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Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Symptoms and Treatment

Overview
Diagnosis
Treatment
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What Is CTEPH?

CTEPH — or chronic thromboembolic pulmonary hypertension — is a rare, life-threatening condition.

Old blood clots in the lungs (pulmonary emboli) causes scar tissue to build up. This tissue blocks the pulmonary arteries, causing high blood pressure in the lungs.

Doctors treat most people who’ve had a blood clot with blood thinners. These drugs restore blood flow to the lungs, which helps improve their breathing and exercise tolerance.

But in others, blood clots might not dissolve. This can lead to progressive right-sided heart failure and/or pulmonary hypertension (PH) — increased blood pressure in the lung.

The presence of PH in the setting of old blood clots is called CTEPH. It's the only form of PH that's potentially curable.

CTEPH causes and risk factors

A pulmonary embolism (PE) is a blood clot that travels from the leg to the lungs and gets lodged there. 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 (such as a long flight or car trip, or after surgery or a hospital stay).
Obesity.
Cancer.

If a PE goes untreated or doesn’t get better with blood thinners, the clot can become chronic and scar-like.

When this happens, blood backs up in the lungs and causes increased pressures. In some cases, a rare but often treatable type of severe pulmonary hypertension known as CTEPH can form.

CTEPH may also develop from many small clots over a long time, versus 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.

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.

You may also have swelling in the stomach and lower limbs. This occurs as your lung pressures increase and the right ventricle — which pumps blood into the lungs — starts 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 for CTEPH include:

Ventilation/perfusion or “V/Q” scan. This test shows areas in the lung that aren't receiving proper blood flow due to clots blocking the arteries. It's the preferred screening study to look for obstructive pulmonary vascular disease. Obstructions change little beyond 12 weeks after a clot. As a result, they can be permanent and chronic beyond this time.
Echocardiogram. Also known as a heart sonogram, this is a painless test to help doctors make a preliminary diagnosis. It estimates the pressures in the right heart and assesses how well the heart is functioning.
CT scan or CT angiogram. This test is looks for blood clots and evaluates the condition of the rest of the lung besides the arteries. If you get a CT scan, doctors will inject your vein with dye and take pictures of your lungs. This test can help confirm if chronic blood clots are the cause of pulmonary hypertension (PH). It can also tell if surgery would likely improve pulmonary pressures.
Right-heart catheterization. This is the only test that directly measures the pressure inside the pulmonary arteries. Doctors should use this test in all patients at least once to confirm their diagnosis of PH. During the test, doctors insert a thin rubber tube (catheter) through a large vein in your groin or neck. They then pass the tip of the tube up into your heart and arteries in the lungs. This measures the blood pressure in the right side of the heart and lungs. Doctors will order a right-heart cath with exercise for people who have shortness of breath largely with exertional activities. This test will measure pulmonary pressures while at rest and with exercise.
Pulmonary angiography. Like a right-heart cath, doctors place a small tube in a neck (or groin) vein into the pulmonary arteries. They then inject dye into the arteries and take pictures. This test can also confirm if chronic blood clots are the cause of PH and whether surgery would help.
IVC (inferior vena cava) filter placement. The IVC is the main vein that returns blood from your legs to your heart. You'll have this procedure in the radiology department. A doctor will place a stainless steel filter in the IVC and your body will not react to it. This filter will help prevent any clots in the leg veins from reaching the lungs. A doctor will place a stainless steel filter in the IVC through a vein in your groin or neck. Since it’s steel, your body will not react to it. The doctor will use numbing medications then pass the filter through a small incision via a wire. They'll take an x-ray to confirm its proper placement. You'll need to stay in bed 4 to 6 hours after IVC filter placement. Many times, doctors use removable filters that they can take out soon after you’ve finished treatment.
Coronary angiography or left-heart cath. People who are candidates for surgery and at risk for coronary artery disease (CAD) will need this test. It shows if you have significant CAD — blockages in the arteries that supply the heart with blood and oxygen. In this case, doctors will treat it before or at the time they remove blood clots from the lung’s arteries.
Carotid Doppler. This non-invasive exam evaluates blood flow through the carotid arteries in your neck. These major arteries supply blood flow to the brain. Doctors often order the study in older people or those with a history of stroke. It tells them whether these vessels are fully opened. The tech uses a transducer — a hand-held device that uses sound waves to create pictures. They’ll check the size of the artery and blood flow patterns. There's no numbing or premedication required for this procedure. In CTEPH, this test helps doctors assess the risk of surgery in those 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

Because this disease is so rare and has similar symptoms to other cardiopulmonary conditions, seeing a CTEPH expert is vital. UPMC's Comprehensive Pulmonary Hypertension Program is one of only a few nationwide specializing in treatment of CTEPH.

Diagnosing and treating this disease requires the kind of expertise that is the hallmark of our doctors. With proper treatment, survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.

CTEPH is potentially curable. Our team is well-skilled in providing treatment options best suited for each person, even at advanced stages of disease.

Pulmonary thromboendarterectomy (PTE) or endarterectomy (PEA)

Doctors perform this complex surgery with cardiopulmonary bypass where they:

Cool your body temperature.
Stop blood flow for a short time to allow for complete visualization.
Remove 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 you without emboli and 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) to treat CTEPH

For people with inoperable CTEPH or those with residual pulmonary hypertension after surgery, BPA might be an option.

UPMC is one of a few centers in the country offering this minimally invasive procedure treatment for CTEPH.

The technique for BPA is similar to a right-heart cath, where doctors:

Place a catheter with a balloon at its tip into the pulmonary artery.
Inflate the balloon in the area where the chronic blood clots are located. This pushes the clots against the wall of the artery and restores blood flow.

BPA can complement surgery and/or medicine to improve the quality of life of people with CTEPH.

Medications for CTEPH

For people who do not achieve a cure from PTE or are not surgical candidates, medications are a treatment option.

Lung transplant

Some people may be candidates for a lung transplant.

Our doctors are leading experts in lung transplantation. We’re part of the UPMC Thomas E. Starzl Transplantation Institute, a nationally recognized center of excellence in transplants.

Refer a patient for CTEPH treatment