Clinical trial NCT03195543: Assessment of Blood Coagulation Disorders in Patients With Pulmonary Hypertension
Official title: Assessment of Blood Coagulation Disorders in Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension.
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Pulmonary Artery Hypertension
Chronic Thromboembolic Pulmonary Hypertension
Keywords: Blood coagulation disorders
Platelets
Thrombin
Treatment
Lead sponsor: National and Kapodistrian University of Athens
Sponsor class: OTHER
First posted: 2017-06-22
Enrollment: 60
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: The study participants are recruited among the pulmonary hypertension patients of the Pulmonary Hypertension Unit, In the Second Department of Critical Care Medicine, Attiko University Hospital, Athens, Greece

Brief summary: 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.

Detailed description: 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.

Eligibility criteria: 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.

Primary outcomes:
measure: PFA-100 in detection of platelet abnormalities in PAH and CTEPH patients.
timeFrame: 6 months
measure: Light transmission aggreggometry in detection of platelet abnormalities in PAH and CTEPH patients.
timeFrame: 6 months
measure: ROTEM in detection of coagulation abnormalities in PAH and CTEPH patients.
timeFrame: 6 months
measure: Endogenous thrombin potential in detection of thrombin abnormalities in PAH and CTEPH patients.
timeFrame: 6 months
Secondary outcomes:
Other outcomes:
Locations:
city: Athens
country: Greece
facility: Attikon University Hospital
Study officials:
affiliation: National and Kapodistrian University of Athens
name: Eleni Vrigkou, MD, MSc
role: PRINCIPAL_INVESTIGATOR
affiliation: National and Kapodistrian University of Athens
name: Argyrios Tsantes, MD, PhD
role: STUDY_DIRECTOR
affiliation: National and Kapodistrian University of Athens
name: Iraklis Tsagkaris, MD, PhD
role: STUDY_CHAIR
affiliation: National and Kapodistrian University of Athens
name: Apostolos Armaganidis, MD, PhD
role: STUDY_CHAIR
References:
citation: Berger G, Azzam ZS, Hoffman R, Yigla M. Coagulation and anticoagulation in pulmonary arterial hypertension. Isr Med Assoc J. 2009 Jun;11(6):376-9.
citation: Herve P, Humbert M, Sitbon O, Parent F, Nunes H, Legal C, Garcia G, Simonneau G. Pathobiology of pulmonary hypertension. The role of platelets and thrombosis. Clin Chest Med. 2001 Sep;22(3):451-8. doi: 10.1016/s0272-5231(05)70283-5.
citation: Lopes AA, Caramuru LH, Maeda NY. Endothelial dysfunction associated with chronic intravascular coagulation in secondary pulmonary hypertension. Clin Appl Thromb Hemost. 2002 Oct;8(4):353-8. doi: 10.1177/107602960200800407.
citation: Preston IR, Farber HW. Anti-coagulation in pulmonary arterial hypertension: the real blood and guts. J Thorac Dis. 2016 Sep;8(9):E1106-E1107. doi: 10.21037/jtd.2016.08.48. No abstract available.
citation: Remkova A, Simkova I, Valkovicova T. Platelet abnormalities in chronic thromboembolic pulmonary hypertension. Int J Clin Exp Med. 2015 Jun 15;8(6):9700-7. eCollection 2015.
citation: Lang IM, Dorfmuller P, Vonk Noordegraaf A. The Pathobiology of Chronic Thromboembolic Pulmonary Hypertension. Ann Am Thorac Soc. 2016 Jul;13 Suppl 3:S215-21. doi: 10.1513/AnnalsATS.201509-620AS.