title: High prevalence of elevated clotting factor VIII in chronic thromboembolic pulmonary hypertension.
abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is an enigmatic disorder lacking signs, symptoms and classical risk factors for venous thromboembolism. The objective of the prospective case controlled study, carried out at the Pulmonary Hypertension Unit, University Hospital Vienna, Austria, was to investigate whether plasma FVIII is elevated in CTEPH patients. The study examined 122 consecutive patients diagnosed with CTEPH. Plasma FVIII was measured and compared with plasma FVIII of healthy controls (n = 82) and of patients with nonthromboembolic pulmonary arterial hypertension (PAH, n = 88). Results show that CTEPH patients had higher FVIII levels than controls (233 +/- 83IU/dl versus 123 +/- 40IU/dl, p < 0.0001) and PAH patients (158 +/- 61IU/dl, p < 0.0001). Plasma FVIII one year after surgery (212 +/- 94IU/dl) was statistically unchanged compared with preoperative values (FVIII: 226 +/- 88IU/dl, n = 25). FVIII > 230IU/dl was more prevalent in CTEPH patients (41%) than in controls (5%, p < 0.0001) and PAH patients (22%, p = 0.022). We can conclude that elevated plasma FVIII is the first prothrombotic factor identified in a large proportion of CTEPH patients.
authors.affiliation: Department of Internal Medicine II, Division of Cardiology, University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. irene.lang@univie.ac.at
authors.forename: Diana
authors.initials: D
authors.lastname: Bonderman
authors.forename: Peter L
authors.initials: PL
authors.lastname: Turecek
authors.forename: Johannes
authors.initials: J
authors.lastname: Jakowitsch
authors.forename: Ansgar
authors.initials: A
authors.lastname: Weltermann
authors.forename: Christopher
authors.initials: C
authors.lastname: Adlbrecht
authors.forename: Barbara
authors.initials: B
authors.lastname: Schneider
authors.forename: Meinhard
authors.initials: M
authors.lastname: Kneussl
authors.forename: Lewis J
authors.initials: LJ
authors.lastname: Rubin
authors.forename: Paul A
authors.initials: PA
authors.lastname: Kyrle
authors.forename: Walter
authors.initials: W
authors.lastname: Klepetko
authors.forename: Gerald
authors.initials: G
authors.lastname: Maurer
authors.forename: Irene M
authors.initials: IM
authors.lastname: Lang
mesh_terms: D000328:Adult; D000368:Aged; D001789:Blood Group Antigens; D016022:Case-Control Studies; D002908:Chronic Disease; D005169:Factor VIII / Q000032:analysis*; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011184:Postoperative Period; D015995:Prevalence; D011446:Prospective Studies; D011655:Pulmonary Embolism / Q000097:blood* / Q000503:physiopathology; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D001789:Blood Group Antigens; D014841:von Willebrand Factor; D005169:Factor VIII