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Journal articles on the topic 'Pulmonary arterial hypertension (PAH) metabolomics'

Author: Grafiati

Published: 4 June 2025

Last updated: 1 August 2025

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1

Zhao, Jun-Han, Yang-Yang He, Shan-Shan Guo, et al. "Circulating Plasma Metabolomic Profiles Differentiate Rodent Models of Pulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension Patients." American Journal of Hypertension 32, no. 11 (2019): 1109–17. http://dx.doi.org/10.1093/ajh/hpz121.

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Abstract BACKGROUND Pulmonary arterial hypertension (PAH) is a severe progressive disease with systemic metabolic dysregulation. Monocrotaline (MCT)-induced and hypoxia-induced pulmonary hypertension (PH) rodent models are the most widely used preclinical models, however, whether or not these preclinical models recapitulate metabolomic profiles of PAH patients remain unclear. METHODS In this study, a targeted metabolomics panel of 126 small molecule metabolites was conducted. We applied it to the plasma of the 2 preclinical rodent models of PH and 30 idiopathic pulmonary arterial hypertension

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He, Yang-Yang, Yi Yan, Xin Jiang, et al. "Spermine promotes pulmonary vascular remodelling and its synthase is a therapeutic target for pulmonary arterial hypertension." European Respiratory Journal 56, no. 5 (2020): 2000522. http://dx.doi.org/10.1183/13993003.00522-2020.

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Pathological mechanisms of pulmonary arterial hypertension (PAH) remain largely unexplored. Effective treatment of PAH remains a challenge. The aim of this study was to discover the underlying mechanism of PAH through functional metabolomics and to help develop new strategies for prevention and treatment of PAH.Metabolomic profiling of plasma in patients with idiopathic PAH was evaluated through high-performance liquid chromatography mass spectrometry, with spermine identified to be the most significant and validated in another independent cohort. The roles of spermine and spermine synthase we

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Alotaibi, Mona, Yunxian Liu, Gino A. Magalang, et al. "Deriving Convergent and Divergent Metabolomic Correlates of Pulmonary Arterial Hypertension." Metabolites 13, no. 7 (2023): 802. http://dx.doi.org/10.3390/metabo13070802.

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High-dimensional metabolomics analyses may identify convergent and divergent markers, potentially representing aligned or orthogonal disease pathways that underly conditions such as pulmonary arterial hypertension (PAH). Using a comprehensive PAH metabolomics dataset, we applied six different conventional and statistical learning techniques to identify analytes associated with key outcomes and compared the results. We found that certain conventional techniques, such as Bonferroni/FDR correction, prioritized metabolites that tended to be highly intercorrelated. Statistical learning techniques g

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Chen, Yi-Hang, Wen Yuan, Liu-Kun Meng, Jiu-Chang Zhong, and Xiao-Yan Liu. "The Role and Mechanism of Gut Microbiota in Pulmonary Arterial Hypertension." Nutrients 14, no. 20 (2022): 4278. http://dx.doi.org/10.3390/nu14204278.

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Pulmonary arterial hypertension (PAH) is a malignant pulmonary vascular disease characterized by increased pulmonary vascular resistance, pulmonary vasoconstriction, and right ventricular hypertrophy. Recent developments in genomics and metabolomics have gradually revealed the roles of the gut microbiota (GM) and its metabolites in cardiovascular diseases. Accumulating evidence reveals that the GM plays important roles in the occurrence and development of PAH. Gut microbiota dysbiosis directly increases the gut permeability, thereby facilitating pathological bacterial translocation and allowin

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Zhou, Xue-liang, Zhi-bo Liu, Rong-rong Zhu, et al. "NSD2 silencing alleviates pulmonary arterial hypertension by inhibiting trehalose metabolism and autophagy." Clinical Science 133, no. 9 (2019): 1085–96. http://dx.doi.org/10.1042/cs20190142.

