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 heart may begin to fail as it becomes overworked.

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

As a 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 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 and 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 can include:

  • Lung ventilation-perfusion scan – to check how well blood is moving through the lungs
  • Echocardiogram – an ultrasound of the heart
  • Right-heart catheterization – measures pressure in the heart and lungs, which indicates if the heart is sufficiently pumping blood
  • Pulmonary angiogram – shows blood flow through the lungs or blockages in the blood vessels
  • CT scan – helps locate blood clots 

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. 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. 

Balloon pulmonary angioplasty (BPA)

BPA is a newer endovascular procedure for patients who are not candidates for PTE because their clots are surgically inaccessible or if they have other health problems that won’t allow them to undergo a major operation. 

BPA is performed in the cardiac catheterization lab and led by an interventional radiologist. We will access the lungs via a catheter (long, thin tube) inserted in your groin. With advanced imaging technology, we are able to see the catheter inside your body and maneuver it directly to the clots. The catheter is equipped with a balloon at its tip that we insert into the clogged vessel to open it and restore blood flow.

You 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.

Our Team

Our CTEPH physician team includes:

Request an Appointment

To schedule an appointment with a CTEPH specialist at UT Southwestern’s facilities in Dallas or for more information about our services, request an appointment or call 214-645-8300.