Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism. As for most rare diseases, epidemiologic data are scarce, but recent registries suggest an incidence of at least 5 per million inhabitants per year. A history of massive or recurrent acute pulmonary embolism is observed in most patients with CTEPH, but the proportion of patients who develop CTEPH after acute pulmonary embolism is a matter of debate, further complicated by the possible misdiagnosis of CTEPH as acute pulmonary embolism. A complete resolution of thrombi is usually not achieved after acute pulmonary embolism, and the clinical relevance of a "postpulmonary embolism syndrome" with persistent perfusion defects and exercise intolerance is discussed. Risk factors most consistently associated with CTEPH are circulating anti-phospholipid antibodies or lupus anticoagulant, increased factor VIII, non-O blood groups, and chronic inflammatory diseases. There is no female predominance, and it is a disease of older age. Survival in the absence of specific surgical or medical treatment is poor and depends on the hemodynamic severity.

作者：Marion, Delcroix；Kim, Kerr；Peter, Fedullo

来源：Annals of the American Thoracic Society 2016 年 13 Suppl 3卷