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The American Journal of Cardiology Oct 2023
Topics: Humans; Hypertension, Pulmonary; Exercise
PubMed: 37666015
DOI: 10.1016/j.amjcard.2023.08.008 -
European Respiratory Review : An... Dec 2023Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared... (Review)
Review
Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared pathophysiological and clinical features. In this paper, the first of a series covering all five PH classification groups, the clinical, radiological and pathological features of pulmonary arterial hypertension (PAH) will be reviewed. PAH may develop in the presence of associated medical conditions or a family history, following exposure to certain medications or drugs, or may be idiopathic in nature. Although all forms of PAH share common histopathological features, the presence of certain pulmonary arterial abnormalities, such as plexiform lesions, and extent of co-existing pulmonary venous involvement differs between the different subgroups. Radiological investigations are key to diagnosing the correct form of PH and a systematic approach to interpretation, especially of computed tomography, is essential.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Familial Primary Pulmonary Hypertension; Pulmonary Artery
PubMed: 38123231
DOI: 10.1183/16000617.0138-2023 -
Experimental Cell Research Oct 2023Hypoxia-induced pulmonary hypertension is a subgroup of type 3 pulmonary hypertension (PH) with the recommended treatment limited to oxygen therapy and lacks potential... (Review)
Review
Hypoxia-induced pulmonary hypertension is a subgroup of type 3 pulmonary hypertension (PH) with the recommended treatment limited to oxygen therapy and lacks potential therapeutic targets. To investigate the role of NLRC3 in hypoxia-induced PH and its potential mechanism, we first collected lung tissues of high-altitude pulmonary hypertension (HAPH) patients. Immunohistochemistry and immunofluorescence showed that NLRC3 was downregulated and was mainly co-localized with the smooth muscle cells of the pulmonary vessels in HAPH patients. Besides, we found that NLRC3 was also expressed in endothelial cells in HAPH patients for the first time. Then, wild type (WT) and NLRC3 knockout (NLRC3) mice were used to construct hypoxia models and primary pulmonary arterial smooth muscle cells (PASMCs) of rats and endothelial cells were cultured for verification. Right heart catheterization and echocardiography suggested that NLRC3 knockout promoted right ventricular systolic pressure (RVSP) up-regulation, right ventricular hypertrophy and fibrosis in hypoxia-induced mice. This study first demonstrated that NLRC3 deficiency promoted hypoxia-stimulated PASMCs proliferation, Human umbilical vein endothelial cells (HUVECs) apoptosis, migration and inflammation through IKK/NF-κB p65/HIF-1α pathway in vitro and in vivo, further promoted vascular remodeling and PH progression, which provided a new target for the treatment of hypoxia-induced PH.
Topics: Animals; Humans; Mice; Rats; Cell Proliferation; Cells, Cultured; Endothelial Cells; Hypertension, Pulmonary; Hypoxia; Intercellular Signaling Peptides and Proteins; Myocytes, Smooth Muscle; NF-kappa B; Pulmonary Artery; Vascular Remodeling
PubMed: 37586455
DOI: 10.1016/j.yexcr.2023.113755 -
The European Respiratory Journal Dec 2023Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well...
BACKGROUND
Peripheral pulmonary artery stenosis (PPS) refers to stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although paediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Our objectives in this study were to characterise the disease profile of adult-onset PPS.
METHODS
We collected data in Japanese centres. This cohort included patients who underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterisation (RHC) findings, imaging findings and treatment profiles were collected.
RESULTS
44 patients (median (interquartile range) age 39 (29-57) years; 29 females (65.9%)) with PPS were enrolled from 20 centres. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. 35 patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH) and 22 patients (50.0%) received combination therapy. 25 patients (56.8%) underwent transcatheter pulmonary angioplasty. RHC data showed improvements in both mean pulmonary arterial pressure (44 40 mmHg; p<0.001) and pulmonary vascular resistance (760 514 dyn·s·cm; p<0.001) from baseline to final follow-up. The 3-, 5- and 10-year survival rates of patients with PPS were 97.5% (95% CI 83.5-99.6%), 89.0% (95% CI 68.9-96.4%) and 67.0% (95% CI 41.4-83.3%), respectively.
CONCLUSIONS
In this study, patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favourable treatment response to PAH drugs combined with transcatheter pulmonary angioplasty.
