Chronic Thromboembolic Pulmonary Hypertension

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

CTEPH is often caused by scar tissue that builds up over time after a pulmonary embolism (a blood clot in the lungs). The scar tissue blocks blood flow to the pulmonary arteries, causing the pressure to increase in these vessels and the right side of the heart to enlarge. Without treatment, the right side of the heart may begin to fail as it becomes overworked.

Fortunately, with the proper diagnosis and treatment, CTEPH is often curable. UT Southwestern Medical Center is the only place in North Texas that provides all of the modern treatment options for CTEPH.

As an accredited Pulmonary Hypertension Comprehensive Care Center and home to one of the largest pulmonary hypertension programs in the country, UT Southwestern offers a dedicated team that is trained not only to perform the complex procedures to cure CTEPH, but also to diagnose and evaluate this often unrecognized condition.

Symptoms

In its early stages, the symptoms of CTEPH can be vague and non-specific, which is why it often goes undiagnosed or misdiagnosed as congestive heart failure, asthma, or chronic obstructive pulmonary disease (COPD).

The main symptom is shortness of breath, especially with light exercise, such as climbing stairs. As the disease progresses, you may feel fatigue or light-headedness. Fainting is a possibility. Eventually, fluid may back up into the abdomen or cause your legs to swell.

Diagnosis

If we suspect that you have CTEPH or another type of pulmonary hypertension (or if you have been referred to us with symptoms of CTEPH), we’ll conduct a series of tests to help us make a diagnosis. These tests will include:

• Lung ventilation-perfusion scan: To compare how blood and air are being distributed in your lungs
• Echocardiogram: An ultrasound to look at the size and function of the right side of the heart
• Right-heart catheterization: A procedure that directly measures pressure in the heart and lungs
• Pulmonary angiogram: Shows blood flow through the lungs and the precise location of blockages in the blood vessels
• Spectral CT scan: A new kind of computed tomography (CT) scanner that can create well-defined images of the pulmonary arteries and show if any blockages are reducing blood flow to the lung tissue

Treatments

Once a diagnosis is made, our team of CTEPH experts evaluates each patient’s situation to determine the best procedure to address it.

We offer surgical and endovascular approaches to treating CTEPH.

Pulmonary thromboendarterectomy (PTE)

This is a surgical procedure in which a cardiothoracic surgeon cleans out the scar tissue-like blockages in the blood vessels of the lungs. We perform the procedure through a chest incision while the patient’s heart is stopped, so that the surgeon can see clearly and clean out the blood vessel completely. A heart-lung machine takes over the function of the heart and lungs during the surgery.

PTE is a specialized surgery performed at only a few hospitals across the country. Our surgical team trained with the team that developed this procedure to bring this life-saving surgical option to North Texas.

Balloon pulmonary angioplasty (BPA)

BPA is a newer endovascular procedure for patients who are not candidates for PTE because their clots are too far out to reach with surgery or if the patient has other health problems that won’t allow them to undergo a major operation. 

BPA is performed in the cardiac catheterization lab with a specialized team including a pulmonary hypertension specialist, a cardiologist, and an interventional radiologist. We will access the lungs via a catheter (long, thin tube) inserted in a large vein in the groin or the side of the neck. With advanced imaging technology, we are able to see the catheter inside the body and maneuver it directly to the clots. The catheter is equipped with a balloon at its tip that we inflate carefully into the clogged vessel to open it and restore blood flow.

This procedure is done is stages. Patients may receive several BPA treatments over a few weeks.

Clinical trials

Through clinical trials, UT Southwestern offers treatments for CTEPH that are not yet widely available. Currently, we’re offering clinical trials for medical (nonsurgical) treatment of CTEPH. We are also part of a national CTEPH registry to help promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of patients with CTEPH.