scholarly journals The validation of the questionnaire for the early detection of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

2016 ◽  
Vol 13 (1) ◽  
pp. 34-38
Author(s):  
Z S Valieva ◽  
S I Glukhova ◽  
T V Martyniuk ◽  
I E Chazova
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Early Detection ◽  
Physical Examination ◽  
Arterial Hypertension ◽  
Clinical Symptoms ◽  
Heart Defects ◽  
Lower Limbs ◽  
Control Group ◽  
Pulmonary Arterial

The objective: to identify pathognomonic symptoms and signs of different groups of pulmonary hypertension (PH), analyze the possibility of early diagnosis of patients with PH and the validity of the questionnaire.Material and methods. The questionnaire consists of 6 sections: clinical symptoms, physical examination, anamnestic information; diseases associated with PH; signs of pulmonary arterial hypertension (PAH) according to electrocardiography, chest radiography, echocardiography. All sections of the questionnaire were filled by physician. Was held an analysis of clinical symptoms, medical history, physical examination, methods of instrumental examination of 81 patients with idiopathic PH in comparison with 29 patients with PAH due to congenital heart defects - PAH-CHD, 36 patients chronic thromboembolic PH - CTEPH and the control group - 48 patients with essential hypertension. In addition, the validity of the questionnaire was evaluated: structural, the criterion and discriminant validity.Results. The leading complaint in IPAH patients was syncope; in patients with PAH-CHD the most often detected signs were cyanosis and accentuated pulmonary component of 2d heart sound; in patients with CTEPH - cough, they had a history of venous thrombosis of the lower limbs and the debut of the disease has been associated with pregnancy.Conclusion. Development and validation of a screening questionnaire allows to improve approaches to early detection of various forms of PH, allows identify specific symptoms that more typical of definite forms of PH.

scholarly journals The development of a screening questionnaire to improve the early detection of pulmonary arterial hypertension

2014 ◽  
Vol 11 (4) ◽  
pp. 62-67
Author(s):  
Z S Valieva ◽  
E G Valeeva ◽  
S I Glukhova ◽  
T V Martynyuk ◽  
I E Chazova
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Early Detection ◽  
Physical Examination ◽  
Arterial Hypertension ◽  
Heart Sound ◽  
Clinical Symptoms ◽  
Control Group ◽  
Pulmonary Arterial ◽  
Second Heart Sound

Objective: development of a questionnaire for the early detection of pulmonary arterial hypertension (PAH), and its further introduction for a broad routine medical practice.Were identified clinical symptoms, allowing differentiate various forms of the disease (IPAH and PAH-CHD), because these forms of PAH most frequent in cardiology practice.Material and methods. The questionnaire includes 6 sections: clinical symptoms, physical examination, anamnestic information, diseases associated with pulmonary hypertension; signs of PAH according to electrocardiography, chest radiography, echocardiography. All sections of the questionnaire filled by physician. Was held a retrospective analysis of the clinical symptoms, physical examination, anamnestic information, methods of instrumental examination of 70 patients with IPAH in comparison with 29 patients with PAH-CHD and the control group - 48 patients with arterial hypertension (AH).Results. The leading complaints in patients with PAH are dyspnea (99%), palpitations (58%), fatigue (59%), syncope (21%), dry cough (28%), peripheral edema (38%). The physical signs of PAH include an accentuated pulmonary component of second heart sound (93%), cyanosis (40%). Most often, the development of pulmonary hypertension precipitated by stress, pregnancy, acute respiratory infections.Conclusion. According to the results we concluded that the developed questionnaire may be offered for wide use in routine clinical practice and recommended not only for screening patients with IPAH, but also with PAH-CHD.

scholarly journals Assessing Response to Therapy in Idiopathic Pulmonary Arterial Hypertension: A Consensus Survey of Canadian Pulmonary Hypertension Physicians

2011 ◽  
Vol 18 (4) ◽  
pp. 230-234 ◽  
Author(s):  
Adrienne E Borrie ◽  
David N Ostrow ◽  
Robert D Levy ◽  
John R Swiston
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Physical Examination ◽  
Arterial Hypertension ◽  
Venous Pressure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Response To Therapy ◽  
Clinical Parameters ◽  
Standard Practice ◽  
Pulmonary Arterial

BACKGROUND: Many treatment options are now available for patients with idiopathic pulmonary arterial hypertension (IPAH). Data regarding the optimal combination of therapies are lacking, as is consensus on how to assess response to therapy and when to change therapeutic regimens.OBJECTIVES: To gather the opinions of Canadian pulmonary hypertension (PH) experts regarding standard practice in the care of IPAH patients after therapy is initiated.METHODS: Canadian PH physicians were surveyed using short questionnaires to assess their opinions and practices in the care of IPAH patients. A Delphi forecasting approach was used to gain consensus among Canadian physicians on the most important clinical parameters to consider when assessing patients after the initiation of therapy.RESULTS: Twenty-six of 37 Canadian PH experts who were invited to participate completed the study. All endorsed the use of combination therapy for IPAH patients despite the lack of universal provincial coverage for this approach. By consensus, WHO functional class, 6 min walk distance and hospitalization for right heart failure were the most important clinical parameters. The most highly rated physical examination parameters were jugular venous pressure, peripheral edema, the presence of ascites and body weight.CONCLUSIONS: The overall approach to care of IPAH patients is similar across PH centres in Canada. A limited number of clinical and physical examination parameters were considered to be most important to reassess patients after therapy is initiated. These parameters, along with definition of threshold values, will facilitate the development of standard practice guidelines for IPAH patients in Canada.