Topics: Adult; Female; Humans; Child; Stenosis, Pulmonary Artery; Hypertension, Pulmonary; Constriction, Pathologic; Pulmonary Artery; Familial Primary Pulmonary Hypertension; Pulmonary Arterial Hypertension
PubMed: 38061784
DOI: 10.1183/13993003.00763-2023 -
Cells Oct 2023Pulmonary hypertension is a debilitating condition that frequently develops in the setting of interstitial lung disease, likely related to chronic alveolar hypoxemia and... (Review)
Review
Pulmonary hypertension is a debilitating condition that frequently develops in the setting of interstitial lung disease, likely related to chronic alveolar hypoxemia and pulmonary vascular remodeling. This disease process is likely to be identified more frequently by providers given recent advancements in definitions and diagnostic modalities, and provides practitioners with emerging opportunities to improve patient outcomes and quality of life. Despite years of data suggesting against the efficacy of pulmonary vasodilator therapy in patients with pulmonary hypertension due to interstitial lung disease, new data have emerged identifying promising advancements in therapeutics. The authors present to you a comprehensive review of pulmonary hypertension in interstitial lung disease, reviewing our current understanding of pathophysiology, updates in diagnostic approaches, and highlights of recent clinical trials which provide an effective approach for medical management.
Topics: Humans; Hypertension, Pulmonary; Quality of Life; Lung Diseases, Interstitial; Lung
PubMed: 37830608
DOI: 10.3390/cells12192394 -
JAMA Network Open Nov 2023Bronchopulmonary dysplasia (BPD) is often associated with pulmonary vascular disease and secondary pulmonary hypertension (PH). The pathogenesis of BPD-associated PH... (Meta-Analysis)
Meta-Analysis
IMPORTANCE
Bronchopulmonary dysplasia (BPD) is often associated with pulmonary vascular disease and secondary pulmonary hypertension (PH). The pathogenesis of BPD-associated PH (BPD-PH) is complex and involves prenatal and postnatal factors that disrupt pulmonary vascular development, and patent ductus arteriosus (PDA) is a factor potentially associated with risk of BPD-PH that has been identified in very recent studies.
OBJECTIVE
To explore the association of PDA with BPD-PH using a bayesian model-averaged (BMA) meta-analysis of studies.
DATA SOURCES
PubMed and Embase were searched up to April 2023. Key search terms included BPD and PH.
STUDY SELECTION
Studies examining infants with gestational age 32 weeks or less and reporting data on PDA and risk of BPD-PH.
DATA EXTRACTION AND SYNTHESIS
This study followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and the Meta-Analysis of Observational Studies in Epidemiology reporting guidelines. Two independent reviewers extracted data, with a third reviewer checking for accuracy and completeness. Data pooling and effect size calculations were performed by BMA.
MAIN OUTCOMES AND MEASURES
The primary outcome was BPD-PH. BMA was used to calculate Bayes factors (BFs). The BF10 is the ratio of the probability of the data under the alternative hypothesis (H1, association of PDA with BPD-HP) over the probability of the data under the null hypothesis (H0).
RESULTS
A total of 32 studies (8513 infants) were included. BMA showed that the evidence in favor of H1 was weak for any PDA (BF10 = 2.90; 10 studies), moderate for hemodynamically significant PDA (BF10 = 3.77; 3 studies), and extreme for surgically ligated or catheter-occluded PDA (BF10 = 294.9; 16 studies). In contrast, the evidence in favor of H0 was weak for medically treated PDA (BF10 = 0.55; 6 studies). In addition, BMA found strong evidence in favor of H1 when prolonged exposure to PDA was analyzed as a dichotomous variable (BF10 = 11.80; 6 studies) and extreme evidence (BF10 = 113.60; 3 studies) when PDA exposure time was analyzed as a continuous variable.
CONCLUSIONS AND RELEVANCE
In this bayesian meta-analysis, the data suggest that prolonged exposure to PDA might be associated with increased risk of pulmonary vascular disease in extremely preterm infants. This highlights the need to monitor for PH in high-risk preterm infants with prolonged exposure to PDA and to incorporate PH risk into clinical decisions regarding PDA management.
Topics: Infant, Newborn; Infant; Female; Pregnancy; Humans; Ductus Arteriosus, Patent; Bronchopulmonary Dysplasia; Bayes Theorem; Hypertension, Pulmonary; Vascular Diseases; Infant, Extremely Premature; Observational Studies as Topic
PubMed: 38015504
DOI: 10.1001/jamanetworkopen.2023.45299 -
Journal of Nanobiotechnology Nov 2023Venous thromboembolism (VTE) is a multifactorial disease, and pulmonary hypertension (PH) is a serious condition characterized by pulmonary vascular remodeling leading... (Review)
Review
Venous thromboembolism (VTE) is a multifactorial disease, and pulmonary hypertension (PH) is a serious condition characterized by pulmonary vascular remodeling leading with increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Although VTE and PH have distinct primary etiologies, they share some pathophysiologic similarities such as dysfunctional vasculature and thrombosis. In both conditions there is solid evidence that EVs derived from a variety of cell types including platelets, monocytes, endothelial cells and smooth muscle cells contribute to vascular endothelial dysfunction, inflammation, thrombosis, cellular activation and communications. However, the roles and importance of EVs substantially differ between studies depending on experimental conditions and parent cell origins of EVs that modify the nature of their cargo. Numerous studies have confirmed that EVs contribute to the pathophysiology of VTE and PH and increased levels of various EVs in relation with the severity of VTE and PH, confirming its potential pathophysiological role and its utility as a biomarker of disease severity and as potential therapeutic targets.
