Chronic thromboembolic pulmonary hypertension (CTEPH): what do we know about it? A comprehensive review of the literature

Chronic thromboembolic disease (CTED), especially when associated with pulmonary hypertension (CTEPH), is a fearsome and under-diagnosed but potentially curable complication of pulmonary embolism, thanks to recent therapeutic options. Aspecific symptoms and signs make its diagnosis challenging, requiring clinicians to be ready to suspect its presence in patients with dyspnea of unknown origin or persistent symptoms after acute pulmonary embolism. The aim of this review is to provide an update to the general practitioner or the specialist physician on the state of the art regarding the epidemiology, pathophysiology and clinical management of chronic thromboembolic syndromes.