Chronic Thromboembolic Pulmonary Hypertension

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Our specialists in heart and lung disease have advanced training in complex procedures to treat chronic thromboembolic pulmonary hypertension (CTEPH). UT Southwestern Medical Center offers comprehensive diagnostic and treatment services with safe, effective care that can successfully cure this rare yet serious disease.

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.

A CT scan of patient with CTEPH. The straight arrow shows a blood clot in a pulmonary artery. The curved arrow shows the segment of lung with reduced or no blood flow caused by the blood clot.

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:

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)

Clots removed from a patient during PTE surgery

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

Treatment for CTEPH

Once we confirm a diagnosis based on tests and imaging, our team of CTEPH experts evaluates each patient’s situation to determine the best treatment plan.

We offer the latest surgical and endovascular (minimally invasive) approaches to treat CTEPH safely and effectively.

Pulmonary thromboendarterectomy (PTE)

In this surgical procedure, a cardiothoracic (heart and lung) surgeon removes the scar-like blockages in the lung arteries. We perform the procedure through a chest incision while the patient’s heart is stopped. A heart-lung machine takes over heart and lung function during the surgery. 

PTE is a specialized surgery performed at only a few hospitals across the country. Our surgical team developed their experience by training with the team that developed PTE. 

Balloon pulmonary angioplasty (BPA)

BPA is a minimally invasive, endovascular procedure for patients who cannot have PTE for a variety of reasons. An interventional radiologist performs this cardiac catheterization procedure, and patients can receive several BPA treatments over a few weeks.

The doctor accesses the lungs via a catheter (thin, flexible tube) inserted into a small incision in the groin area. With advanced imaging technology, doctors can maneuver the catheter directly to the blood clots. The catheter has a tiny balloon at its tip that is inserted into the clogged vessel to open it and restore blood flow.

Clinical Trials

Through clinical trials, UT Southwestern offers treatments for CTEPH that are not yet widely available. Currently, we are offering clinical trials for nonsurgical treatment of CTEPH using medications.

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.