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Abstract Nuclear receptor binding SET domain 2 (NSD2)-mediated metabolic reprogramming has been demonstrated to regulate oncogenesis via catalyzing the methylation of histones. The present study aimed to investigate the role of NSD2-mediated metabolic abnormality in pulmonary arterial hypertension (PAH). Monocrotaline (MCT)-induced PAH rat model was established and infected with adeno-associated virus carrying short hairpin RNA (shRNA) targeting NSD2. Hemodynamic parameters, ventricular function, and pathology were evaluated by microcatheter, echocardiography, and histological analysis. Metabo

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Mey, Jacob T., Adithya Hari, Christopher L. Axelrod, et al. "Lipids and ketones dominate metabolism at the expense of glucose control in pulmonary arterial hypertension: a hyperglycaemic clamp and metabolomics study." European Respiratory Journal 55, no. 4 (2020): 1901700. http://dx.doi.org/10.1183/13993003.01700-2019.

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Individuals with idiopathic pulmonary arterial hypertension (PAH) display reduced oral glucose tolerance. This may involve defects in pancreatic function or insulin sensitivity but this hypothesis has not been tested; moreover, fasting nutrient metabolism remains poorly described in PAH. Thus, we aimed to characterise fasting nutrient metabolism and investigated the metabolic response to hyperglycaemia in PAH.12 participants (six PAH, six controls) were administered a hyperglycaemic clamp, while 52 (21 PAH, 31 controls) underwent plasma metabolomic analysis. Glucose, insulin, C-peptide, free f

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Sanders, Jason L., Yuchi Han, Mariana F. Urbina, David M. Systrom, and Aaron B. Waxman. "Metabolomics of exercise pulmonary hypertension are intermediate between controls and patients with pulmonary arterial hypertension." Pulmonary Circulation 9, no. 4 (2019): 204589401988262. http://dx.doi.org/10.1177/2045894019882623.

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Mechanisms underlying pulmonary arterial hypertension (PAH) remain elusive. Pulmonary arterial hypertension and exercise PH share similar physiologic consequences; it is debated whether they share biologic mechanisms and if exercise PH represents an early phase of pulmonary arterial hypertension. We conducted an observational study to test if there is a graded metabolic disturbance along the severity of PH, which may indicate shared or disparate pathophysiology. Individuals referred to an academic medical dyspnea center with unexplained exertional intolerance underwent invasive cardiopulmonary

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Wilkins, Martin R., Jurjan Aman, Lars Harbaum, Anna Ulrich, John Wharton, and Christopher J. Rhodes. "Recent advances in pulmonary arterial hypertension." F1000Research 7 (July 24, 2018): 1128. http://dx.doi.org/10.12688/f1000research.14984.1.

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Pulmonary arterial hypertension (PAH) is a rare disorder with a high mortality rate. Treatment options have improved in the last 20 years, but patients still die prematurely of right heart failure. Though rare, it is heterogeneous at the genetic and molecular level, and understanding and exploiting this is key to the development of more effective treatments. BMPR2, encoding bone morphogenetic receptor type 2, is the most commonly affected gene in both familial and non-familial PAH, but rare mutations have been identified in other genes. Transcriptomic, proteomic, and metabolomic studies lookin

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Bassareo, Pier Paolo, and Michele D’Alto. "Metabolomics in Pulmonary Hypertension—A Useful Tool to Provide Insights into the Dark Side of a Tricky Pathology." International Journal of Molecular Sciences 24, no. 17 (2023): 13227. http://dx.doi.org/10.3390/ijms241713227.

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Pulmonary hypertension (PH) is a multifaceted illness causing clinical manifestations like dyspnea, fatigue, and cyanosis. If left untreated, it often evolves into irreversible pulmonary arterial hypertension (PAH), leading to death. Metabolomics is a laboratory technique capable of providing insights into the metabolic pathways that are responsible for a number of physiologic or pathologic events through the analysis of a biological fluid (such as blood, urine, and sputum) using proton nuclear magnetic resonance spectroscopy or mass spectrometry. A systematic review was finalized according to

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Wawrzyniak, Renata, Tamara Gaillard, Margot Biesemans, et al. "Plasma Multiplatform Metabolomics Towards Evaluation of Gender Differences in Pulmonary Arterial Hypertension—A Pilot Study." Biomedicines 13, no. 7 (2025): 1637. https://doi.org/10.3390/biomedicines13071637.