scholarly journals Evolution of pathogenetic therapy of pulmonary arterial hypertension

2019 ◽  
Vol 91 (12) ◽  
pp. 4-9
Author(s):  
I E Chazova ◽  
S Yu Yarovoy ◽  
N M Danilov
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Kinase Inhibitors ◽  
Heart Defects ◽  
New Drugs ◽  
Receptor Antagonists ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial ◽  
The Right

Pulmonary arterial hypertension (PAH) is a severe, disabling disease characterized by an increase pressure in the pulmonary artery (PA), an increase pressure in the right atrium, and a decrease of the cardiac output. It combines several diseases: idiopathic pulmonary hypertension, inherited pulmonary hypertension, PAH induced by medication and toxins, PAH associated with systemic diseases of connective tissue, HIV infection, portal hypertension, congenital heart defects, schistosomiasis. In the absence of treatment, PAH quickly leads to insufficiency of the right heart and premature death. An effective PAH therapy did not exist for a long time. However, in 1987 there was established a positive effect of taking large doses of calcium channel blockers in patients, who “responded” to their prescription in the short term, and in recently several groups of specific drugs have been developed and approved for the treatment of this pathology: prostacyclin analogues and prostacyclin receptors agonists, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors and soluble guanylate cyclase stimulators. Modern studies of treatment of PAH are based on the latest data of the molecular transmission mechanisms of intracellular and intercellular signals, the action of hormones and tissue enzymes. The available results of these studies allow to suggest the inclusion to clinical guidelines several new drugs for the pathogenetic treatment of PAH in the near future: receptor tyrosine kinase inhibitors, Rho - kinase inhibitors, immunosuppressants and type 2 activin receptor agonists, protein kinase C inhibitors, aromatase inhibitors and estrogen receptor antagonists, poly-(ADP-ribose)-polymerase inhibitors and bromodomain protein 4, elastase inhibitors. Some of the drugs have already passed the III phase of clinical trials (imatinib), others are at the preclinical stage or at the I-II phase tests (olaparib, enzastaurin, elafin).

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 Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry

2019 ◽  
Vol 91 (1) ◽  
pp. 25-31 ◽  
Author(s):  
I E Chazova ◽  
O A Arkhipova ◽  
T V Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Heart Defects ◽  
Connective Tissue Diseases ◽  
National Registry ◽  
Current Therapy ◽  
Pulmonary Arterial

Aim. Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry. Materials and methods. In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug - and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017. Results. Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug - and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years. Conclusion. The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.

scholarly journals Principles of pharmacological correction of pulmonary arterial hypertension

2018 ◽  
Vol 4 (2) ◽  
pp. 59-76 ◽  
Author(s):  
Mikhail Korokin ◽  
Nina I. Zhernakova ◽  
Liliya Korokina ◽  
Olga Pokopejko
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Heart Defects ◽  
Pulmonary Veins ◽  
Primary Therapy ◽  
Pulmonary Arterial ◽  
Pharmacological Correction

Definition and classification: Pulmonary hypertension (PH) is a group of life-threatening progressive diseases of various genesis, characterized by a progressive increase in arterial pressure (AP) in the pulmonary artery (PA), the remodeling of pulmonary vessels, which leads to an increase in pulmonary vascular resistance and pulmonary arterial pressure and more often leads to right ventricular heart failure and premature death. Pulmonary hypertension is clinically divided into five groups: patients in the first group have idiopathic pulmonary arterial hypertension (IPAH), whereas in patients of other groups secondary PH associated with cardiopulmonary or other systemic diseases is observed. The development of secondary LH is caused by congenital heart defects, collagenoses, presence of thrombus in the pulmonary artery, prolonged high pressure in the left atrium, hypoxemia, chronic obstructive pulmonary diseases (COPDs). In case of secondary PH, thrombosis and other changes in the pulmonary veins occur. Ways of pharmacological correction of pulmonary hypertension: Over the last decade pharmacotherapy of PH has been developing rapidly, and the introduction of modern methods of treatment, especially for primary PAH, has led to positive results. However, despite the progress in treatment, the functional limitations and survival of patients remain unsatisfactory. Currently, there are two levels of treatment for pulmonary hypertension: primary and specific pathogenetic therapies. Primary therapy is aimed at the main cause of PH. It also includes supportive therapy. Pathogenetic therapy includes prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors. Tactics of therapy can be established on the basis of either clinical classification, or functional class. Prostanoids are a promising group of drugs for the treatment of pulmonary arterial hypertension (PAH), since they possess not only vasodilating, but also antiplatelet and antiproliferative actions. Therefore, it seems logical to use prostacyclin and its analogs to treat patients with various forms of PAH.

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