Chronic Thromboembolic Pulmonary Hypertension

Expert Care for Chronic High Blood Pressure in the Lungs

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension, or high blood pressure in the lung, or pulmonary, arteries. 

CTEPH can develop after a pulmonary embolism (a blood clot in the lungs. The clot forms scar-like tissue that blocks blood flow to the lung arteries. Blood pressure then increases in these arteries, and the right side of the heart enlarges. Without treatment, the heart can begin to fail as it becomes overworked.

As a Pulmonary Hypertension Center of Comprehensive Care, UT Southwestern is home to one of the largest pulmonary hypertension programs in the country. We have a dedicated team that is trained to diagnose and evaluate this often overlooked condition and perform the complex procedures to cure it.

Causes and Risk Factors of CTEPH

CTEPH results from chronic blood clots in the lungs (pulmonary embolisms) that block or narrow the lung arteries. Certain factors can increase the risk of pulmonary embolism, such as:

• Surgeries, such as hip replacement or knee replacement
• Family or personal medical history of blood clots
• Health conditions, such as heart disease, cancer, and peripheral vascular disease
• Extended periods of inactivity, such as bed rest or long car or plane trips
• Obesity
• Older age
• Smoking 

After a pulmonary embolism, the blood clot will usually break down over weeks to months, leaving little or no remaining blockage. CTEPH develops when the blood clot does not break down. 

Risk factors for CTEPH include:

• History of pulmonary embolism
• Recurring blood clots
• Health conditions, such as blood disorders or inflammatory diseases
• History of cancer

Symptoms of CTEPH

In its early stages, CTEPH might not produce any symptoms. When they do appear, symptoms can be vague, such as shortness of breath with light exercise. Other common symptoms include:

• Bluish lips and skin (cyanosis)
• Chest pressure or pain (angina), especially with exertion
• Dizziness
• Enlarged abdomen
• Fainting (syncope)
• Fatigue
• Rapid pulse or irregular heartbeat
• Reduced ability to exercise
• Swelling in the ankles or legs (edema)

Diagnosis of CTEPH

Our skilled pulmonologists have extensive experience in evaluating symptoms to confirm a diagnosis. We begin with a thorough evaluation that includes a:

• Physical exam
• Review of personal and family medical history
• Discussion of symptoms

If we suspect that a patient has CTEPH or another type of pulmonary hypertension, we conduct two tests that can rule out CTEPH, when the results are normal. These tests are:

• Ventilation/perfusion (VQ) scan to check for blood clots and to evaluate blood flow through the lungs
• Echocardiogram, or echo, to see how well the heart is beating and pumping blood and to evaluate blood pressure in the lung arteries

If the VQ and echo test results show that CTEPH might be present, our doctors will recommend additional tests, such as:

• Computed tomography (CT) scan to produce 3D images of the chest that can show blood clots in lung arteries, using specialized X-ray technology that takes cross-sectional images
• Pulmonary angiogram to show blood flow through the lungs or blockages in the blood vessels
• Right-heart catheterization to measure pressure in the heart and lungs, which indicates whether the heart is sufficiently pumping blood