Pulmonary Hypertension: Beyond the Swan Ganz Catheter

Thromboembolic Disease• Endothelial dysfunction– Regulates balance between thrombotic and antithrombotic mechanisms– Activates factor X– Forms prothrombinase complex (activates thrombin)– Activates extrinsic pathway of coagulation by releasing tissue factor– Alters production & release of von Willebrand factor, nitric oxide, prostacyclin– Expresses thrombomodulin (prevents conversion of fibrinogen to fibrin)– Produces TPA and plasminogen activator inhibitor‐1 (inhibits TPA)• Abnormal platelet function– TXA 2– Alteration in nitric oxide and prostacyclin levels– Serotonin release defects• Inherited coagulation defecits– Deficient factor V lieden or antithrombin III– Responsible for chronic thromboembolic disease• Pulmonary vascular‐specific fibrin formation– Perhaps not generalized vascular prothrombotic state• Altered fibrinogen metabolism– Decreased ½life– Increased fibrinogen levels