Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare type of pulmonary hypertension (high blood pressure in the arteries of your lungs called the pulmonary arteries). CTEPH occurs after a condition called pulmonary embolism, or a blood clot in your lungs.
When a pulmonary embolism doesn’t completely go away after treatment, it is known as a chronic pulmonary embolism. A chronic pulmonary embolism can block blood flow in your pulmonary arteries, causing CTEPH. If left untreated, CTEPH can lead to right ventricular failure and death.
UPMC has one of only five programs nationwide specializing in the treatment of CTEPH. UPMC's experts are skilled in diagnosing and treating CTEPH, offering personalized treatment options tailored to each person’s condition and needs.
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What Is Chronic Thromboembolic Pulmonary Hypertension?
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare type of pulmonary hypertension (high blood pressure in the arteries of your lungs called the pulmonary arteries). CTEPH occurs after a condition called pulmonary embolism, or a blood clot in your lungs.
When a pulmonary embolism doesn’t completely go away after treatment, it is known as a chronic pulmonary embolism. A chronic pulmonary embolism can block blood flow in your pulmonary arteries, causing CTEPH. If left untreated, CTEPH can lead to right ventricular failure and death.
How common is CTEPH?
CTEPH is rare. It affects 2-4 percent of people who are diagnosed with pulmonary embolism.
The American Lung Association estimates that about 3,000 people per year are diagnosed with CTEPH in the U.S. However, since this condition is difficult to diagnose, the actual number may be higher.
What causes CTEPH?
CTEPH occurs after you develop a pulmonary embolism, or a blood clot in your lungs. If the blood clot doesn’t go away completely, it can become chronic and turn into scar-like tissue that can block blood flow in your pulmonary arteries. Reduced blood flow in your pulmonary arteries can lead to high blood pressure in your lungs known as pulmonary hypertension.
CTEPH risk factors
Having a history of pulmonary embolism is the primary risk factor for CTEPH. Other risk factors include:
- A history of cancer.
- A personal or family history of blood clots in the legs.
- Bone infection (osteomyelitis).
- Heart infections caused by a pacemaker or another device.
- Inflammatory bowel disease and other chronic gastrointestinal issues.
- Prior spleen removal.
- Taking thyroid hormone replacement medications.
Complications of CTEPH
If left untreated, CTEPH can be life-threatening. When the pulmonary arteries are blocked or narrowed, the right side of your heart has to work harder to supply blood to your lungs. The increased workload for your heart can lead to right ventricular heart failure. CTEPH can also cause low blood oxygen levels that lead to organ damage over time.
How can I prevent CTEPH?
There is nothing you can do to prevent CTEPH. However, you can decrease your risk by following your treatment plan after a pulmonary embolism diagnosis. You can also take steps to reduce your risk of developing blood clots, including getting regular exercise, maintaining a healthy weight, and eating a balanced diet.
What Are the Signs and Symptoms of CTEPH?
CTEPH symptoms are often similar to those of other cardiopulmonary conditions like pneumonia, heart failure, or COPD, so it often goes undiagnosed or is detected late in the advancement of the disease.
The symptoms of CTEPH include:
- Chest pain.
- Fatigue.
- Shortness of breath, especially with exercise.
- Swelling of the lower extremities or abdomen (edema).
When should I see a doctor about my CTEPH symptoms?
CTEPH is often mistaken for other cardiopulmonary conditions. However, an accurate, early diagnosis can improve treatment outcomes. If you have symptoms of CTEPH — especially if you have a history of pulmonary embolism — schedule an appointment with your doctor as soon as possible.
If you have severe symptoms, dial 911 or go to the nearest hospital Emergency Department.
How Do You Diagnose CTEPH?
Your doctor will perform a physical exam, review your health history and medications, and order tests to diagnose CTEPH.
What to expect during your visit
If your doctor suspects CTEPH, he or she will:
- Ask about your symptoms.
- Order tests.
- Perform a physical exam.
- Review your health history and medications.
Tests to diagnose CTEPH
Doctors diagnose CTEPH using tests including:
- Cardiac catheterization — This test is a diagnostic procedure that allows your doctor to see blockages, blood flow, and other problems within your arteries.
- Coronary CT angiography (CCTA) imaging — Also known as a CT angiogram, this test uses computed tomography (CT) to look at the blood flow in your coronary arteries.
- CT scans — CT scans create images of your lungs and heart, as well as other bones, organs, and tissues inside your body.
- Echocardiogram — Also known as an ECHO, this noninvasive heart ultrasound procedure assesses the function and structures of your heart.
- Lung function tests — Checks to see how well your lungs are working.
- Pulmonary angiography — Shows images of your lungs using a contrast agent to highlight your blood vessels.
- Six-minute walk test — Shows how far you can walk and measures your oxygen levels during exercise.
- Ventilation/perfusion scan (V/Q scan) — Measures air and blood flow in your lungs.
CTEPH prognosis
When left untreated, CTEPH has a poor prognosis that can shorten life expectancy. However, CTEPH is curable. When properly diagnosed and treated, the survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.
How Do You Treat CTEPH?
Effective CTEPH treatment requires multidisciplinary care from a team of experts. Treatment options for CTEPH may include:
Pulmonary thromboendarterectomy (PTE)
Also called pulmonary endarterectomy (PEA), this complex surgical procedure can provide a cure for CTEPH by removing blood clots, restoring normal blood flow to the lungs, and preventing heart failure and death.
It is performed with cardiopulmonary bypass, which uses a machine to temporarily take over heart and lung function during surgery. During the procedure, the patient’s body temperature is cooled, and blood flow is temporarily stopped to allow for the complete removal of the chronic blood clot in the lungs.
After the blood clot is removed, most people no longer need pulmonary vasodilator therapy — which widens the blood vessels in the lungs and improves blood flow — to treat pulmonary hypertension.
Balloon pulmonary artery angioplasty (BPA)
For patients with inoperable CTEPH or those with residual pulmonary hypertension after surgery, minimally invasive balloon pulmonary artery angioplasty may be an option.
UPMC is one of very few centers in the country offering this treatment. The technique for BPA is similar to a right-heart catheterization, where a balloon-tipped catheter is inserted into a blood vessel in the leg and guided to the pulmonary artery. The balloon is inflated in the area where the chronic blood clots are located, pushing the clots against the wall of the artery and restoring blood flow. BPA can complement surgery and/or medical therapy to improve the quality of life of CTEPH patients.
Medications
For patients who are not surgical candidates, medications are a treatment option. Medications may include pulmonary vasodilator therapy, which widens the blood vessels in the lungs and improves blood flow.
Lung transplantation
Some patients may be candidates for a lung transplant. Our lung transplant experts are part of the UPMC's nationally recognized center of excellence in transplantation.
Why Choose UPMC for CTEPH Care?
When you choose UPMC for CTEPH care, you will receive:
- Nationally recognized expertise — UPMC is one of only five centers nationwide specializing in the treatment of CTEPH.
- Advanced diagnostic and treatment capabilities — Our pulmonary hypertension are skilled in the diagnosis and treatment of CTEPH, taking a multidisciplinary, personalized approach to each person’s care.
- Experience in advanced procedures — UPMC is a national leader in PTE surgery and one of the few centers in the country to offer BPA. People who require a lung transplant have access to advanced transplantation care from one of the country’s highest-volume lung transplant programs.
