Chronic Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the arteries in your lungs. You can get it after you've had a pulmonary embolism (PE) -- a blood clot in your lung. There are about 5,000 new cases a year. Around 1% to 5% of people who've had a PE will get it.

Symptoms

Doctors often find it hard to diagnose CTEPH because the symptoms are a lot like ones they see in other diseases. Typically, symptoms come on slowly. They may include:

  • Shortness of breath, especially during exercise
  • Fatigue
  • Weakness
  • Pain or pounding in your chest
  • Swelling in your legs (edema)
  • Bluish fingers or toes (cyanosis)
  • Fainting

Rarely, CTEPH can also make you cough up blood.

Causes and Risk Factors

Scarring from blood clots is behind CTEPH. When you get blood clots in your lungs, they can turn into scar tissue. Scar tissue makes blood vessels narrower, or can even block them. Blocked or narrowed arteries make it harder for your heart to pump blood through them. This raises your blood pressure. Your heart muscle can also get weaker over time.

Things that raise your chances of a blood clot in your lungs include:

If you already have pulmonary hypertension before you get a blood clot in your lung, you're more likely to get CTEPH. Your risk also goes up if you have one or more of the following:

Certain types of cancers, blood diseases, or inflammatory diseases can raise the chances that you’ll get it, too.

Diagnosis

CTEPH can be hard to diagnose. There are tests that can help:

  • Ventilation/perfusion scan (V/Q scan). This is the go-to test that doctors use as a screen for the condition. Doctors inject radioactive protein into your bloodstream so they can track how blood flows through your lungs.
  • CT scan. A computed tomography scan looks for blood clots. You get an injection of dye through an IV to help doctors see them.
  • Echocardiogram. Doctors take a sonogram of your heart to take pressure readings from its right side and look at how well it's squeezing and relaxing.
  • Right-heart catheterization. This procedure involves a small tube called a catheter that doctors put into a vein in your groin, neck, or wrist. The doctor guides the tube toward the right side of your heart and then into your lung arteries. They take pressure readings as it goes.
  • Pulmonary angiogram. A pulmonary angiogram starts the same as a right-heart catheterization. Once the tube is in the lung arteries, the doctor injects dye into them and takes X-rays to see where the clots are.

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Treatment

The most common way to treat CTEPH is through surgery. A pulmonary thromboendarterectomy (PTE), also called a pulmonary endarterectomy, cures the condition for over 90% of people who have it.

During the surgery, a surgeon stops your heart, and a heart-lung bypass machine takes over to move your blood. The surgeon then removes the clots from your lungs.

Since the surgery is tricky, it's best to have it done by a surgeon who's familiar with it. If you don't have access to one, or have other medical conditions that make the surgery risky, there are other treatments. A balloon pulmonary angioplasty is a procedure where doctors inflate a tiny balloon inside your lung arteries. This helps widen them to lower the pressure. There are also medications your doctor can prescribe to help treat it if you can't have surgery.

Prevention

To help keep your risk of CTEPH low, your doctor will likely put you on anticoagulants, or blood thinners. These medicines help stop clots from forming in your blood. A low-salt diet can also help lower your blood pressure. Doctor-approved exercise is also key to helping you manage your blood pressure and keep your heart and lungs healthy.

WebMD Medical Reference Reviewed by Paul Boyce, MD on April 01, 2020

Sources

SOURCES:

American Lung Association: “Pulmonary Arterial Hypertension (PAH).”

Cleveland Clinic: "Chronic Thromboembolic Pulmonary Hypertension (CTEPH),” “Pulmonary Thromboendarterectomy.”

UpToDate: "Clinical manifestations and diagnosis of chronic thromboembolic pulmonary hypertension."

Harvard Health Publishing: “Ventilation-Perfusion Scan or V-Q Scan”

Mayo Clinic: "Pulmonary hypertension."

Pulmonary Hypertension Association: "CTEPH."

European Respiratory Review: “Medical management of chronic thromboembolic pulmonary hypertension.”

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