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Background: Pulmonary arterial hypertension (PAH) is a rare and severe condition characterized by increased pulmonary arterial pressure and vascular resistance. Women are more susceptible to PAH yet have higher survival rates than men, a phenomenon called the “estrogen paradox”. This study aims to investigate the sex-based differences in PAH using plasma untargeted metabolomics. Methods: Plasma samples were collected from 43 PAH patients and 37 healthy controls. The samples were analyzed using two complementary analytical techniques: gas chromatography–mass spectrometry (GC-QqQ/MS) and liquid

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Hogan, Sarah E., Maria Pia Rodriguez Salazar, John Cheadle, et al. "Mesenchymal stromal cell-derived exosomes improve mitochondrial health in pulmonary arterial hypertension." American Journal of Physiology-Lung Cellular and Molecular Physiology 316, no. 5 (2019): L723—L737. http://dx.doi.org/10.1152/ajplung.00058.2018.

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Secreted exosomes are bioactive particles that elicit profound responses in target cells. Using targeted metabolomics and global microarray analysis, we identified a role of exosomes in promoting mitochondrial function in the context of pulmonary arterial hypertension (PAH). Whereas chronic hypoxia results in a glycolytic shift in pulmonary artery smooth muscle cells (PASMCs), exosomes restore energy balance and improve O2 consumption. These results were confirmed in a hypoxia-induced mouse model and a semaxanib/hypoxia rat model of PAH wherein exosomes improved the mitochondrial dysfunction a

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Mamazhakypov, Argen, Astrid Weiß, Sven Zukunft, et al. "Effects of macitentan and tadalafil monotherapy or their combination on the right ventricle and plasma metabolites in pulmonary hypertensive rats." Pulmonary Circulation 10, no. 4 (2020): 204589402094728. http://dx.doi.org/10.1177/2045894020947283.

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Pulmonary arterial hypertension is a severe respiratory disease characterized by pulmonary artery remodeling. RV dysfunction and dysregulated circulating metabolomics are associated with adverse outcomes in pulmonary arterial hypertension. We investigated effects of tadalafil and macitentan alone or in combination on the RV and plasma metabolomics in SuHx and PAB models. For SuHx model, rats were injected with SU5416 and exposed to hypoxia for three weeks and then were returned to normoxia and treated with either tadalafil (10 mg/kg in chow) or macitentan (10 mg/kg in chow) or their combinatio

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Mukesh, Kataria, Welch Bonnie, E. Heck Diane, and Duck Kim Hong. "Integrative molecular navigator in preventable diseases: Pulmonary arterial hypertension (PAH) and rare diseases." GSC Biological and Pharmaceutical Sciences 12, no. 3 (2020): 204–7. https://doi.org/10.5281/zenodo.4266069.

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Pulmonary arterial hypertension (PAH) is a debilitating lung condition for which there has been no cure that leads to complications on the right side of the heart. This disorder is currently distinguished from other conditions of the right ventricle by a DNA biorepository, invasive hemodynamics, echocardiography, genotype-tissue expression, imaging, and other diagnostic tools. Present treatment strategies include mainly medications and oxygen therapy choices. Advanced omics developments, including next generational genetic analysis, massively parallel gene-editing, metabolomics, and pharmacoge

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Yuan, Chao, Huan-Xin Chen, Hai-Tao Hou, Jun Wang, Qin Yang, and Guo-Wei He. "Protein biomarkers and risk scores in pulmonary arterial hypertension associated with ventricular septal defect: integration of multi-omics and validation." American Journal of Physiology-Lung Cellular and Molecular Physiology 319, no. 5 (2020): L810—L822. http://dx.doi.org/10.1152/ajplung.00167.2020.

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The molecular mechanisms underlying pulmonary arterial hypertension (PAH) in congenital ventricular septal defects (VSD) are unclear. We aimed to reveal molecular pathways and potential biomarkers by multi-omics analysis in VSD-PAH. Plasma from 160 children, including 120 VSD patients with/without PAH and 40 healthy children was studied by integrated proteomics, metabolomics, and bioinformatics analyses. Proteomics identified 107 differential proteins (DPs) between patients with/without PAH including significantly increased adiponectin (ADIPO), dopamine β-hydroxylase (DBH), alanyl membrane ami

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Alamri, Abdulwahab, Abdulhadi Burzangi, Paul Coats, and David Watson. "Untargeted Metabolic Profiling Cell-Based Approach of Pulmonary Artery Smooth Muscle Cells in Response to High Glucose and the Effect of the Antioxidant Vitamins D and E." Metabolites 8, no. 4 (2018): 87. http://dx.doi.org/10.3390/metabo8040087.

