title: Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study.
abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) and idiopathic pulmonary hypertension (IPAH) share a similar clinical presentation, and a differential diagnosis requires a thorough workup. Once CTEPH is confirmed, patients who can be safely operated have to be identified. We investigated risk factors associated with CTEPH and IPAH, and the criteria for the selection of operable CTEPH patients. This case-control study included 436 consecutive patients with CTEPH and 158 with IPAH in eight European centres, between 2006 and 2010. Conditions identified as risk factors for CTEPH included history of acute venous thromboembolism (p < 0.0001), large size of previous pulmonary embolism (p = 0.0040 in univariate analysis), blood groups non-O (p < 0.0001 in univariate analysis), and older age (p = 0.0198), whereas diabetes mellitus (p = 0.0006), female gender (p = 0.0197) and higher mean pulmonary artery pressure (p = 0.0103) were associated with increased likelihood for an IPAH diagnosis. Operability of CTEPH patients was associated with younger age (p = 0.0108), proximal lesions (p ≤ 0.0001), and pulmonary vascular resistance below 1200 dyn.s.cm⁻⁵ (p = 0.0080). Non-operable CTEPH patients tended to be less differentiable from IPAH patients by risk factor analysis than operable patients. This study confirmed the association of CTEPH with history of acute venous thromboembolism and blood groups non-O, and identified diabetes mellitus and higher mean pulmonary artery pressure as factors suggesting an IPAH diagnosis. Non-operable CTEPH is more similar to IPAH than operable CTEPH regarding risk factors.
authors.affiliation: Irene Lang, Department of Cardiology, Medical University of Vienna, Währinger Gürtel 18 - 20, 1090 Vienna, Austria. irene.lang@meduniwien.ac.at
authors.forename: Irene M
authors.initials: IM
authors.lastname: Lang
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Joanna W
authors.initials: JW
authors.lastname: Pepke-Zaba
authors.forename: Eckhard
authors.initials: E
authors.lastname: Mayer
authors.forename: David
authors.initials: D
authors.lastname: Ambrož
authors.forename: Isabel
authors.initials: I
authors.lastname: Blanco
authors.forename: Adam
authors.initials: A
authors.lastname: Torbicki
authors.forename: Sören
authors.initials: S
authors.lastname: Mellemkjaer
authors.forename: Azzedine
authors.initials: A
authors.lastname: Yaici
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D001789:Blood Group Antigens; D016022:Case-Control Studies; D002908:Chronic Disease; D003920:Diabetes Mellitus / Q000453:epidemiology; D005060:Europe; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D011655:Pulmonary Embolism / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D012307:Risk Factors; D012737:Sex Factors; D019616:Thoracic Surgical Procedures; D016896:Treatment Outcome; D054556:Venous Thromboembolism / Q000453:epidemiology*
chemical_list: D001789:Blood Group Antigens