title: Chronic thromboembolic pulmonary hypertension: an updated review.
abstract: OBJECTIVE
  Venous thromboembolism is a spectrum of disease comprising deep vein thrombosis, thrombus in transit, acute pulmonary embolism, and chronic thromboembolic pulmonary hypertension (CTEPH) as a rare and late possible sequela.
  
  
  RESULTS
  On the basis of a prospective long-term study, the incidence of CTEPH is estimated at 3.8% within 2 years of all patients surviving an episode of symptomatic idiopathic pulmonary embolism. Young age, a large perfusion defect, and idiopathic clinical presentation are associated with a higher probability of CTEPH. Current pathophysiological concepts suggest a misguided thrombus resolution process that is triggered by infection, inflammation, autoimmunity, and malignancy. Diagnosis and therapy of CTEPH are interdisciplinary achievements and nowadays still based on a positive lung perfusion scan and for assessment of operability on a classical pulmonary angiography. Treatment of choice is surgical pulmonary endarterectomy of the pulmonary obstructions, which leads to restoration of normal pulmonary hemodynamics at rest in nearly 80% of patients. In expert centers, surgical mortality is under 10%.
  
  
  CONCLUSIONS
  CTEPH has emerged as a 'dual' pulmonary vascular disorder with major vessel vascular remodeling of thrombus organization, combined with a small vessel pulmonary arteriopathy that is a target for classic vasodilator treatments.
authors.affiliation: Department of Internal Medicine II, Division of Cardiology, Austria. irene.lang@meduniwien.ac.at
authors.forename: Irene M
authors.initials: IM
authors.lastname: Lang
authors.forename: Walter
authors.initials: W
authors.lastname: Klepetko
mesh_terms: D000792:Angiography; D002908:Chronic Disease; D004691:Endarterectomy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D011655:Pulmonary Embolism / Q000150:complications / Q000503:physiopathology* / Q000601:surgery; D054556:Venous Thromboembolism / Q000150:complications / Q000503:physiopathology* / Q000601:surgery