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Pulmonary arterial hypertension (PAH) is a multi-factorial disease characterized by the hyperproliferation of pulmonary artery smooth muscle cells (PASMCs). Excessive reactive oxygen species (ROS) formation resulted in alterations of the structure and function of pulmonary arterial walls, leading to right ventricular failure and death. Diabetes mellitus has not yet been implicated in pulmonary hypertension. However, recently, variable studies have shown that diabetes is correlated with pulmonary hypertension pathobiology, which could participate in the modification of pulmonary artery muscles.

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Xu, Beizhu, Caihua Huang, Caojin Zhang, Donghai Lin, and Weifeng Wu. "NMR-Based Metabolomic Analysis of Plasma in Patients with Adult Congenital Heart Disease and Associated Pulmonary Arterial Hypertension: A Pilot Study." Metabolites 12, no. 9 (2022): 845. http://dx.doi.org/10.3390/metabo12090845.

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Patients with unrepaired congenital heart disease (CHD) are prone to pulmonary arterial hypertension (PAH). The ovine pulmonary arterial smooth muscle cells exposed to increased pulmonary blood flow (PBF) exhibited hyperproliferation and metabolic alterations, but the metabolic disorders of patients with CHD and associated PAH (PAH-CHD) have not yet been fully understood. Adult CHD patients were prospectively included and divided into the PAH-CHD group (n = 24) and CHD group (n = 38), while healthy adults were included as healthy control (HC) group (n = 29). Plasma from each subject was prepar

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Reem, El Kabbout, Abi Sleimen Antonella, Boucherat Olivier, Bonnet Sebastien, Provencher Steeve, and Potus Francois. "Multiomics Integration for Identifying Treatment Targets, Drug Development, and Diagnostic Designs in PAH." Advances in Pulmonary Hypertension 23, no. 2 (2025): 33–42. https://doi.org/10.21693/1933-088x-23.2.33.

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Unraveling the complexities of pulmonary arterial hypertension (PAH) is challenging due to its multifaceted nature, encompassing molecular, cellular, tissue, and organ-level alterations. The advent of omics technologies, including genomics, epigenomics, transcriptomics, metabolomics, and proteomics, has generated a vast array of public and nonpublic datasets from both humans and model organisms, opening new avenues for understanding PAH. However, the insights provided by individual omics datasets into the molecular mechanisms of PAH are inherently limited. In response, efforts are increasing

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Gu, Sue, Khushboo Goel, Lindsay M. Forbes, et al. "Tensions in Taxonomies: Current Understanding and Future Directions in the Pathobiologic Basis and Treatment of Group 1 and Group 3 Pulmonary Hypertension." Comprehensive Physiology 13, no. 1 (2023): 4295–319. https://doi.org/10.1002/j.2040-4603.2023.tb00251.x.

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AbstractIn the over 100 years since the recognition of pulmonary hypertension (PH), immense progress and significant achievements have been made with regard to understanding the pathophysiology of the disease and its treatment. These advances have been mostly in idiopathic pulmonary arterial hypertension (IPAH), which was classified as Group 1 Pulmonary Hypertension (PH) at the Second World Symposia on PH in 1998. However, the pathobiology of PH due to chronic lung disease, classified as Group 3 PH, remains poorly understood and its treatments thus remain limited. We review the history of the

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Jendrek, Sebastian T., Franziska Schmelter, Christian Sina, Ulrich L. Günther, and Gabriela Riemekasten. "The Metabolomic View of Systemic Sclerosis—A Systematic Literature Review." Sclerosis 3, no. 2 (2025): 18. https://doi.org/10.3390/sclerosis3020018.

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The mortality risk in systemic sclerosis (SSc) is primarily determined by pulmonary involvement (interstitial lung disease (ILD), pulmonary fibrosis), pulmonary arterial hypertension (PAH), and cardiac involvement. With timely and intensive treatment, the disease can be halted or even improved. Therefore, early diagnosis remains crucial. Unfortunately, biomarkers currently available cannot meet this requirement. SSc is characterized by autoimmune inflammation, vasculopathy, and fibrosis. The immunometabolic characterization of autoimmune diseases contributes to a better understanding of the un

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Ahmad, Imama, Gabriele Grunig, Anna Hemnes, et al. "0449 Unbiased Examination of Metabolomic Signatures in Sleep Disordered Breathing and Group 1 PAH in the (PVDOMICS) Cohort." SLEEP 46, Supplement_1 (2023): A199—A200. http://dx.doi.org/10.1093/sleep/zsad077.0449.

