Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a poorly understood disorder. It is characterized by pulmonary hypertension associated with an apparent failure to resolve extensive, typically major-vessel pulmonary thromboemboli. Although CTEPH is believed to be a thromboembolic disease, the typical risk factors for venous thromboembolism are absent. According to the 2003 Venice classification of pulmonary hypertension, CTEPH represents group IV of pulmonary hypertensive diseases and thus does not rank as a subgroup of pulmonary arterial hypertension. Since 2003, however, it is well recognized that CTEPH predominantly results in changes in the major pulmonary artieries, in combination with significant pulmonary vascular disease, which is indistinguishable from classic idiopathic pulmonary hypertension. The present review provides an update on our current knowledge of the pathophysiology, diagnosis, and treatment options for CTEPH.
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Lang, I.M., Klepetko, W. (2007). Chronic Thromboembolic Pulmonary Hypertension. In: Konstantinides, S.V. (eds) Management of Acute Pulmonary Embolism. Contemporary Cardiology. Humana Press. https://doi.org/10.1007/978-1-59745-287-8_19
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