Assessment of Blood Coagulation Disorders in Patients With Pulmonary Hypertension

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.

Inclusion Criteria:

• Pulmonary Arterial Hypertension,
• Chronic Thromboembolic Pulmonary Hypertension.

Exclusion Criteria:

• renal insufficiency,
• hepatic insufficiency,
• thyroid dysfunction,
• malignancy,
• active infections,
• receiving anticoagulant or antiplatelet therapy,
• history of hemostatic disorders irrelevant to their primary disease,
• abnormal red blood counts.