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Cell Calcium Jul 2024Calcium (Ca) is a secondary messenger that regulates various cellular processes. However, Ca mishandling could lead to pathological conditions. Orai1 is a Cachannel... (Review)
Review
Calcium (Ca) is a secondary messenger that regulates various cellular processes. However, Ca mishandling could lead to pathological conditions. Orai1 is a Cachannel contributing to the store-operated calcium entry (SOCE) and plays a critical role in Ca homeostasis in several cell types. Dysregulation of Orai1 contributed to severe combined immune deficiency syndrome, some cancers, pulmonary arterial hypertension (PAH), and other cardiorespiratory diseases. During its activation process, Orai1 is mainly regulated by stromal interacting molecule (STIM) proteins, especially STIM1; however, many other regulatory partners have also been recently described. Increasing knowledge about these regulatory partners provides a better view of the downstream signalling pathways of SOCE and offers an excellent opportunity to decipher Orai1 dysregulation in these diseases. These proteins participate in other cellular functions, making them attractive therapeutic targets. This review mainly focuses on Orai1 regulatory partners in the physiological and pathological conditions of the pulmonary circulation and inflammation.
Topics: Humans; ORAI1 Protein; Animals; Stromal Interaction Molecules; Calcium Signaling; Calcium; Vascular Diseases
PubMed: 38735127
DOI: 10.1016/j.ceca.2024.102892 -
Arquivos Brasileiros de Cardiologia 2019The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of... (Review)
Review
The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of PH. When direct involvement of pulmonary circulation is suspected, invasive hemodynamic monitoring is recommended to establish the diagnosis. This assessent provides, in addition to the diagnostic confirmation, the correct identification of the vascular territory predominantly involved (arterial pulmonary or postcapillary). Treatment with specific medication for PH (phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostacyclin analogues) has been proven effective in patients with pulmonary arterial hypertension, but its use in patients with PH due to left heart disease can even be damaging. In this review, we discuss the diagnosis criteria, how etiological investigation should be carried out, the clinical classification and, finally, the therapeutic recommendations for PH.
Topics: Echocardiography; Heart Diseases; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Pulmonary Disease, Chronic Obstructive; Risk Assessment
PubMed: 31621783
DOI: 10.5935/abc.20190188 -
Cardiovascular Diagnosis and Therapy Oct 2022Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) >20 mmHg and its presence is associated with worse outcomes. A comprehensive... (Review)
Review
BACKGROUND AND OBJECTIVE
Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) >20 mmHg and its presence is associated with worse outcomes. A comprehensive hemodynamic evaluation of the pulmonary circulation is essential for diagnosis, hemodynamic classification, and prognostication. A multitude of indices assess different aspects of the pulmonary circulation but there are no reviews that describe their specific value in PH.
METHODS
We performed a thorough literature search of relevant articles in English from 1970-2021 using PubMed.
KEY CONTENT AND FINDINGS
In this article, we present both static and dynamic indices used for the hemodynamic assessment of PH. While some of these indices are routinely used in clinical practice, including cardiac index (CI), stroke volume (SV), and pulmonary vascular resistance (PVR); others such as pulmonary artery compliance (PAC), pulmonary effective arterial elastance (Ea), and pulmonary artery pulsatility index (PAPi) are gaining popularity by enhancing the understanding of different aspects of the pulmonary circulation. We described the advantages and pitfalls of various indices, including when to use them in the hemodynamic evaluation of patients with PH.
CONCLUSIONS
A variety of indices measuring different aspects of the right ventricle (RV)-pulmonary arteries (PA) system provide valuable information in patients with PH. However, it remains important to develop and validate indices that provide a comprehensive hemodynamic evaluation to improve outcomes in patients with PH.
PubMed: 36329964
DOI: 10.21037/cdt-22-244 -
International Journal of Chronic... 2020Pulmonary hypertension (PH) is a frequent and important complication of chronic obstructive pulmonary disease (COPD). It is associated with worse clinical courses with... (Review)
Review
Pulmonary hypertension (PH) is a frequent and important complication of chronic obstructive pulmonary disease (COPD). It is associated with worse clinical courses with more frequent exacerbation episodes, shorter survival, and greater need of health resources. PH is usually of moderate severity and progresses slowly, without altering right ventricular function in the majority of cases. Nevertheless, a reduced subgroup of patients may present disproportionate PH, with pulmonary artery pressure (PAP) largely exceeding the severity of respiratory impairment. These patients may represent a group with an exaggerated vascular impairment (pulmonary vascular phenotype) to factors that induce PH in COPD or be patients in whom idiopathic pulmonary arterial hypertension (PAH) coexist. The present review addresses the current definition and classification of PH in COPD, the distinction among the different phenotypes of pulmonary vascular disease that might present in COPD patients, and the therapeutic approach to PH in COPD based on the available scientific evidence.