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Abstract Introduction Metabolic alterations have been identified as a novel driver of pulmonary arterial hypertension (PAH) pathogenesis. Sleep disordered breathing (SDB), characterized by intermittent hypoxemic episodes, has also been implicated in PAH, particularly when associated with more profound degrees of hypoxia, and metabolic dysfunction. We hypothesize to observe in SDB and PAH in humans a similar pattern of metabolic alterations as previously described in animal models of PAH. Methods Pulmonary Vascular Disease Phenomics (PVDOMICS,NCT02980887), a multicenter study, was designed in p

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Olsson, Karen M., and Richard Channick. "Pregnancy in pulmonary arterial hypertension." European Respiratory Review 25, no. 142 (2016): 431–37. http://dx.doi.org/10.1183/16000617.0079-2016.

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Despite advanced therapies, maternal mortality in women with pulmonary arterial hypertension (PAH) remains high in pregnancy and is especially high during the post-partum period. However, recent data indicates that morbidity and mortality during pregnancy and after birth have improved for PAH patients. The current European Society of Cardiology/European Respiratory Society guidelines recommend that women with PAH should not become pregnant. Therefore, the risks associated with pregnancy must be emphasised and counselling offered to women at the time of PAH diagnosis and to women with PAH who b

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Graham, Brian B. "Schistosomiasis-Associated Pulmonary Arterial Hypertension." Advances in Pulmonary Hypertension 21, no. 4 (2022): 109–14. http://dx.doi.org/10.21693/1933-088x-21.4.109.

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Schistosomiasis is a major cause of group 1 pulmonary arterial hypertension (PAH) worldwide. Schistosomiasis results from a parasitic infection present in over 200 million individuals worldwide. Schistosomiasis-associated PAH was initially thought to be obstructive due to egg embolization but has a pulmonary vascular pathology like other forms of group 1 PAH and can be treated using conventional PAH therapies. Mechanisms that underlie the development of schistosomiasis-associated PAH include type 2 inflammation which triggers TGF-β signaling; importantly, TGF-β signaling is a pathway shared wi

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Celermajer, David, Anne Keogh, Vivek Thakkar, and Edmund Lau. "Screening of Pulmonary Arterial Hypertension." Seminars in Respiratory and Critical Care Medicine 38, no. 05 (2017): 596–605. http://dx.doi.org/10.1055/s-0037-1606202.

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The majority of patients with pulmonary arterial hypertension (PAH) present in advanced stages of the disease, when therapeutic interventions may potentially be less effective. Early diagnosis is associated with improved outcomes, supporting best practice recommendations that asymptomatic patients in high-risk populations such as systemic sclerosis or carriers of a PAH-causing mutation should be offered periodic screening for PAH. An evidence-based algorithm for screening systemic sclerosis-associated PAH is now available based on the DETECT study. A multimodal approach using Doppler echocardi

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Savale, Laurent, and Alessandra Manes. "Pulmonary arterial hypertension populations of special interest: portopulmonary hypertension and pulmonary arterial hypertension associated with congenital heart disease." European Heart Journal Supplements 21, Supplement_K (2019): K37—K45. http://dx.doi.org/10.1093/eurheartj/suz221.

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Abstract Guidelines exist for management of pulmonary arterial hypertension (PAH), but information is limited for certain patient subgroups, including adults with portopulmonary hypertension (PoPH) or with PAH associated with congenital heart disease (PAH-CHD). This article discusses screening, clinical management, and prognosis in PoPH and PAH-CHD and, as such, considers the most recent clinical data and expert advice. A multidisciplinary consultation and follow-up by specialists are crucial for management of both PoPH and PAH-CHD, but each condition presents with unique challenges. Developme

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Chhabra, Shibba, Gurleen Kaur, Gagandeep Nagi, et al. "Pulmonary Arterial Hypertension and Pregnancy." Indian Journal of Cardiovascular Disease in Women WINCARS 03, no. 02/03 (2018): 139–48. http://dx.doi.org/10.1055/s-0038-1677055.

