Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) occurs when a pulmonary embolism fails to undergo complete thrombolysis leading to vascular occlusion and pulmonary hypertension. Despite the fact that CTEPH is a potential consequence of pulmonary embolism, diagnosis requires a high degree of vigilance as many patients will not have a history of thromboembolic disease. The ventilation perfusion scan is used to evaluate for the possibility of CTEPH although right heart catheterization and pulmonary artery angiogram are needed to confirm the diagnosis. Pulmonary thromboendarterectomy is the first-line treatment for patients who are surgical candidates. Recently, riociguat has been approved for patients with nonsurgical disease or residual pulmonary hypertension despite surgical intervention. This review describes the pathophysiology, risk factors, diagnosis, and management of CTEPH.
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Alison S. Witkin declares that she has no conflict of interest. Richard N. Channick has been a consultant and received research grants from Bayer Pharmaceuticals, Actelion Pharmaceuticals, and United Therapeutics Corporation.
Human and Animal Rights and Informed Consent
This article does reference studies with human subjects performed by Richard N. Channick. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
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This article is part of the Topical Collection on Peripheral Vascular Disease
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Witkin, A.S., Channick, R.N. Chronic Thromboembolic Pulmonary Hypertension: the End Result of Pulmonary Embolism. Curr Cardiol Rep 17, 63 (2015). https://doi.org/10.1007/s11886-015-0621-9
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DOI: https://doi.org/10.1007/s11886-015-0621-9