Chronic Thromboembolic Pulmonary Hypertension

My doctor says I have chronic thromboembolic pulmonary hypertension. What is this?

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by old blood clots in the lungs (pulmonary embolism). In most patients who have suffered a blood clot, blood thinners are enough to restore blood flow to the lungs, improving breathing and exercise tolerance, and preventing development of pulmonary hypertension.

However, a minority of patients will not respond adequately to blood thinners, and may develop CTEPH. In these patients, problems arise not only from lung segments being obstructed by clots, but because seemingly healthy blood vessels (supplying uninvolved lung segments) may develop progressive narrowing, similar to pulmonary arterial hypertension (PAH).

Importantly, patients may develop CTEPH without any history of a blood clot. It is 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. Learn more, watch Chronic Thromboembolic Pulmonary Hypertension - What You Need to Know.

What are the symptoms of CTEPH?

The symptoms of CTEPH are non-specific, and similar to other types of pulmonary hypertension. The most common symptoms of CTEPH are shortness of breath and exercise intolerance. Less frequent symptoms include a dry cough, chest pain, and heart palpitations.  As the disease progresses, symptoms of light-headedness or exercise-related dizziness may occur. Abdominal and lower extremity swelling may develop as lung pressures increase, and the right ventricle, which pumps blood into the lungs, begins to weaken. 

How common is CTEPH and who gets it?

Approximately 1% of patients with an acute pulmonary embolism will develop CTEPH. People who have very large pulmonary emboli may be at increased risk of developing CTEPH.  Patients who already manifest some pulmonary hypertension at the time of diagnosis of a pulmonary embolism may also have an increased likelihood of developing CTEPH. Some underlying blood clotting disorders are more common in patients with CTEPH, though not all clotting disorders increase risk. Additionally, some chronic medical conditions, such as inflammatory bowel disease and osteomyelitis, are associated with increased CTEPH risk.

How is CTEPH diagnosed?

If a doctor suspects that you have CTEPH, he or she will run a number of tests. The goals of these tests are to establish whether you have CTEPH or another type of PH. If you do have CTEPH, he or she will want to determine if you are a candidate for pulmonary thromboendartectomy (PTE) surgery.

Common tests include:

  1. Nuclear Scan (a.k.a. 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.
  2. Echocardiogram. In this procedure, electrodes are placed on the patient’s skin and a sonogram of the heart is taken. 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.
  3. CT scan. Some patients undergo this test 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 veins will be injected with dye and photographed.
  4. 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 with 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 catheter up into the patient's heart to measure the blood pressure in the right side of the heart and lungs.
  5. 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 test will confirm if chronic blood clots are the cause of the pulmonary hypertension and if PTE surgery would improve pulmonary pressures.
  6. Coronary angiography. Patients who are deemed candidates for PTE surgery and are 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 when they remove blood clots from the arteries in the lung.

What are the differences and similarities between CTEPH and other types of PH?

The symptoms of CTEPH are quite similar to those of pulmonary arterial hypertension (PAH) and the other types of PH. Chest pain may be a more prominent feature of CTEPH, particularly in those patients who have had scarring in an area of lung from a prior blood clot. The distinguishing feature of CTEPH is that it is the only type of PH that truly has a potential “cure” for those who are eligible for PTE surgery.

What are the treatments for CTEPH?

The therapy of choice for operable patients is a pulmonary thromboendarterectomy, or PTE surgery, a highly specialized surgical procedure that removes chronic blood clots from the arteries in the lung. Learn more

All CTEPH patients will need to be on a blood thinner, typically warfarin, for the rest of their lives. For patients who have prominent abdominal swelling and swollen legs, diuretics may be beneficial.

PH-specific medications have been investigated in CTEPH, though the number of patients studied is much smaller than those enrolled in PAH clinical trials. These studies have confirmed the safety of several therapies, and varying degrees of benefit. Medical therapies are mainly used for inoperable patients, or for patients who have residual pulmonary hypertension after surgery. If you fall into one of these categories, your PH team will work with you to choose a medication based on your disease severity, side effects, and other relevant personal factors. Watch Thromboembolic Disease and PAH: The Spectrum of Therapies.

Where can I find support?

One of our PH Email Mentors, Amanda, is available to talk about her experiences as a CTEPH patient and with PTE surgery. Contact Amanda@PHAMentors.org.

Join PHA CTEPH, PHA’s email group for patients living with CTEPH or having had PTE surgery. (Membership requires a free Google account.)

Connect with other patients on PHA CTEPH, PHA's Facebook Group for those affected by CTEPH.

This article was written by Gautam V. Ramani, MD, Assistant Professor of Medicine at the University of Maryland School of Medicine, and Myung H. Park, MD, FACC, Director of the Pulmonary Vascular Diseases Program at the University of Maryland Heart Center.

To review Conflict of Interest Disclosures for PHA's medical leadership, visit: Disclosures
Last reviewed: February 2012

 

Connect with a PHriend

Amanda

Amanda is living with CTEPH. She runs a local PH support group and works full-time. She is also a PHA PHriend - which means she is here to help and support you - if you have any questions or concerns about living with PH and lupus. Email Amanda today

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