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AbstractPulmonary hypertension in pregnancy has always scared the treating physician and gynecologist due to reported high mortality since ages. The upcoming therapies targeting pulmonary hypertension (calcium channel blockers, nitric oxide, endothelin receptor antagonist, phosphodiesterase type 5 inhibitors) and improvement in hemodynamic monitoring and intensive management in pulmonary arterial hypertension (PAH) specialist centers give a ray of hope to these patients. Termination of pregnancy continues to be a management modality in pregnant patients with PAH. Multidisciplinary approach tar

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Azar, Jehad, Mohammed Ayyad, Yasmin Jaber, and Laith Azzam Ayasa. "Scurvy-induced pulmonary arterial hypertension." BMJ Case Reports 16, no. 4 (2023): e254730. http://dx.doi.org/10.1136/bcr-2023-254730.

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Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that results in precapillary pulmonary hypertension. PAH is caused by a group of clinical conditions involving multiple organ systems. Several cases have been reported in the literature demonstrating an association between vitamin C deficiency and PAH. Low endothelial nitric oxide levels in the pulmonary vasculature, combined with the inappropriate activation of hypoxia-inducible transcription factors, seen in patients with ascorbic acid deficiency, are believed to be the main contributors to the pathog

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Chew, Joshua, James Loyd, and Eric Austin. "Genetics of Pulmonary Arterial Hypertension." Seminars in Respiratory and Critical Care Medicine 38, no. 05 (2017): 585–95. http://dx.doi.org/10.1055/s-0037-1606201.

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Tremendous progress has been made in understanding the genetics of pulmonary arterial hypertension (PAH) since its description in the 1950s as a primary disorder of the pulmonary vasculature. Heterozygous germline mutations in the gene coding bone morphogenetic receptor type 2 (BMPR2) are detectable in the majority of cases of heritable PAH, and in approximately 20% of cases of idiopathic pulmonary arterial hypertension (IPAH). However, recent advances in gene discovery methods have facilitated the discovery of additional genes with mutations among those with and without familial PAH. Heritabl

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Welch, Carrie L., and Wendy K. Chung. "Channelopathy Genes in Pulmonary Arterial Hypertension." Biomolecules 12, no. 2 (2022): 265. http://dx.doi.org/10.3390/biom12020265.

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Pulmonary arterial hypertension (PAH) is a rare, progressive vasculopathy with significant cardiopulmonary morbidity and mortality. The underlying pathogenetic mechanisms are heterogeneous and current therapies aim to decrease pulmonary vascular resistance but no curative treatments are available. Causal genetic variants can be identified in ~13% of adults and 43% of children with PAH. Knowledge of genetic diagnoses can inform clinical management of PAH, including multimodal medical treatment, surgical intervention and transplantation decisions, and screening for associated conditions, as well

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Boucherat, Olivier, Sophie Chabot, Fabrice Antigny, Frédéric Perros, Steeve Provencher, and Sébastien Bonnet. "Potassium channels in pulmonary arterial hypertension." European Respiratory Journal 46, no. 4 (2015): 1167–77. http://dx.doi.org/10.1183/13993003.00798-2015.

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Pulmonary arterial hypertension (PAH) is a devastating cardiopulmonary disorder with various origins. All forms of PAH share a common pulmonary arteriopathy characterised by vasoconstriction, remodelling of the pre-capillary pulmonary vessel wall, and in situ thrombosis. Although the pathogenesis of PAH is recognised as a complex and multifactorial process, there is growing evidence that potassium channels dysfunction in pulmonary artery smooth muscle cells is a hallmark of PAH. Besides regulating many physiological functions, reduced potassium channels expression and/or activity have signific

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Sahay, Sandeep, Marc Humbert, and Olivier Sitbon. "Medical Treatment of Pulmonary Arterial Hypertension." Seminars in Respiratory and Critical Care Medicine 38, no. 05 (2017): 686–700. http://dx.doi.org/10.1055/s-0037-1607208.

