The objective of the present study is to assess blood coagulation disorders in patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension. The investigators aim to evaluate any possible coagulation abnormalities related to the patients' primary disease and any possible effects the pulmonary hypertension- specific therapy may have on hemostasis.
Pulmonary hypertension (PH) is a chronic, progressive, pulmonary vascular disease with a multifactorial etiology and a not fully elucidated pathophysiological background. There is a complex and not adequately understood association between PH and the coagulation process. The aim of the present study is to evaluate hemostasis in patients with PH classified as category 1 of the World Health Organization Pulmonary Hypertension Group (Pulmonary Arterial Hypertension, PAH) and 4 (Chronic Thromboembolic Pulmonary Hypertension, CTEPH). Patients with CTEPH are diagnosed as inoperable. The investigators perform diagnostic tests on blood samples collected directly from the pulmonary artery during the right heart catheterization performed as part of the patients' routine medical care for the diagnosis of the disease or for follow-up 6 months after the initiation of PH-specific treatment. All blood samples are processed by platelet function analyzer-100 (PFA-100), light transmission aggregometry (LTA), rotational thromboelastometry (ROTEM) and endogenous thrombin potential (ETP).The primary objective of the study is to assess platelet function, coagulation and anti-coagulation pathways and fibrinolysis in PAH and inoperable CTEPH patients and to investigate the possible effects of PH- specific therapy on hemostasis.
Study Type
OBSERVATIONAL
Enrollment
60
The PFA-100 system evaluates primary hemostasis in whole blood samples.
Light transmission aggregometry is the gold standard method for assessing platelet function.
ROTEM is a viscoelastic method for hemostasis testing in whole blood.This assay investigates the interaction of blood cells, coagulation factors and their inhibitors during clotting and subsequent fibrinolysis.
Attikon University Hospital
Athens, Greece
RECRUITINGPFA-100 in detection of platelet abnormalities in PAH and CTEPH patients.
Change in the percentage of PAH and CTEPH patients who are detected with platelet abnormalities in PFA-100 testing from the date of the first right heart catheterization performed for the diagnosis of their disease (before treatment implementation) to the date of the second right heart catheterization performed for follow-up (after treatment implementation) at 6 months.
Time frame: 6 months
Light transmission aggreggometry in detection of platelet abnormalities in PAH and CTEPH patients.
Change in the percentage of PAH and CTEPH patients who are detected with platelet abnormalities in LTA testing from the date of the first right heart catheterization performed for the diagnosis of their disease (before treatment implementation) to the date of the second right heart catheterization performed for follow-up (after treatment implementation) at 6 months.
Time frame: 6 months
ROTEM in detection of coagulation abnormalities in PAH and CTEPH patients.
Change in the percentage of PAH and CTEPH patients who are detected with coagulation abnormalities in ROTEM testing from the date of the first right heart catheterization performed for the diagnosis of their disease (before treatment implementation) to the date of the second right heart catheterization performed for follow-up (after treatment implementation) at 6 months.
Time frame: 6 months
Endogenous thrombin potential in detection of thrombin abnormalities in PAH and CTEPH patients.
Change in the percentage of PAH and CTEPH patients who are detected with thrombin deficits in endogenous thrombin potential testing from the date of the first right heart catheterization performed for the diagnosis of their disease (before treatment implementation) to the date of the second right heart catheterization performed for follow-up (after treatment implementation) at 6 months.
Time frame: 6 months
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
The endogenous thrombin potential assesses the amount of thrombin which can be generated after the in vitro activation of coagulation and represents the balance between pro- and anti-coagulant forces in plasma.