Current understanding of the pathophysiology of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension arising from fibrotic obliteration of major pulmonary arteries. Pro-thrombotic states, large clot burden and impaired dissolution are believed to contribute to the occurrence and progression of thrombosis after an acute pulmonary embolic event. Recent data utilizing several models have facilitated the understanding of clot resolution. This review summarizes current knowledge on pathophysiological mechanisms of major vessel occlusion in CTEPH.