scholarly journals High CD200 Expression on T CD4+ and T CD8+ Lymphocytes as a Non-Invasive Marker of Idiopathic Pulmonary Hypertension—Preliminary Study

2021 ◽  
Vol 10 (5) ◽  
pp. 950
Author(s):  
Michał Tomaszewski ◽  
Ewelina Grywalska ◽  
Weronika Topyła-Putowska ◽  
Piotr Błaszczak ◽  
Marcin Kurzyna ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Peripheral Blood ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Peripheral Blood Lymphocytes ◽  
Endothelial Cell Proliferation ◽  
Control Group ◽  
Barr Virus ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) can develop subsequently to disorganized endothelial cell proliferation within the pulmonary arteriolar layers that provide mechanical limits to the pulmonary vascular bed. Although the actual factor triggering vascular endothelial proliferation remains unknown to date, genetic susceptibility, hypoxia, inflammation, as well as response to drugs and toxins have been proposed as possible contributors. Since inflammation contributes to vascular remodeling, the changed immune response is increasingly considered a plausible cause of this cardiovascular disease. The interaction of a membrane glycoprotein cluster of differentiation 200 (CD200) and its structurally similar receptor (CD200R) plays a crucial role in the modulation of the inflammatory response. Our previous studies have shown that the overexpression of the other negative co-stimulatory molecule (programmed death cell - PD-1) and its ligand-1 (PD-L1) is closely related to iPAH and the presence of Epstein-Barr virus (EBV) reactivation markers. Therefore, we considered it necessary to analyze the different types of PAH in terms of CD200 and CD200R expression and to correlate CD200/CD200R pathway expression with important clinical and laboratory parameters. The CD200/C200R-signaling pathway has not been subject to much research. We included 70 treatment-naïve, newly diagnosed patients with PAH in our study. They were further divided into subsets according to the pulmonary hypertension classification: chronic thromboembolic pulmonary hypertension (CTEPH) subset, pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH), pulmonary arterial hypertension associated with connective tissue disease (CTD-PAH), and idiopathic pulmonary arterial hypertension (iPAH). The control group consisted of 20 healthy volunteers matched for sex and age. The highest percentages of T CD200+CD4+ and T CD200+CD8+ lymphocytes were observed in the group of patients with iPAH and this finding was associated with the presence of EBV DNA in the peripheral blood. Our assessment of the peripheral blood lymphocytes expression of CD200 and CD200R indicates that these molecules act as negative co-stimulators in the induction and persistence of PAH-associated inflammation, especially that of iPAH. Similar results imply that the dysregulation of the CD200/CD200R axis may be involved in the pathogenesis of several immune diseases. Our work suggests that CD200 and CD200R expression may serve to distinguish between PAH cases. Thus, CD200 and CD200R might be useful as markers in managing PAH and should be further investigated.

scholarly journals Evaluation of inflammatory and coagulation markers in patients with idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension with comorbid hypercholesterolemia

2019 ◽  
Vol 29 (2) ◽  
pp. 175-183
Author(s):  
Irina N. Taran ◽  
Ol’ga A. Arkhipova ◽  
Valeriy P. Masenko ◽  
Anatoliy B. Dobrovol’skiy ◽  
Tamila V. Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Control Group ◽  
Total Blood ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
D Dimer

The aim of the study was to assess inflammatory and coagulation disorders in patients with idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in dependence on dyslipidemia. Methods. The study involved 22 patients with IPAH and 6 patients with inoperable CTEPH complicated by hypercholesterolemia (total cholesterol (TC) level > 5.8 mmol/L). Healthy volunteers with normal TC level (n = 20) were included in a control group. We measured serum cytokine concentrations, such as interleukin (IL)-2, IL-4, IL-5, IL-6, IL-10, IL-12, and interferon (INF)-γ, using a multiplex fluorescent analyzer GEN-PROBE (Luminex 200) with Bio-Plex Precision Pro set. Von Willebrand factor was measured using an immunoturbidimetric assay with STA-Liatest VWF: Ag set. D-dimer, fibrinogen, C-reactive protein, and the total blood cell count were also measured. Results. CTERH patients had lower IL-2 level and more prominent signs of chronic heart failure (CHF) compared to patients with IPAH. Serum levels of IL-10, IL-4, and IL-12 were significantly related to syncope frequency, INF-γ level and cardiac index (CI). High density lipoprotein (HDL) level was inversely related to mean pulmonary arterial pressure (mPAP) in IPAH/CTEPH patients with TC > 6.5 mmol/L. Decreased thrombocyte count and decreased thrombocrit were associated with increased mPAP and mean right atrium (RA) pressure in patients with dilated right pulmonary artery. Increased D-dimer level was associated with enlarged RA area, enlarged right ventricle size, tricuspid regurgitation and decreased CI. Conclusion. Serum concentrations of the inflammatory and coagulation markers, excepting LI-2, did no differ significantly in patients with IPAH and CTEPH. Dyslipidemia was associated with activation of inflammation. Further studies are needed to estimate a predictive value of decreased HDL in patients with IPAH and CTEPH.

scholarly journals Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with chronic thromboembolic pulmonary hypertension (CTEPH): a qualitative interview study

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Brooke Currie ◽  
Evan Davies ◽  
Amélie Beaudet ◽  
Larissa Stassek ◽  
Leah Kleinman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Shortness Of Breath ◽  
Response Options ◽  
Qualitative Interview Study ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Impact

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients’ lives is needed to optimally assess the impact of the disease and treatment. Objectives This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients’ lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™‘s instructions, items, and response options. Results Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an “extremely important” symptom, and seven participants rated fatigue as “extremely important.” The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH.

Pulmonary arterial hypertension and acute and chronic thromboembolism

ESC CardioMed ◽  
2018 ◽  
pp. 406-409
Author(s):  
Thomas Henzler
Keyword(s):  
Computed Tomography ◽  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Imaging Modality ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Right Ventricular Dysfunction ◽  
Chronic Pulmonary Embolism ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) and acute and chronic pulmonary embolism represent severe cardiovascular diseases with a high mortality if left undiagnosed and untreated. Computed tomography of the chest plays a pivotal role in the diagnosis of all three disorders. In acute pulmonary embolism, computed tomography pulmonary angiography has become the gold-standard imaging modality due to its high diagnostic accuracy, cost-effectiveness, 24-hour availability at most institutions, as well as the ability to diagnose alternative chest pathologies and right ventricular dysfunction within a single examination. In PAH, computed tomography of the chest is also deeply embedded within the diagnostic algorithm in order to exclude other causes of pulmonary hypertension, such as structural lung disease and chronic thromboembolic pulmonary hypertension of left heart disease. This article intends to provide a short overview on imaging techniques and characteristic findings in PAH, as well as acute and chronic pulmonary embolism.

scholarly journals Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 9 (1) ◽  
pp. 204589401983489 ◽  
Author(s):  
Meghan M. Cirulis ◽  
John J. Ryan ◽  
Stephen L. Archer
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Molecular Mechanisms ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Outcome Data ◽  
Current Evidence ◽  
Clinical Aspects ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Arrhythmias are increasingly recognized as serious, end-stage complications of pre-capillary pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Although arrhythmias contribute to symptoms, morbidity, in-hospital mortality, and possibly sudden death in PAH/CTEPH, there remains a paucity of epidemiologic, pathophysiologic, and outcome data to guide management of these patients. This review summarizes the most current evidence on the topic: from the molecular mechanisms driving arrhythmia in the hypertrophied or failing right heart, to the clinical aspects of epidemiology, diagnosis, and management.

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