in turn helps them identify treatment options.
- Computed tomography (CT, or “CAT”) scan pulmonary angiograph. This test is similar to a pulmonary angiogram, but instead of X-ray images it shows cross-sectional (thin section) images of your lungs. A CT pulmonary angiograph gives your provider a better picture of what’s happening in your lungs and where the blood clots are.
- Magnetic resonance imaging (MRI). An MRI scanner uses magnets and radio waves to create a detailed image. MRI makes it easy for providers to see which parts of your body are normal and which parts are not. In some patients, MRI may reveal chronic blood clots in the lungs but no evidence of high blood pressure in the lungs at rest—a condition referred to as chronic thromboembolic disease.
- Exercise tolerance test (also known as a 6-minute walk test). This test compares your exercise capacity, oxygen levels, and symptoms over time to evaluate how they change over time and with therapy.
The best and preferred treatment for CTEPH is a surgical procedure called pulmonary thromboendarterectomy (PTE) or pulmonary endarterectomy. PTE is a very delicate surgery and should only be performed in centers that have doctors experienced in the procedure. If your health care provider doesn’t think that you are a candidate for PTE, it’s worthwhile to seek a second opinion.
Treating CTEPH with surgery (PTE)
During the PTE procedure, first, you’re put on a heart-lung bypass machine to provide blood flow to your other organs. Then, your heart is stopped while the surgeon removes all clots from the arteries in your lung.
The good news is that PTE surgery results in significant improvement in most patients and a return to normal PAP. It’s important that you follow up with your provider closely after this surgery so that he or she can monitor you for increases in PAP and determine the best treatment if you have PH following surgery.
If you can’t have PTE, you may be a candidate for a newer treatment called balloon pulmonary angioplasty (BPA). In this procedure, the doctor uses a tiny balloon that he or she inflates inside the pulmonary artery to widen it. This treatment can lower PAP and help reduce strain on the right side of your heart.
In addition, medications are available if you can’t have surgery or still have some PH after PTE.
- Make sure your provider sends you to a medical center that has doctors experienced 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)
Treating CTEPH with surgery (lung transplantation)
Although lung transplant is rarely needed for CTEPH, it’s still an option for some patients with advanced disease when no other treatment options are working. Lung transplantation should be considered a last resort because there significant complications are associated with it, such as infections, kidney failure, and rejection. Although long-term outcomes with lung transplantation are improving, they are still far from ideal. Therefore, it’s often a complex decision that should be made with family, caregivers, your health care team, and the transplant team.
Treating CTEPH with medication
Medications should be used only in patients who are not candidates for surgery or who have persistent PH after surgery. Riociguat is the only medication specifically approved by the US Food and Drug Administration for PH, although other medications can be used, if necessary.
All patients with CTEPH, including those who successfully undergo PTE surgery or BPA, should take anticoagulants for the rest of their lives to keep new blood clots from forming. Talk to your provider about the best medication to take as well as their risks and side effects.
Self-care and CTEPH
If you have CTEPH, you can take steps to make your life easier. Many patients change their lifestyle to include exercise, nutrition, and support groups. You may also need to take precautions when you travel.
Exercise is a great way to rebuild, make you stronger, and help you breathe 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 by offering:
- 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 to strengthen muscles that decrease shortness of breath
Because PR can improve quality of life for many people with CTEPH, be sure ask your provider 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 decrease your strength and cause other physical problems. Other people with CTEPH may be overweight, which adds strain to their heart and lungs. Talk to your provider 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 provider about how much liquid you should drink each day so that you don’t get too much fluid.
- Eat slowly, and chew your food thoroughly.
- Make sure you’re on a low-salt diet.
Depression and Anxiety
Many people with CTEPH may feel depressed or anxious. In fact, depression is the rule—not the exception—for patients with complex medical problems like CTEPH. It can also be hard to keep up with personal relationships and maintain regular levels of activity.
For some people, medication helps with depression, especially if the person wants to hurt him- or herself. Treatments for depression and anxiety other than medication include talk therapy, exercise, and meetings with a support group. Never be afraid to talk to your health care team about your feelings.
A 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 you and your family.
It can be hard to travel with CTEPH, so it’s important that you make plans in advance:
- Review your travel plans with your health care team to see if you need special supplies, medication, or extra oxygen. Even if you don’t usually need oxygen, you may need it if you’re traveling to a high-altitude location.
- Know where to get medication and oxygen on your trip.
- 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, which are electric devices that remove nitrogen from the air and make oxygen more concentrated
Questions to ask If your provider 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?
Airlines have rules about using oxygen on planes. You cannot travel on an airplane with an oxygen canister. You must use either an airline-supplied oxygen canister or a US Federal Aviation Administration–certified portable oxygen container. There may be other rules, as well, so check with your airline or oxygen supplier at least 2 weeks before you plan to travel.
There are several organizations and programs to help not only patients with PH and CTEPH but their family members and caregivers. The Pulmonary Hypertension Association helps patients find medical specialists and facilities with expertise in CTEPH. It also provides access to support groups, including for patients who have undergone PTE surgery.
The following websites also help patients with PH and CTEPH find support:
- American Thoracic Society
- International CTEPH Association Resources
- Cleveland Clinic
- phaware® global association
I’m Aware That I’m Rare: the phaware® podcast
A new podcast series from phaware global association is devoted to raising global awareness of PH through dynamic stories from patients with PH, caregivers, and medical professionals from around the world. Subscribe to this series of impactful, insightful, and hopeful conversations with members of the global PH community.