Endothelial cell dysfunction correlates differentially with survival in primary and secondary pulmonary hypertension

Background Plasma von Willebrand factor antigen (vWF:Ag) has been used as a marker of endothelial perturbation in a number of vascular disorders. In t his study, vWF:Ag was determined as an attempt to evaluate the severity of endothelial cell dysfunction in primary pulmonary hypertension (PPH) and co ngenital heart disease-associated pulmonary hypertension (CHD-PH) comparati vely and to determine its impact on short-term survival. Methods and Results Clinical, hemodynamic, and biochemical data were obtain ed from 1 1 patients with PPH and 24 with CHD-PH. Patient groups were simil ar in terms of age and pulmonary artery pressure. vWF:Ag was measured by el ectroimmunodiffusion. Patients were followed vp for 7 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 refere nce value 87% +/- 23% activity, mean +/- SD), with higher revels 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 pat ient age, sex, or pulmonary artery pressure. Seven of 10 nonsurvivors but o nly 4 of 25 survivors had PPH (P = .007). vWF:Ag was 255% +/- 90% in the no nsurvivor 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 add ition, exceedingly high vWF:Ag levels in PPH might reflect a particular pat tern of endothelial cell dysfunction that could be associated with decrease d short-term life expectancy in this disorder compared with secondary terms of pulmonary hypertension.