[Coagulation and fibrinolytic abnormality related to endothelial injury in pulmonary arterial hypertension].

ABSTRACT

It remains unclear whether a coagulation abnormality plays an important role in the clinical course in pulmonary arterial hypertension as well as vasoconstriction and vascular-wall remodeling, although several studies of pathological specimens and clinical studies demonstrating that anticoagulation prolongs life have suggested the importance of thrombosis. Several parameters for hemostasis related to endothelial injury are expected to reflect abnormal coagulation and fibrinolytic systems in pulmonary hypertension. Recently, we demonstrated that plasma level of soluble P-selectin was increased and that of thrombomodulin (TM) was decreased in patients with pulmonary arterial hypertension. The abnormal values of P-selectin and TM were improved by continuous prostacyclin infusion. Altered hemostasis may be important as a pathogenesis in pulmonary hypertension and adequate anti-coagulant therapy is expected.