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Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Symptoms and Treatment​

What Is CTEPH?

CTEPH — or chronic thromboembolic pulmonary hypertension — is a rare, life-threatening medical condition typically caused by old blood clots in the lungs (pulmonary emboli).

Most people who have suffered a blood clot, can be treated with blood thinners to restore blood flow to the lungs, which helps improve their breathing and exercise tolerance. However in others, blood clots might not dissolve and can lead to progressive right-sided heart failure and/or development of pulmonary hypertension (increased blood pressure in the lung).

The presence of pulmonary hypertension in the setting of old blood clots is referred as CTEPH. CTEPH is considered the only form of pulmonary hypertension that is potentially curable.

CTEPH causes and risk factors

A blood clot in the lungs is known as a pulmonary embolism (PE), which can become lodged there andulate other clots. PE can block blood flow into the lungs and can be life threatening. These blood clots usually form in the legs that break loose and then travel to the lung.

Lifestyle and health issues that can increase the risk of getting blood clots include:

  • Smoking
  • Inactivity for long periods of time (such as after surgery or hospitalization, or a long flight or car trip)
  • Obesity
  • Cancer

If a PE goes untreated, or if it does not get better with blood thinning therapy, the clot can become chronic and scar-like over time. When this happens, blood backs up in the lungs and causes increased pressures, and a rare but often treatable type of severe pulmonary hypertension known as CTEPH can develop.

It's also possible for CTEPH to develop from multiple small clots over a long period of time, as opposed to one or two large blood clots.

People without evidence of prior pulmonary clot can still develop CTEPH.

CTEPH risk factors include:

  • Ventricular shunts (heart shunts)
  • Indwelling catheters
  • Spleen removal
  • Thyroid replacement therapy
  • Inflammatory bowel disease
  • A history of cancer

CTEPH is more common in women and in those with a family history of blood clots.

Refer a patient to UPMC's CTEPH Program

Call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.

CTEPH Symptoms and Diagnosis

Symptoms of CTEPH

CTEPH symptoms are non-specific, and similar to other types of pulmonary hypertension and cardiopulmonary conditions like:

  • Pneumonia,
  • Heart failure
  • COPD (chronic obstructive pulmonary disease)

It often goes undiagnosed or is detected late in the advancement of the disease.

The most common symptoms of CTEPH include:

  • Fatigue
  • Shortness of breath, especially with exercise

Less common symptoms include:

  • Chest pain
  • Heart palpitations or fluttering
  • Dry cough
  • Swelling of the lower extremities or stomach (edema)

As the disease progresses, symptoms of light-headedness or exercise-related dizziness may occur.

Stomach and lower extremity swelling may develop as your lung pressures increase, and the right ventricle — which pumps blood into the lungs — begins to weaken.

Diagnosing CTEPH

Pulmonary emboli (PE) are quite common with approximately 600,000 diagnosed each year. Among people with PE, CTEPH affects approximately 2 to 4 percent of those individuals. About 5,000 Americans are diagnosed with CTEPH each year.

Additionally, almost one third of people diagnosed with CTEPH have no known history of PE.

To determine the best route for treatment, a UPMC specialist will:

  • Ask about your symptoms
  • Evaluate your medical history
  • Perform a physical exam

Testing is essential to the diagnosis and evaluation of CTEPH.

Common tests include:

