The pathophysiological background of chronic thromboembolic pulmonary hypertension (CTEPH) has not been fully elucidated. Evidence suggests that abnormal platelet function and ineffective fibrinolysis may play a key role in the development of the disease. The purpose of this study was to evaluate platelet and coagulation function in CTEPH, using non-conventional global coagulation assays, and platelet activation and endothelial dysfunction laboratory markers. A total of 40 newly-diagnosed CTEPH patients were studied, along with 35 healthy controls. Blood samples from CTEPH patients were taken directly from the pulmonary artery. All subjects were assessed with platelet function analyzer-100, light transmission aggregometry, thromboelastometry, endogenous thrombin potential. von Willebrand antigen and activity, p-selectin, thromboxane A2 and serotonin levels were also assessed. The results showed that CTEPH patients present diminished platelet aggregation, presence of disaggregation, decreased rate of fibrinolysis, defective thrombin generation and increased levels of thromboxane A2, p-selectin, von Willebrand antigen and activity. Serotonin levels did not present any differences between the two groups. The results of this study suggest that CTEPH patients present platelet function, fibrinolytic, thrombin generation and other clot formation abnormalities. Well-designed clinical studies are needed to further evaluate the complex hemostatic abnormalities in the CTEPH setting and assess their potential clinical applications.
Keywords: chronic thromboembolic pulmonary hypertension; fibrinolysis; p-selectin; platelets; serotonin; thrombin; thromboxane A2; von Willebrand.