What Is Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

Pulmonary hypertension is high blood pressure in the arteries and blood vessels of your lungs. The elevated blood pressure strains the heart and limits its ability to pump blood through your lungs.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of prolonged pulmonary hypertension. CTEPH develops when pulmonary hypertension causes blood clots in the arteries of the lungs. These blood clots lead to scar tissue which blocks normal blood flow. When the amount of blood flowing to the lungs is reduced or slowed, oxygen levels can decline and damage other organs in the body.

Fewer than 10,000 cases of CTEPH are diagnosed each year in the United States. CTEPH is a rare but serious condition that can lead to complications including heart failure. Without treatment, CTEPH can be life-threatening.

What Are the Causes of CTEPH?

Recurrent and persistent blood clots in the lung’s blood vessels and arteries cause pulmonary hypertension to become CTEPH. Scar tissue narrows the blood vessels, leading to high blood pressure.

What Are the Symptoms of CTEPH?

The primary symptom of CTEPH is shortness of breath with exertion or exercise. Patients may also experience:

  • Chest tightness
  • Chest pain
  • Lightheadedness, dizziness or fainting
  • Fatigue
  • Heart palpitations
  • Swelling in the legs, feet or abdomen
  • Bluish color in the fingers or toes
  • Coughing up blood

What Are the Risk Factors of CTEPH and Can It Be Prevented?

People with the following characteristics may be at an increased risk of developing CTEPH:

  • Pulmonary hypertension
  • History of blood clots in the lungs
  • Prior heart surgery or heart procedure
  • Prior heart infection
  • Thyroid hormone therapy

It may not be possible to prevent CTEPH, but making healthy lifestyle choices can enhance your heart and lung health. Avoiding smoking is important.

How Is CTEPH Diagnosed?

Patients with pulmonary hypertension, a history of blood clots and symptoms like shortness of breath may need testing for CTEPH. Diagnostic testing can include:

  • Lung ventilation-perfusion scan (V/Q scan)
  • Pulmonary function test (breathing test)
  • Echocardiogram
  • Pulmonary angiogram
  • Pulmonary CT
  • Pulmonary MRI
  • Exercise stress test
  • Right-heart catheterization

What Treatments Are Available for CTEPH?

Medications can help reduce symptoms, but a procedure is typically necessary to treat CTEPH.

Medication

Prescription medication to control blood pressure by dilating pulmonary arteries and blood vessels may alleviate some symptoms of CTEPH. Blood thinners help prevent new blood clots from forming.

Oxygen Therapy

Some CTEPH patients need supplemental oxygen until a procedure can be performed.

Minimally Invasive Procedure or Surgery

Two procedures are used to treat CTEPH, and both have high success rates.

Balloon Pulmonary Angioplasty

This is a minimally invasive procedure to expand narrowed pulmonary arteries. A catheter with a medical-grade balloon inside is inserted into the pulmonary artery. Once it reaches the narrowed portion of the artery, the balloon is inflated which compresses scar tissue and leaves a wider space for blood flow. When blood can flow more freely, blood pressure comes down.

Pulmonary Thromboendarterectomy

Also called pulmonary endarterectomy or PTE, pulmonary thromboendarterectomy is a surgical procedure to remove blood clots from the pulmonary arteries and blood vessels. An incision is made in the chest to reach the narrowed portion of the pulmonary arteries, and the blood clots and scar tissue are removed.

After undergoing either of these options, patients will need to take blood thinners indefinitely. Health care providers may also recommend pulmonary rehabilitation to help rebuild strength.

Specialized Care for Rare Conditions

The heart and pulmonary specialists affiliated with Memorial Hermann are experienced at treating rare conditions like CTEPH. Our care teams are proficient in leading-edge therapies to treat patients with serious conditions that are not often seen in other health systems. With an interdisciplinary approach to care, Memorial Hermann brings different specialists together to find the most appropriate treatment for CTEPH patients to recover and live healthier lives.

Contact Us

If you’d like a referral to a heart and vascular specialist or want more information about our services, please fill out the form below. For other inquiries, such as obtaining medical records, imaging reports or test results, please call (713) 222-CARE (2273).

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You!

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