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AbstractPulmonary arterial hypertension (PAH) is a progressive and life-threatening disease leading to right ventricular failure and death if left untreated. Over the past two decades, progress in the understanding of pathophysiological mechanisms of the disease has led to the development of medications targeting the three major pathways of endothelial dysfunction: prostanoids, endothelin-receptor antagonists, and phosphodiesterase type-5 inhibitors. Efficacy of PAH-targeted medications has been demonstrated in monotherapy through randomized clinical trials leading to their regulatory approval

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Napoli, Claudio, Giuditta Benincasa, and Joseph Loscalzo. "Epigenetic Inheritance Underlying Pulmonary Arterial Hypertension." Arteriosclerosis, Thrombosis, and Vascular Biology 39, no. 4 (2019): 653–64. http://dx.doi.org/10.1161/atvbaha.118.312262.

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In pulmonary arterial hypertension (PAH), the Warburg effect (glycolytic shift) and mitochondrial fission are determinants of phenotype alterations characteristic of the disease, such as proliferation, apoptosis resistance, migration, endothelial-mesenchymal transition, and extracellular matrix stiffness. Current therapies, focusing largely on vasodilation and antithrombotic protection, do not restore these aberrant phenotypes suggesting that additional pathways need be targeted. The multifactorial nature of PAH suggests epigenetic changes as potential determinants of vascular remodeling. Tran

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Foris, Vasile, Gabor Kovacs, Leigh M. Marsh, et al. "CD133+ cells in pulmonary arterial hypertension." European Respiratory Journal 48, no. 2 (2016): 459–69. http://dx.doi.org/10.1183/13993003.01523-2015.

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Circulating mononuclear cells may play an important role for the vascular remodelling in pulmonary arterial hypertension (PAH), but studies addressing multiple progenitor populations are rare and inconsistent.We used a comprehensive fluorescence-activated cell sorting analysis of circulating mononuclear cells in 20 PAH patients and 20 age- and sex-matched controls, and additionally analysed CD133+ cells in the lung tissue of five PAH transplant recipients and five healthy controls (donor lungs).PAH patients were characterised by increased numbers of circulating CD133+ cells and lymphopenia as

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Fowler, Robin M., Kevin R. Gain, and Eli Gabbay. "Exercise Intolerance in Pulmonary Arterial Hypertension." Pulmonary Medicine 2012 (2012): 1–10. http://dx.doi.org/10.1155/2012/359204.

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Pulmonary arterial hypertension (PAH) is associated with symptoms of dyspnea and fatigue, which contribute to exercise limitation. The origins and significance of dyspnea and fatigue in PAH are not completely understood. This has created uncertainly among healthcare professionals regarding acceptable levels of these symptoms, on exertion, for patients with PAH. Dysfunction of the right ventricle (RV) contributes to functional limitation and mortality in PAH; however, the role of the RV in eliciting dyspnea and fatigue has not been thoroughly examined. This paper explores the contribution of th

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Al-Naamani, Nadine, Aaron W. Trammell, and Zeenat Safdar. "Circulating Biomarkers in Pulmonary Arterial Hypertension." Advances in Pulmonary Hypertension 14, no. 1 (2015): 21–27. http://dx.doi.org/10.21693/1933-088x-14.1.21.

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Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that leads to exercise limitation, right heart failure, and death. There is a need for biomarkers that can aid in early detection, disease surveillance, and treatment monitoring in PAH. Several potential molecules have been investigated; however, only brain natriuretic peptide is currently recommended at diagnosis and for follow-up of PAH patients. This review will focus on potential biomarkers in PAH and will discuss their pathophysiology, prognostic significance, as well as their limitations.

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Koudstaal, Thomas, Karin A. Boomars, and Mirjam Kool. "Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: An Immunological Perspective." Journal of Clinical Medicine 9, no. 2 (2020): 561. http://dx.doi.org/10.3390/jcm9020561.

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Pulmonary hypertension (PH) is a debilitating progressive disease characterized by increased pulmonary arterial pressures, leading to right ventricular (RV) failure, heart failure and, eventually, death. Based on the underlying conditions, PH patients can be subdivided into the following five groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) PH due to lung disease, (4) chronic thromboembolic PH (CTEPH), and (5) PH with unclear and/or multifactorial mechanisms. Currently, even with PAH-specific drug treatment, prognosis for PAH and CTEPH patients remains p

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Dumitrescu, Daniel, Olivier Sitbon, Jason Weatherald, and Luke S. Howard. "Exertional dyspnoea in pulmonary arterial hypertension." European Respiratory Review 26, no. 145 (2017): 170039. http://dx.doi.org/10.1183/16000617.0039-2017.

