Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of high blood pressure in the lungs.

It’s a complication of long-term pulmonary embolism, or a blood clot in the vessels of the lungs. The clot increases blood pressure in the vessels, resulting in high blood pressure, known as pulmonary hypertension.

CTEPH is a “silent” condition, meaning it causes no symptoms in the early stages. It’s also life threatening. However, CTEPH can be cured, so it’s important to get treatment as soon as possible.

Read on to learn about the symptoms, causes, and treatment options of the condition.

CTEPH develops when a pulmonary embolism stays in your body.

A pulmonary embolism happens when a blood clot from another part of your body travels to your lungs. The clot often comes from your leg veins.

In your lungs, the clot blocks your blood vessels, which increases resistance in your blood. This causes pulmonary hypertension.

The clot can be dissolved with treatment. But without treatment, the clot remains attached to your blood vessel walls. It also turns into a substance that’s similar to scar tissue.

Over time, the clot continues to decrease blood flow to your lungs. It might also make other blood vessels in your body become narrow.

As a result, your lungs are unable to receive oxygen-rich blood. This causes damage to your lungs and other organs in your body. It can eventually lead to heart failure.

Often, CTEPH causes no symptoms in the early stages. It typically causes symptoms as it progresses.

Symptoms of CTEPH may include:

In later stages, CTEPH might make it difficult for you to do any physical activity.

CTEPH is a type of pulmonary hypertension that’s due to chronic blood clots. The exact cause is unknown, but it may be related to:

  • Large or recurring pulmonary embolism. CTEPH may be a complication of a large or recurring pulmonary embolism. However, about 25 percent of people with CTEPH have no history of pulmonary embolism.
  • Deep vein thrombosis. Often, pulmonary embolism is caused by deep vein thrombosis, or a blood clot in a deep vein.
  • Primary arteriopathy. Arteriopathy, or disease of the arteries, may lead to CTEPH.
  • Endothelial dysfunction. This occurs when your blood vessels become impaired and no longer tighten and relax, which can lead to coronary artery disease.

The following risk factors increase the risk of CTEPH:

Sometimes, CTEPH can develop without any underlying causes or risk factors.

Since early CTEPH usually causes no symptoms, it’s difficult to diagnose. CTEPH also develops slowly.

To diagnose CTEPH, a doctor will likely use several techniques:

  • Medical history. A doctor will ask questions about your symptoms.
  • Physical exam. This lets a doctor check for unusual pain, swelling, and other symptoms.
  • Chest X-ray. A chest X-ray uses radiation to create an image of your lungs, heart, blood vessels, and surrounding structures.
  • Echocardiography. This imaging test is an ultrasound of your heart. It lets a doctor examine the blood flow in your heart.
  • Ventilation/perfusion scan. Also known as a VQ scan, this test measures air flow and blood flow in your lungs.
  • Pulmonary angiography. This is a test that checks the blood vessels in your lungs. It might involve a CT or MRI to create an image of your blood vessels.
  • Right heart catheterization (RHC). An RHC is used to examine how well your heart is pumping. The combination of an RHC and pulmonary angiography is the gold standard for diagnosing CTEPH.

CTEPH is a life threatening condition. It’s important to receive treatment as soon as possible. Treatment may include:

Medical therapy

Medical therapy for CTEPH includes:

You may need to continue medical therapy after getting surgery.

If you’re unable to get surgery, you can take a medication called riociguat. You might also need to take this drug if the disease persists after getting surgery.

Pulmonary thromboendarterectomy

A pulmonary thromboendarterectomy, or pulmonary endarterectomy, is a surgery that removes blood clots from your lungs. It’s the preferred treatment for CTEPH.

The procedure is complex and is performed at select hospitals in the country. A doctor can determine if the surgery is right for you.

Pulmonary balloon angioplasty

If you’re not a good candidate for surgery, a pulmonary balloon angioplasty might be a better choice.

This procedure uses balloons to widen blood vessels that have become blocked or narrow. This promotes blood flow into your lungs and improves breathing.

CTEPH is rare. It affects about 1 to 5 percent of people who have pulmonary embolism. In the general population, it affects between 3 to 30 out of every 1 million people.

However, a 2019 review notes that CTEPH is often underdiagnosed. This is due to the nonspecific symptoms of the disease. This means the actual prevalence of CTEPH might be different from the recorded prevalence.

If you’ve been diagnosed with a pulmonary embolism, visit a doctor as needed. This way, a doctor can monitor your condition and provide the proper treatment.

You should also see a doctor if you have the following symptoms:

Without treatment, CTEPH can result in death in 1 to 3 years.

However, it’s possible to cure CTEPH. Pulmonary thromboendarterectomy is the best treatment option, as it can cure up to two-thirds of CTEPH cases.

This can improve long-term survival, breathing, and exercise tolerance. The 10-year survival rate is more than 70 percent.

About 10 to 15 percent of people may still experience symptoms after surgery. This will require additional treatment and management to prevent organ failure.

It’s possible to prevent CTEPH by lowering the risk of pulmonary embolism.

The following methods can help lower your risk:

  • If you’re prone to blood clots, visit a doctor routinely. Take your medication as directed.
  • If you’ve been diagnosed with pulmonary embolism, follow a doctor’s instructions for treatment.
  • Use compression stockings or inflatable compression devices during long hospital stays.
  • If you travel often, change your sitting position frequently. Try to walk every 1 or 2 hours.

CTEPH is a major complication of a pulmonary embolism, or a blood clot in the lungs. It’s a rare and life threatening condition, but it can be cured.

CTEPH is associated with large or recurring pulmonary embolisms and blood clotting disorders. Other risk factors include thyroid disease, spleen removal, inflammatory bowel disease, cancer, and having an infected pacemaker.

Surgery to remove the blood clot is the best method of treatment. It can improve long-term survival and quality of life.