title: Endothelial cell dysfunction correlates differentially with survival in primary and secondary pulmonary hypertension.
abstract: BACKGROUND
  Plasma von Willebrand factor antigen (vWF:Ag) has been used as a marker of endothelial perturbation in a number of vascular disorders. In this study, vWF:Ag was determined as an attempt to evaluate the severity of endothelial cell dysfunction in primary pulmonary hypertension (PPH) and congenital heart disease-associated pulmonary hypertension (CHD-PH) comparatively and to determine its impact on short-term survival.
  
  
  RESULTS
  Clinical, hemodynamic, and biochemical data were obtained from 11 patients with PPH and 24 with CHD-PH. Patient groups were similar in terms of age and pulmonary artery pressure. vWF:Ag was measured by electroimmunodiffusion. Patients were followed up for 1 year and at that time, data collected at the beginning of the study were subjected to univariate and multivariate analyses. vWF:Ag was increased in patients (normal reference value 87% +/- 23% activity, mean +/- SD), with higher levels in the PPH group (231% +/- 89%) in comparison with the CHD-PH group (127% +/- 68%) (P <.001). Multivariate analysis showed that survival was influenced by the underlying cause of pulmonary hypertension and vWF:Ag levels but not by patient age, sex, or pulmonary artery pressure. Seven of 10 nonsurvivors but only 4 of 25 survivors had PPH (P =.007). vWF:Ag was 255% +/- 90% in the nonsurvivor group and 121% +/- 54% in the survivors (P <.001).
  
  
  CONCLUSIONS
  Our findings suggest that short-term survival is related to the severity of endothelial cell dysfunction in pulmonary hypertension. In addition, exceedingly high vWF:Ag levels in PPH might reflect a particular pattern of endothelial cell dysfunction that could be associated with decreased short-term life expectancy in this disorder compared with secondary forms of pulmonary hypertension.
authors.affiliation: Heart Institute and Department of Hematology, University of São Paulo School of Medicine, Fundação Pró-Sangue Hemocentro de São Paulo, Brazil. conaugusto@incor.usp.br
authors.forename: A A
authors.initials: AA
authors.lastname: Lopes
authors.forename: N Y
authors.initials: NY
authors.lastname: Maeda
authors.forename: R C
authors.initials: RC
authors.lastname: Gonçalves
authors.forename: S P
authors.initials: SP
authors.lastname: Bydlowski
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004730:Endothelium, Vascular / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology*; D007223:Infant; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D015996:Survival Rate; D014841:von Willebrand Factor / Q000502:physiology*
chemical_list: D014841:von Willebrand Factor