Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Pulmonary hypertension (PH) relates to high blood pressure in the lung arteries. One particular type, called chronic thromboembolic pulmonary hypertension (CTEPH), is rare but serious in children. It is caused by blood clots in the arteries of the lungs.

Symptoms of CTEPH in Children

When diagnosed early, pediatric CTEPH is often curable. Surgery can be performed to remove blood clots and scar tissue that are causing the problem. If untreated, it can lead to heart failure. It can occur alongside other illnesses, such as sickle cell disease.

If a child gets tired or short of breath easily, their doctor may recommend testing for a range of potential heart and lung concerns. Symptoms of CTEPH may also include a dry cough, chest pain, or dizziness.

Diagnosis of CTEPH

It is important to fully understand what is going on with a child’s heart and lungs in order to find any areas of concern, whether it’s CTEPH or something else. The pulmonary hypertension team at Riley at IU Health may order one or more tests to help determine the cause of your child’s symptoms. These might include:

Chest x-ray: A x-ray can let the medical team see the child’s heart and lungs to get a clearer picture of their health.
Pulmonary function testing: This may include a single test or a series of tests, such as spirometry, lung volumes, diffusion testing, respiratory muscle strength, or treadmill testing.
Echocardiogram: An echocardiogram captures a moving image of the child’s heart as it works. The doctor can review the image to look for evidence of blood clots.
Ventilation/perfusion scan (V/Q): This diagnostic tool tests for blood clots in the lungs by looking at how air and blood flow to the lungs. The doctor will review the resulting images to evaluate the health of the lungs.
Cardiac catheterization: A small, flexible tube is threaded through a vein in the arm, upper thigh, or neck. Special dye is injected to allow the doctor to see how the blood flows through the heart and blood vessels.
Cardiac MRI: Magnetic resonance imaging (MRI) uses strong magnets and radio waves to form detailed images, while the child lies still on a table. These images can help the medical team see greater detail than an x-ray.
CT scan: In a computed tomography (CT) scan, an x-ray beam moves around the body for a 360-degree view. It takes many images of the lungs and inside the chest. The child may be asked to drink a contrast dye in order to help certain areas show up on the images.

Treatments

Surgery for CTEPH

If your child is diagnosed with CTEPH, they may be a candidate for surgery called pulmonary thromboendarterectomy (PTE). It is a complex procedure, and can sound frightening, but it has an excellent long-term survival rate. Without it, blood clots could eventually cut off blood flow to the lungs, causing severe or even fatal damage.

During PTE, a surgeon removes the blood clots from the blood vessels in the lungs. It can take several hours to perform, and the child will need to recover in the hospital for a week to 10 days. They will ease back into regular activity and may need supplemental oxygen for a while.

Following the surgery, the child’s lungs should have normal blood flow through the pulmonary arteries. They will be able to breathe more easily and be more active.

Key Points to Remember

Early diagnosis and treatment are critical.

When recognized early, the disease is often curable with PTE surgery.

Support Services & Resources

Visit the links below to learn more about CTEPH and find support services.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Pulmonary Hypertension Association

The Pulmonary Hypertension Association provides information related to CTEPH as well as patient resources and support.

Locations

In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.