About CTEPH (Chronic Thromboembolic Pulmonary Hypertension)

While most pulmonary embolism (blood clots to the lungs) cases can be effectively treated with blood thinners, about 2% to 3% of cases become chronic.

This can lead to a much more serious condition known as CTEPH, which requires treatment by experienced specialists in heart and lung care. Fortunately, many CTEPH patients can have dramatic responses, or at times be considered cured, with early diagnosis and proper treatment.

What Is CTEPH?

CTEPH stands for chronic thromboembolic pulmonary hypertension.

• Chronic = long term
• Thromboembolic = related to blood clots
• Pulmonary hypertension = high blood pressure in the lungs

How Does CTEPH Happen?

CTEPH is a rare type of pulmonary hypertension caused by old blood clots that don't go away on their own or with blood-thinning medications. These clots block blood flow in the lungs and raise the pressure in the lungs' arteries.

Unlike other forms of pulmonary hypertension, CTEPH can usually be treated with surgery or interventional procedures with dramatic results. Early and accurate diagnosis is especially important for CTEPH.

CTEPH Symptoms

CTEPH can often be treated and cured if caught early. This is why it's important to get diagnosed by an experienced CTEPH team.

CTEPH symptoms include:

• Shortness of breath: You may feel winded doing things that used to be easy, like climbing stairs, walking or even getting dressed.
• Fatigue: CTEPH can make you feel unusually tired or worn out, even if you've had a full night's sleep. Your body is working harder to get oxygen.
• Chest discomfort: You might feel pressure, heaviness or mild pain in your chest, especially during activity.
• Heart racing: You might feel like your heart is racing or pounding, especially when you move around or climb stairs.
• Lightheadedness or dizziness: Some people feel faint or dizzy, especially when bending down or being active. This happens because the heart struggles to pump enough blood through the lungs.
• Swelling in the legs: Fluid can build up in the lower parts of your body, making your legs, ankles or feet swell.

These symptoms can come on slowly, leading people to think they're just "getting out of shape" or "getting older." But if you've had a blood clot in your lungs (a pulmonary embolism) and still don't feel right weeks or months later, ask your doctor about CTEPH.

CTEPH symptoms often worsen over time. They may progress like this:

• Early symptoms: Shortness of breath during exercise and mild fatigue.
• Moderate symptoms: Chest pressure, racing heartbeat and dizziness.
• Advanced symptoms: Leg swelling, weight gain, frequent fainting and severe breathlessness even while resting.

Recognizing these symptoms early can significantly improve treatment outcomes, especially with a specialized care team.

CTEPH Causes and Risk Factors

CTEPH causes are often related to a complication of a pulmonary embolism, especially if it was large or happened more than once. Other common CTEPH risk factors include:

• Previous pulmonary embolism (PE)
• Blood clotting disorders, such as lupus anticoagulant or antiphospholipid syndrome
• Splenectomy for any reason
• Cancer or chronic inflammation
• Indwelling catheter or pacemaker leads
• Other surgery or trauma (which may increase clot risk)

CTEPH Diagnosis

CTEPH (chronic thromboembolic pulmonary hypertension) can be tricky to diagnose because its symptoms — like shortness of breath and fatigue — can resemble many other conditions. Experienced doctors can make a complete CTEPH diagnosis with the following tests:

• Echocardiogram: An echocardiogram, or heart ultrasound, is often the first test used to look for signs of high blood pressure in the lungs or strain on the right side of the heart.
• V/Q scan: A V/Q scan, or ventilation/perfusion scan, shows how well air and blood move through your lungs. If parts of the lung have normal air flow but poor blood flow, it may indicate old blood clots blocking the arteries.
• CT scan or pulmonary angiography: Imaging tests give a detailed look at the blood vessels in the lungs to find blockages or scar tissue from old clots.
• Right heart catheterization: This test measures the actual pressure inside the lungs and heart. It confirms whether pulmonary hypertension is present and how severe it is.
• Pulmonary angiogram: A special dye is injected to show blood flow in the lungs' arteries. This helps identify where the blockages are. This is sometimes performed during a heart catheterization.