Topics: Humans; Hypertension, Pulmonary; Venous Thromboembolism; Endothelial Cells; Extracellular Vesicles; Thrombosis
PubMed: 38037042
DOI: 10.1186/s12951-023-02216-3 -
Biomedicine & Pharmacotherapy =... Jun 2024Pulmonary hypertension (PH) is a cardiovascular disorder characterized by substantial morbidity and mortality rates. It is a chronic condition characterized by intricate... (Review)
Review
Pulmonary hypertension (PH) is a cardiovascular disorder characterized by substantial morbidity and mortality rates. It is a chronic condition characterized by intricate pathogenesis and uncontrollable factors. We summarized the pathological effects of estrogen, genetics, neuroinflammation, intestinal microbiota, metabolic reorganization, and histone modification on PH. PH is not only a pulmonary vascular disease, but also a systemic disease. The findings emphasize that the onset of PH is not exclusively confined to the pulmonary vasculature, consequently necessitating treatment approaches that extend beyond targeting pulmonary blood vessels. Hence, the research on the pathological mechanism of PH is not limited to target organs such as pulmonary vessels, but also focuses on exploring other fields (such as estrogen, genetics, neuroinflammation, intestinal microbiota, metabolic reorganization, and histone modification).
Topics: Humans; Hypertension, Pulmonary; Animals; Gastrointestinal Microbiome; Estrogens; Neuroinflammatory Diseases
PubMed: 38788548
DOI: 10.1016/j.biopha.2024.116787 -
TLR7/8 activation induces autoimmune vasculopathy and causes severe pulmonary arterial hypertension.The European Respiratory Journal Jul 2023https://bit.ly/3P3H5V0
https://bit.ly/3P3H5V0
Topics: Humans; Pulmonary Arterial Hypertension; Toll-Like Receptor 7; Hypertension, Pulmonary; Vascular Diseases; Familial Primary Pulmonary Hypertension
PubMed: 37290790
DOI: 10.1183/13993003.00204-2023 -
Clinical Therapeutics Sep 2023Pediatric pulmonary hypertension (PH) is a condition characterized by elevated pulmonary arterial pressure, which has the potential to be life-limiting. The etiology of... (Review)
Review
PURPOSE
Pediatric pulmonary hypertension (PH) is a condition characterized by elevated pulmonary arterial pressure, which has the potential to be life-limiting. The etiology of pediatric PH varies. When compared with adult cohorts, the etiology is often multifactorial, with contributions from prenatal, genetic, and developmental factors. This review aims to provide an up-to-date overview of the causes and classification of pediatric PH, describe current therapeutics in pediatric PH, and discuss upcoming and necessary research in pediatric PH.
METHODS
PubMed was searched for articles relating to pediatric pulmonary hypertension, with a particular focus on articles published within the past 10 years. Literature was reviewed for pertinent areas related to this topic.
FINDINGS
The evaluation and approach to pediatric PH are unique when compared with that of adults, in large part because of the different, often multifactorial, causes of the disease in children. Collaborative registry studies have found that the most common disease causes include developmental lung disease and subsets of pulmonary arterial hypertension, which includes genetic variants and PH associated with congenital heart disease. Treatment with PH-targeted therapies in pediatrics is often guided by extrapolation of adult data, small clinical studies in pediatrics, and/or expert consensus opinion. We review diagnostic considerations and treatment in some of the more common pediatric subpopulations of patients with PH, including developmental lung diseases, congenital heart disease, and trisomy 21.
IMPLICATIONS
The care of pediatric patients with PH requires consideration of unique pediatric-specific factors. With significant variability in disease etiology, ongoing efforts are needed to optimize treatment strategies based on disease phenotype and guide evidence-based practices.
Topics: Pregnancy; Adult; Female; Child; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Heart Defects, Congenital; Phenotype
PubMed: 37517916
DOI: 10.1016/j.clinthera.2023.07.001