Introduction: Haemostatic and fibrinolytic parameters were evaluated in patients with chronic pulmonary hypertension following pulmonary embolism (CPE). The diagnosis of CPE was based on lobar or segmental defects on perfusion lung scans and by pulmonary angiograms which showed complete or partial obstruction of main or segmentary lobar arteries.

Methods: Antithrombin III (AT III), protein C, protein S, and lupus anticoagulant (LA) were assayed in 8 patients with CPE; in 6 out of 8 patients plasma fibrinolytic activity was assessed both under basal conditions and after venous stasis. The control group consisted of 4 normal subjects. Protein C and protein S antigens were assayed by an electrophoretic method. Protein C and protein S biological activities were assayed by a manual clotting system. AT III was assayed by chromogenic method. Fibrinolytic total activity was studied on fibrin plates, tPA and PAI-1 activities by chromogenic method; tPA and PAI-1 antigens by ELISA technique.

Results: One patient out of 8 showed a protein C deficiency and 3 patients out of 8 were positive for a LA. All patients had a statistically significant reduction of plasma fibrinolytic activity (p < 0.001) and of tPA activity (p < 0.0005) after venous stasis as compared to the control group.

Conclusions: Our data show that significant haemostatic abnormalities may underlie this disease. In particular, a) an impairment of fibrinolytic plasma activity and low levels of plasminogen activator may be found, and b) the undiagnosed presence of a LA may be the cause of these thrombotic events. The meaning of these results needs further assessment.

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