CTEPH is treatable — and sometimes curable — especially if caught early. The right tests help your doctors decide whether surgery, medications or other procedures are the best option for you.

CTEPH Treatment Options

CTEPH is one of the few types of pulmonary hypertension that may be curable with proper treatment. Which CTEPH treatment is best depends on overall health, severity of symptoms, and whether surgery is possible.

Pulmonary Thromboendarterectomy (PTE) Surgery

Also called pulmonary endarterectomy (PEA), PTE surgery is a special heart-lung surgery to remove old blood clots. PTE surgery is the most effective treatment for CTEPH and can greatly improve symptoms and even cure the disease. When performed at an experienced center, it is also an extremely safe surgery. However, only a few centers in the world specialize in this surgery. Not every individual is a good candidate for surgery, but if you are, it is usually the first choice.

Balloon Pulmonary Angioplasty (BPA)

A BPA procedure is another form of treatment for CTEPH. BPA uses catheters and balloons to open blocked blood vessels in the lungs. It is a minimally invasive procedure and a great option for those who aren't candidates for PTE surgery or who have residual pulmonary hypertension after PTE. At expert BPA centers, it's often done in four or more sessions under mild sedation.

Medications for CTEPH

Some people with CTEPH may need medicine to lower pressure in the lungs, especially if they aren't good candidates for PTE surgery or BPA, or if they still have symptoms after surgery. Common medications include riociguat (Adempas), the only drug approved specifically for CTEPH.

Lifelong Blood Thinners

Everyone with CTEPH needs to take an anticoagulant, or blood thinner — like warfarin or DOACs (direct oral anticoagulants, such as apixaban) — to prevent new clots and reduce the risk of the condition getting worse.

Supportive Care for CTEPH

Other treatments that can help include:

• Oxygen therapy (if oxygen levels are low)
• Pulmonary rehabilitation to improve strength and breathing
• A healthy diet, exercise and stress management

Living With CTEPH

Chronic thromboembolic pulmonary hypertension can be life-changing, but with the right treatment and daily care, many people live full and active lives. Here's what living with CTEPH can look like and how to manage it day to day. (Note that many of these limitations may not apply to those who respond dramatically to PTE surgery.)

Follow Your Treatment Plan

• Take all medications as prescribed, especially blood thinners.
• Go to follow-up appointments regularly, even if you feel okay.
• Ask your doctor if PTE surgery (pulmonary thromboendarterectomy) or BPA (balloon pulmonary angioplasty) could help you.

Watch Your Breathing

• Let your doctor know if you feel more short of breath than usual.
• Use oxygen therapy if it's prescribed for you.
• Avoid high altitudes unless your doctor says it's safe.

Stay Active, But Pace Yourself

• Regular light activity can improve your quality of life.
• Pulmonary rehab programs can be useful and help build stamina.
• Listen to your body — rest when you need to.

Eat Smart

• Eat a low-salt diet to help prevent fluid buildup.
• Drink plenty of water, unless your doctor tells you to limit fluids.
• Avoid alcohol if you're on blood thinners.

Prevent New Blood Clots

• Take your blood thinner every day — don't skip doses.
• Stay active to keep your blood moving.
• Talk to your doctor before any surgery, travel, or new medication.

Take Care of Your Mental Health

• Living with a chronic illness is hard — it's OK to feel overwhelmed.
• Talk to your doctor, a counselor, or a support group.
• You are not alone — many others live well with CTEPH.

Build a Care Team You Trust

• It's important to see a PH or CTEPH specialist. Don't delay seeking treatment when needed. 
• Ask questions and speak up about your symptoms.
• Keep a list of your medications and medical history with you.

Learn More About How Our Experts Diagnose and Treat CTEPH