Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease associated with pulmonary embolism (PE), a life-threatening blood clot that usually forms in a vein deep in the legs and travels to the lungs.
PEs that are not completely resolved by the body or medical treatment can cause CTEPH, a rare form of pulmonary hypertension (PH). CTEPH is believed to occur in up to 5 percent of patients who suffer from an acute PE. Although some people with CTEPH may not remember a specific PE event. People living with CTEPH may be candidates for a pulmonary thromboendarterectomy (PTE), a surgical procedure to remove the chronic clots that can significantly reduce or even normalize blood pressure in the lungs. People living with CTEPH who are not able to have a PTE, and those with PH that remains after surgery, may be candidates for U.S. FDA-approved oral medication.
PH is high blood pressure in the lungs. It is a progressive disease often misdiagnosed — as asthma, for example — that can lead to right heart failure. PH, which affects adults and children of all ages, can exist alone or in association with other conditions, such as scleroderma, lupus and other connective tissue diseases; congenital heart disease; chronic liver disease; HIV; congestive heart failure; COPD; and other illnesses.
There are two tests central to the CTEPH diagnosis – a right heart catheterization and ventilation/perfusion (V/Q) scan.
Manreet Kanwar, M.D., recently led a PHA Facebook Live chat on CTEPH Awareness Day, Nov.21, 6 p.m. ET. As part of PHA’s #TheRightHeart Awareness Month campaign and CTEPH Awareness Day efforts, Dr. Kanwar took questions in her first Facebook Live event. Dr. Kanwar is an Assistant Professor of Medicine, Temple University School of Medicine and the Associate Medical Director of the CTEPH Program at Allegheny General Hospital Pittsburgh Pennsylvania, a PHA Pulmonary Hypertension Care Center (PHCC)-accredited site.
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PHA “PH: The Basics” CME Curriculum Series CTEPH Programs
WHO Group 4 PH (CTEPH) – William R. Auger, MD
WHO Group 4 PH (CTEPH) Treatment: Surgical v. Medical – Richard N. Channick, MD
For more information on all available CME resources, please visit PHA Online University.
PHA News Research Articles
CTEPH Treatment & Surgery Options
The treatment of choice for chronic thromboembolic (CTEPH) patients is pulmonary thromboendarterectomy, or PTE surgery, a highly specialized surgical procedure that removes chronic blood clots from the arteries in the lung. Because pulmonary thromboendarterectomy1 (PTE) is potentially curative, it is the primary treatment for patients with CTEPH. Every patient diagnosed with CTEPH should be evaluated by an expert CTEPH team, including CTEPH physicians and PTE surgeons, to assess their candidacy for PTE surgery. Learn more about PTE surgery
Some patients with CTEPH are not candidates for PTE for various reasons, however inoperability must be determined by a center with experience and expertise in CTEPH and PTE. Medical therapy should not be used in lieu of a careful evaluation for PTE by an expert center, and is only indicated for patients deemed not to be good candidates for PTE or for patients with residual pulmonary hypertension after PTE. For such patients, medical therapies are indicated. Learn more
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 Living Your Best Life with CTEPH: Life After Diagnosis
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.
Dr. Nicholas Smedira, a cardiothoracic surgeon at Cleveland Clinic’s Department of Thoracic and Cardio-Vascular Surgery spoke with the Caring Voice Coalition in early 2014 about PTE as a treatment for CTEPH. Read what he had to share
1Source: Kim NH et al. J Am Coll Cardiol. 2013;62(suppl D):D92-D99.
PHA would like to thank the following sponsor for their support of CTEPH Awareness Day: