Patient age ranged from 1.2 to 45 (median 24) years, and the mean pulmonary arterial pressure was 27 to 112 (median 65) mm Hg. All but one patients had New York Heart Association class III or IV symptoms. According to the above criteria, the diagnosis of primary pulmonary hypertension was established in 11 patients. In the remaining patients, pulmonary vascular disease was associated with antiphospholipid syndrome (n = 1), collagen vascular disease (n = 1), schistosomiasis (n = 3), or congenital heart defects (Eisenmenger s syndrome) (n = 24). During the period of the study, all patients received antiplatelet drugs, and most patients were under anticoagulant treatment with warfarin. Patients with Eisenmenger s syndrome were subjected to hemodilution procedures to maintain an hematocrit level below 56% read more canadian pharmacy levitra . Medical therapy was not significantly changed during the follow-up period. Moreover, there were no significant differences in therapeutic measures between survivors and nonsurvivors, except that drugs for cardiovascular support were administered to patients of the nonsurvivor group at the very end of their illness.
Laboratory procedures that were performed at the beginning of the study showed that vWF:Ag was significantly increased in patients with pulmonary hypertension compared with control subjects, with respective activity values of 166.3 ± 87.0 and 87.3 ± 22.7% (p < 0.001). Despite the high circulating levels, the biological activity of vWF was decreased. In patients, the ristocetin cofactor activity, expressed as a percentage of the control value obtained in the same assay, was 73.9 ± 36.6% (95% Cl, 57.8% to 90.0%). Patients with primary pulmonary hypertension had higher vWF:Ag levels than patients with secondary pulmonary hypertension (231.1 ±89.1 vs 141.7 ± 73.7% activity, respectively; p = 0.003), although pulmonary artery pressure did not differ significantly between the groups.
Category: Pulmonary Hypertension
Tags: endothelial cells, pulmonary hypertension, von Willebrand factor