title: Von Willebrand factor antigen for detection of hepatopulmonary syndrome in patients with cirrhosis.
abstract: OBJECTIVE
  Hepatopulmonary syndrome (HPS) occurs in 20-30% of patients with liver cirrhosis and is associated with a >2 fold increased mortality. Endothelial dysfunction seems to play a central role in its pathogenesis. von Willebrand factor antigen (vWF-Ag), an established marker of endothelial dysfunction, is significantly elevated in patients with liver cirrhosis, portal hypertension, and in experimental HPS. Aim of the present study was to evaluate the impact of vWF-Ag as a screening marker for presence of HPS in patients with stable cirrhosis.
  
  
  METHODS
  145 patients with stable liver cirrhosis were screened for presence of HPS in this prospective cohort type cross sectional diagnostic study. vWF-Ag and SaO2 levels were assessed at time of screening for HPS. Criteria of HPS were fulfilled in 31 (21%) patients.
  
  
  RESULTS
  vWF-Ag levels were significantly higher in patients with HPS compared to patients without HPS (p<0.001). Furthermore, vWF-Ag correlated significantly with gas exchange in HPS positive patients (p<0.05). vWF-Ag is an independent predictor of HPS after correction for sex, age, model for endstage-liver disease (MELD), and hepatic venous pressure gradient (HVPG) (OR per 1% increase of vWF-Ag: 1.02, 95% CI: 1.00-1.04, p<0.05). The best cut-off was 328% at a sensitivity of 100% and specificity of 53.5%; positive predictive value: 36.9%; negative predictive value: 100%.
  
  
  CONCLUSIONS
  HPS is associated with elevated vWF-Ag levels. vWF-Ag may be a useful screening tool for early detection of HPS. Further studies investigating vWF-Ag in HPS will be needed to confirm our findings.
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria; Department of Intensive Care Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Horvatits
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria; Department of Intensive Care Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Drolz
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Kevin
authors.initials: K
authors.lastname: Roedl
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Harald
authors.initials: H
authors.lastname: Herkner
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Arnulf
authors.initials: A
authors.lastname: Ferlitsch
authors.affiliation: Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria.
authors.forename: Thomas
authors.initials: T
authors.lastname: Perkmann
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Müller
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Michael
authors.initials: M
authors.lastname: Trauner
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Peter
authors.initials: P
authors.lastname: Schenk
authors.affiliation: Department of Internal Medicine 3, Division of Gastroenterology & Hepatology, Medical University of Vienna, Vienna, Austria; Department of Intensive Care Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. Electronic address: valentin.fuhrmann@meduniwien.ac.at.
authors.forename: Valentin
authors.initials: V
authors.lastname: Fuhrmann
mesh_terms: D000368:Aged; D015415:Biomarkers / Q000097:blood; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004730:Endothelium, Vascular / Q000503:physiopathology; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications* / Q000503:physiopathology; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012680:Sensitivity and Specificity; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D015415:Biomarkers; C029417:Von Willebrand antigen; D014841:von Willebrand Factor
keywords: HPS; Hepatopulmonary syndrome; Pulmonary vascular disease; vWF-Ag; von Willebrand factor