CTEPH

CTEPH symptoms often start slowly and can be easy to overlook. Early symptoms may be attributed to aging or being out of shape, or mistaken for asthma, anemia, or other heart or lung conditions.

Common early symptoms include:

As CTEPH gets worse, symptoms may include:

Some people feel better after PE treatment at first. Later, breathlessness or fatigue can come back or get worse. These symptoms can have more than one cause, but persistent, worsening, or returning symptoms deserve medical evaluation.

CTEPH can be difficult to diagnose because its symptoms overlap with many heart and lung conditions. No single test can confirm CTEPH. Instead, your care team uses a combination of tests to reach a diagnosis.

Your clinician will ask about your symptoms, previous blood clots, medications, medical conditions, and how your activity level has changed. The exam may include listening to your heart and lungs and checking for leg swelling or other signs that the right side of your heart is under strain.

An echocardiogram is an ultrasound of the heart. It can show whether the right side of the heart is enlarged or working harder than expected. It can also estimate whether pressure in the pulmonary arteries may be high. An echocardiogram cannot confirm CTEPH or show where old blood clots are.

A ventilation-perfusion scan (V/Q scan) compares airflow and blood flow in the lungs. Areas that get air but have little blood flow might mean there is a blockage in a pulmonary artery. A normal V/Q scan makes CTEPH very unlikely. A V/Q scan is an important screening test even if you’ve already had a computed tomography (CT) scan. Some chronic or smaller blockages can be difficult to identify on a standard CT scan.

CT pulmonary angiography creates detailed images of the pulmonary arteries. It can show scar tissue, webs, narrowed vessels, complete blockages, and other signs of old clotting.

The images also help your care team see where the disease is and which treatments may be possible.

Right heart catheterization directly measures pressure inside the heart and pulmonary arteries. It confirms whether pulmonary hypertension is present and shows how severely it affects blood flow. During the procedure, a thin tube called a catheter is guided through a blood vessel into the right side of the heart and the pulmonary arteries.

Pulmonary angiography provides detailed images of blood flow through the pulmonary arteries. It’s used when other imaging is unclear or when clinicians are planning surgery or balloon pulmonary angioplasty.

Because CTEPH diagnosis and treatment planning are complex, people with suspected or confirmed CTEPH should be evaluated by a team of specialists from different fields, such as pulmonology, cardiology, radiology, and surgery, working together at a center with experience treating the condition.

Treatment aims to improve blood flow through the lungs, lower pressure in the pulmonary arteries, protect the heart, and ease symptoms. The best approach depends on where the blockages are, how much small-vessel disease is present, how severe the pulmonary hypertension is, and overall health. Many people receive more than one type of treatment.

Lifelong anticoagulation, commonly called blood-thinning treatment, is generally recommended for people with CTEPH. These medications lower the risk of new blood clots. They don’t dissolve the scar tissue that causes CTEPH.

The type of anticoagulant used depends on your medical history, other medications, clotting conditions, and bleeding risk. People with antiphospholipid syndrome may require a specific type of anticoagulant.

Pulmonary endarterectomy (PTE) is surgery to remove chronic clot material and scar tissue from the pulmonary arteries. It’s the preferred treatment when the blockages can be reached safely through surgery. In many people, it greatly lowers pulmonary artery pressure, improves symptoms, and helps the right side of the heart recover.

PTE is considered potentially curative, but not everyone is a candidate. Some people still have pulmonary hypertension after surgery because of remaining blockages or disease in the smaller blood vessels.

Determining whether surgery is possible requires evaluation by an experienced CTEPH team. Because this assessment is highly specialized, it’s typically performed at centers with expertise in CTEPH and PTE.

Balloon pulmonary angioplasty (BPA) is a procedure that uses a catheter to open narrowed pulmonary arteries. A clinician guides a small balloon into a blocked or narrowed vessel and gently inflates it to improve blood flow. Because only part of the lung is treated at one time, BPA usually requires several sessions.

BPA may be considered when:

BPA should be performed at a center experienced in the procedure.

Riociguat is a medication approved for people with CTEPH who cannot have surgery or who still have pulmonary hypertension after surgery. It helps relax the pulmonary arteries and may improve blood flow, exercise ability, and symptoms. Specialists may sometimes use other PH medications, but they aren't all specifically approved for CTEPH.

Medications can help control the disease, but they don’t remove the actual blockages.

Supportive treatments may also help manage symptoms and improve quality of life. Some people need supplemental oxygen if the oxygen level in their blood is low. Diuretics may be used to reduce swelling caused by fluid buildup. Pulmonary or cardiac rehabilitation may also help improve strength, activity tolerance, and quality of life.

CTEPH requires long-term care, even after successful surgery or BPA. Regular follow-up helps your care team monitor heart function, pulmonary artery pressure, symptoms, and how much activity you're able to do.

Physical activity can improve endurance and quality of life when CTEPH is stable and appropriately treated. Your clinician can help determine what level of activity is safe for you. You may be referred to a supervised pulmonary or cardiac rehabilitation program, especially when starting exercise or recovering from treatment.

Avoid suddenly starting strenuous exercise or heavy lifting without guidance. Stop and seek medical advice if activity causes chest pain, severe breathlessness, lightheadedness, or fainting.

Don’t stop or change your anticoagulant without speaking with your clinician. Tell your care team about unusual bleeding, falls, planned procedures, new medications, and supplements. Some medications and supplements can interact with anticoagulants.

Many people with well-managed CTEPH can travel safely. However, air travel and higher altitudes may lower oxygen levels and place additional strain on the heart and lungs. Before traveling, ask your clinician whether you need supplemental oxygen or any other travel precautions.

Carry the following items with you rather than in checked luggage:

Pregnancy places major stress on the heart and lungs and can be extremely dangerous for people with pulmonary hypertension. Discuss pregnancy, contraception, and family-planning options with your CTEPH team before trying to conceive. Some medications used to treat CTEPH can cause serious harm during pregnancy.

Respiratory infections can place added strain on the lungs and heart. Ask your clinician which vaccines are recommended for you, including influenza, pneumococcal, and COVID-19 vaccines.

Living with CTEPH can affect emotional well-being. Anxiety, depression, and frustration are common, and support is available. Counseling, medication, rehabilitation, and support groups may help. Connecting with other people who understand CTEPH can also reduce isolation and offer practical support.

Contact your clinician if you develop:

Seek urgent medical care for:

With specialized treatment, regular follow-up, and the right support, many people with CTEPH experience meaningful improvements in symptoms, daily activities, and quality of life.