Topics: Humans; Hypertension, Pulmonary; Lung; Pulmonary Circulation; Pulmonary Disease, Chronic Obstructive; Ventricular Function, Right
PubMed: 32606641
DOI: 10.2147/COPD.S211841 -
Pulmonary Circulation Apr 2024Pollution and climate change constitute a combined, grave and pervasive threat to humans and to the life-support systems on which they depend. Evidence shows a strong... (Review)
Review
Pollution and climate change constitute a combined, grave and pervasive threat to humans and to the life-support systems on which they depend. Evidence shows a strong association between pollution and climate change on cardiovascular and respiratory diseases, and pulmonary vascular disease (PVD) is no exception. An increasing number of studies has documented the impact of environmental pollution and extreme temperatures on pulmonary circulation and the right heart, on the severity and outcomes of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (PH), on the incidence of pulmonary embolism, and the prevalence and severity of diseases associated with PH. Furthermore, the downstream consequences of climate change impair health care systems' accessibility, which could pose unique obstacles in the case of PVD patients, who require a complex and sophisticated network of health interventions. Patients, caretakers and health care professionals should thus be included in the design of policies aimed at adaptation to and mitigation of current challenges, and prevention of further climate change. The purpose of this review is to summarize the available evidence concerning the impact of environmental pollution and climate change on the pulmonary circulation, and to propose measures at the individual, healthcare and community levels directed at protecting patients with PVD.
PubMed: 38933180
DOI: 10.1002/pul2.12394 -
Cureus Apr 2021Heterotaxy syndrome implies a discordance between placement of thoracic organs with respect to abdominal organs. A large number of these have associated congenital heart...
Heterotaxy syndrome implies a discordance between placement of thoracic organs with respect to abdominal organs. A large number of these have associated congenital heart defects. This syndrome is unique as every patient is different and can have any permutation and combination of symptoms. In our case, the five-year-old male child presented with complaints of abdominal distension, fever, and bluish discoloration of limbs with even mild exertion. Radiological evaluation was diagnosed with a large atrial septal defect, cardiomegaly, partial pulmonary venous circulation, multiple small spleens on the right side of body, a large midline liver, malrotated bowel, inferiorly displaced kidneys, and two hemiazygos veins. The echocardiography and electrocardiogram too were consistent with atrial septal defect and right ventricular strain pattern. The reasons for this highly variable pattern are rooted in the genetically complicated process of lateralization with a strong link to the copy number variations. Due to the variable patterns, it is more efficient to report all the findings utilizing a step-by-step process of commenting on each and every individual organ, instead of classifying them under different categories based on atrial isomerism. This is important as any other way of classification predisposes to a certain bias.
PubMed: 34094731
DOI: 10.7759/cureus.14766 -
Microvascular Research Mar 2022Evidence suggests severe coronavirus disease-19 (COVID-19) infection is characterised by pulmonary and systemic microvasculature dysfunction, specifically, acute... (Review)
Review
Evidence suggests severe coronavirus disease-19 (COVID-19) infection is characterised by pulmonary and systemic microvasculature dysfunction, specifically, acute endothelial injury, hypercoagulation and increased capillary permeability. Diabetes, which is also characterised by vascular injury in itself, confers an increased risk of adverse COVID-19 outcomes. It has been suggested that pre-existing endothelial dysfunction and microvascular disease in diabetes will exacerbate the vascular insults associated with COVID-19 and thus lead to increased severity of COVID-19 infection. In this article, we evaluate the current evidence exploring the impact of microvascular complications, in the form of diabetic retinopathy and nephropathy, in individuals with COVID-19 and diabetes. Future insights gained from exploring the microvascular injury patterns and clinical outcomes may come to influence care delivery algorithms for either of these conditions.
Topics: Albuminuria; COVID-19; Capillary Permeability; Delivery of Health Care; Diabetic Angiopathies; Diabetic Nephropathies; Diabetic Neuropathies; Diabetic Retinopathy; Endothelium, Vascular; Humans; Microcirculation; Obesity; Pandemics; Pulmonary Circulation; Pulmonary Edema; SARS-CoV-2; Severity of Illness Index; Thrombophilia; Treatment Outcome
PubMed: 34979154
DOI: 10.1016/j.mvr.2021.104310 -
Circulation Research Apr 2022There is an increased appreciation for the importance of the right heart and pulmonary circulation in several disease states across the spectrum of pulmonary... (Review)
Review
There is an increased appreciation for the importance of the right heart and pulmonary circulation in several disease states across the spectrum of pulmonary hypertension and left heart failure. However, assessment of the structure and function of the right heart and pulmonary circulation can be challenging, due to the complex geometry of the right ventricle, comorbid pulmonary airways and parenchymal disease, and the overlap of hemodynamic abnormalities with left heart failure. Several new and evolving imaging modalities interrogate the right heart and pulmonary circulation with greater diagnostic precision. Echocardiographic approaches such as speckle-tracking and 3-dimensional imaging provide detailed assessments of regional systolic and diastolic function and volumetric assessments. Magnetic resonance approaches can provide high-resolution views of cardiac structure/function, tissue characterization, and perfusion through the pulmonary vasculature. Molecular imaging with positron emission tomography allows an assessment of specific pathobiologically relevant targets in the right heart and pulmonary circulation. Machine learning analysis of high-resolution computed tomographic lung scans permits quantitative morphometry of the lung circulation without intravenous contrast. Inhaled magnetic resonance imaging probes, such as hyperpolarized 129Xe magnetic resonance imaging, report on pulmonary gas exchange and pulmonary capillary hemodynamics. These approaches provide important information on right ventricular structure and function along with perfusion through the pulmonary circulation. At this time, the majority of these developing technologies have yet to be clinically validated, with few studies demonstrating the utility of these imaging biomarkers for diagnosis or monitoring disease. These technologies hold promise for earlier diagnosis and noninvasive monitoring of right heart failure and pulmonary hypertension that will aid in preclinical studies, enhance patient selection and provide surrogate end points in clinical trials, and ultimately improve bedside care.
Topics: Heart Failure; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Xenon Isotopes
PubMed: 35482838
DOI: 10.1161/CIRCRESAHA.121.319990