Chronic Thromboembolic Pulmonary Hypertension Clinical Trial
Official title:
Assessment of Blood Coagulation Disorders in Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.
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.
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