Summary
Primary pulmonary hypertension (PPH) is a rare disorder, with marked in-situ thrombosis
of small pulmonary vessels occurring primarily in adult women. We investigated whether
differences in the plasmin- and thrombin activation system are associated with the
predominate affection of females. Plasma levels of plasminogen activator inhibitor
type 1 (PAI-1), tissue-type plasminogen activator (t-PA), fibrinogen, thrombin-antithrombin
(TAT) complexes, and prothrombin fragments (F1.2) were measured at baseline and after
standardized venous occlusion (VO) in patients with PPH (24 female, 9 male). At baseline,
females showed significant higher TAT levels (p = 0.05), higher t-PA antigen levels
(p = 0.01) and higher fibrinogen levels (p = 0.03) with positive correlation to mean
pulmonary artery pressure (mPAP), as well as nonsignificant lower t-PA activity, higher
PAI-1 antigen and activity and F1.2 levels. After VO, females showed a significantly
blunted increase in t-PA antigen (p = 0.01) and t-PA activity (p = 0.001), correlating
with mPAP, as well as increased PAI-1 activity (p = 0.05). We hypothesize, that the
observed presence of gender differences in the plasmin- and thrombin activation system
in PPH leading to an antifibrinolytic/prothrombotic state might, in part, explain
the female predominant incidence of this disease.
Keywords
Primary pulmonary hypertension - fibrinolysis - blood coagulation - gender