  • Ventilation/Perfusion Scan or “V/Q Scan” — this test shows any areas in the lung that are not receiving the appropriate amount of blood flow due to blockage of the arteries by clots. It is the preferred screening study to look for obstructive pulmonary vascular disease. Obstructions change little beyond 12 weeks after a clot, and as a result, the obstructions can be assumed to be permanent and chronic beyond this time.
  • Echocardiogram — also known as a heart sonogram, this painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning.
  • CT scan or CT angiogram — this test is to look for blood clots and to evaluate the condition of the rest of the lung besides the arteries. If you get a CT scan, your vein will be injected with dye and then pictures will be taken of your lungs. This test also can help confirm if chronic blood clots are the cause of the pulmonary hypertension and whether surgery would likely improve pulmonary pressures.
  • Right-heart catheterization — this is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient's diagnosis of pulmonary hypertension (PH). During the test, doctors insert a catheter (a thin rubber tube) through a large vein in the patient's groin or neck. They then pass the tip of the catheter up into the patient's heart and arteries in the lungs to measure the blood pressure in the right side of the heart and lungs. In some patients if shortness of breath is largely with exertional activities, a right heart catheterization with exercise, where pulmonary pressures are measured at rest and with exercise, will be ordered.
  • Pulmonary angiography — like a right-heart catheterization, a catheter is placed through a vein in the neck (or groin) into the pulmonary arteries. This is done by injecting dye into the arteries and taking pictures. This is also a test used to confirm if chronic blood clots are the cause of the pulmonary hypertension and whether surgery would likely improve pulmonary pressures.
  • IVC filter placement — this procedure is performed in the radiology department. A filter is placed in the inferior vena cava (IVC) which is the main vein that returns blood from your legs to your heart. This filter will help prevent any clots in the leg veins from reaching the lungs. It is made of stainless steel and your body will not react to it. The filter will be inserted through a vein in your groin or your neck by a physician. Numbing medications will be used and then a small incision is made to pass the filter via a wire. You will be asked to stay in bed 4-6 hours after the procedure. An x-ray will be taken to confirm its proper placement. Many times removable filters are now used that can be taken out soon after treatment has been completed.
  • Coronary angiography or left heart catheterization — People who are deemed candidates for surgery and at risk for coronary artery disease will also undergo this test to see if they have significant coronary artery disease (blockages in the arteries that supply the heart muscle with blood and oxygen). If the doctors identify significant coronary artery disease, they will typically treat it prior to or at the time that they remove blood clots from the arteries in the lung.
  • Carotid Doppler — a carotid Doppler exam is a method of non-invasively evaluating blood flow through the carotid arteries, the major arteries in your neck, which supply blood flow to the brain. The study is usually performed in older patients or in those with a history of stroke to determine whether these vessels are fully opened. The tech uses a transducer, similar to an echocardiogram transducer, to examine the size of the artery and blood flow patterns. There is no numbing or premedication required for this procedure. In CTEPH evaluation this test is used to help evaluate risk of surgery in a population at higher risk of complications.

Refer a patient for CTEPH symptoms and care

To make a referral to UPMC's CTEPH program, call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.

CTEPH Treatment at UPMC

UPMC's Comprehensive Pulmonary Hypertension Program is one of only a few programs nationwide specializing in treatment of CTEPH. Because this disease is so rare and has similar symptoms to other cardiopulmonary conditions, diagnosing and treating this disease requires the kind of multi-disciplinary expertise that is the hallmark of our physicians.

This disease is potentially curable, and UPMC's experts are well-skilled in providing treatment options that are best suited for each person, even at advanced stages of disease. When properly treated, the survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.

Pulmonary Thromboendarterectomy (PTE)

Also called Pulmonary endarterectomy (PEA). In this complex surgical procedure performed with cardiopulmonary bypass, the patient’s body temperature is cooled and cessation of blood flow is sustained for a short period of time to allow for complete visualization and removal of the chronic pulmonary emboli. The goal is to restore normal blood flow to the lungs and prevent heart failure and death. This surgery can potentially cure CTEPH, leaving the patient without emboli and without the need for pulmonary vasodilator therapy. The success of the surgery depends on the combination of proper patient selection as well as surgical expertise and technique. PTE is considered safe with an experienced team.

Balloon Pulmonary Artery Angioplasty (BPA)

For patients with inoperable CTEPH or those with residual pulmonary hypertension after surgery, this minimallyinvasive procedure might 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 catheter is introduced into the pulmonary artery with a balloon at its tip. 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 do not achieve a cure from PTE or are not surgical candidates, medications are a treatment option. 

Lung Transplantation 

Some patients may be candidates for a lung transplant. Our physicians are leading experts in lung transplantation as part of the UPMC Thomas E. Starzl Transplantation Institute, a nationally recognized center of excellence in transplantation. 

Refer a patient for CTEPH treatment

To make a referral to UPMC's CTEPH program, call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.

Contact the CTEPH Program at UPMC

Meet the CTEPH team

Belinda Rivera-Lebron, MD, MS
Assistant Professor of Medicine
Division of Pulmonary, Allergy & Critical Care Medicine
riveralebronbn@upmc.edu

Other members of the multidisciplinary team:

How to refer a patient to the CTEPH Program

Call 1-877-PH4-UPMC (1-877-744-8762), toll free or email us at PHprogram@upmc.edu.