title: Chronic Thromboembolic Pulmonary Hypertension. Epidemiology and Risk Factors.
abstract: 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.
authors: 1 Department of Respiratory Diseases, Katholieke Universiteit Leuven-University of Leuven and University Hospitals Leuven, Leuven, Belgium; and.
authors: Marion
authors: 0000-0001-8394-9809
authors: M
authors: Delcroix
authors: 2 Division of Pulmonary and Critical Care Medicine, University of California San Diego, La Jolla, California.
authors: Kim
authors: K
authors: Kerr
authors: 2 Division of Pulmonary and Critical Care Medicine, University of California San Diego, La Jolla, California.
authors: Peter
authors: P
authors: Fedullo
mesh_terms: D002908:Chronic Disease; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology*; D015994:Incidence; D011655:Pulmonary Embolism / Q000150:complications* / Q000503:physiopathology; D012307:Risk Factors; D054556:Venous Thromboembolism / Q000150:complications* / Q000503:physiopathology
keywords: epidemiology; pulmonary embolism; pulmonary hypertension; risk factors