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Dyspnoea is a principal presenting symptom in pulmonary arterial hypertension (PAH), and often the most distressing. The pathophysiology of PAH is relatively well understood, with the primary abnormality of pulmonary vascular disease resulting in a combination of impaired cardiac output on exercise and abnormal gas exchange, both contributing to increased ventilatory drive. However, increased ventilatory drive is not the sole explanation for the complex neurophysiological and neuropsychological symptom of dyspnoea, with other significant contributions from skeletal muscle reflexes, respiratory

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Popova, E. K., N. S. Arkhipova, N. V. Ilyin, et al. "Pulmonary arterial hypertension associated with systemic scleroderma." Clinical Medicine (Russian Journal) 99, no. 1 (2021): 68–74. http://dx.doi.org/10.30629/0023-2149-2021-99-1-68-74.

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Pulmonary arterial hypertension (PAH) is an orphan disease characterized by an increase in pulmonary vascular resistance (PVR). PAH is a pathology, difficult to diagnose due to the non-specificity of its first strokes. The prognosis of PAH is extremely unfavorable without early diagnosis and treatment, as with systemic scleroderma, 60% of patients die in the first 2 years. In the Republic of Sakha (Yakutia), there are currently 38 patients with PAH, and a tendency towards an increase in their number is noted. The necessity to conduct scientific research on PAH patients living in the Republic o

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Vang, Shia, Phillip Cochran, Julio Sebastian Domingo, Stefanie Krick, and Jarrod Wesley Barnes. "The Glycobiology of Pulmonary Arterial Hypertension." Metabolites 12, no. 4 (2022): 316. http://dx.doi.org/10.3390/metabo12040316.

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Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease of complex etiology. Cases of PAH that do not receive therapy after diagnosis have a low survival rate. Multiple reports have shown that idiopathic PAH, or IPAH, is associated with metabolic dysregulation including altered bioavailability of nitric oxide (NO) and dysregulated glucose metabolism. Multiple processes such as increased proliferation of pulmonary vascular cells, angiogenesis, apoptotic resistance, and vasoconstriction may be regulated by the metabolic changes demonstrated in PAH. Recent reports have u

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Rhee, Laura S., Alisha A. Morgan, and Hilary M. DuBrock. "Palliative Care and Pulmonary Arterial Hypertension." Advances in Pulmonary Hypertension 21, no. 1 (2022): 7–11. http://dx.doi.org/10.21693/1933-088x-21.1.7.

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Background Pulmonary arterial hypertension (PAH) is a chronic and progressive disease associated with impaired health-related quality of life and survival. Palliative care (PC) is patient- and family-centered care provided by an interdisciplinary team with an overarching goal of alleviating suffering and improving quality of life for patients with advanced illness. PC in other chronic diseases is associated with improved quality of life, symptom management, illness understanding, and reduced caregiver burden, but there is limited data regarding PC in PAH. Despite limited evidence, there is str

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Winicki, Nolan M., Cristian Puerta, Casandra E. Besse, Yu Zhang, and Patricia A. Thistlethwaite. "NOTCH3 and Pulmonary Arterial Hypertension." International Journal of Molecular Sciences 25, no. 11 (2024): 6248. http://dx.doi.org/10.3390/ijms25116248.

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NOTCH3 receptor signaling has been linked to the regulation of smooth muscle cell proliferation and the maintenance of smooth muscle cells in an undifferentiated state. Pulmonary arterial hypertension (World Health Organization Group 1 idiopathic disease: PAH) is a fatal disease characterized clinically by elevated pulmonary vascular resistance caused by extensive vascular smooth muscle cell proliferation, perivascular inflammation, and asymmetric neointimal hyperplasia in precapillary pulmonary arteries. In this review, a detailed overview of the specific role of NOTCH3 signaling in PAH, incl

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Hu, Yijie, Leon Chi, Wolfgang M. Kuebler, and Neil M. Goldenberg. "Perivascular Inflammation in Pulmonary Arterial Hypertension." Cells 9, no. 11 (2020): 2338. http://dx.doi.org/10.3390/cells9112338.

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Perivascular inflammation is a prominent pathologic feature in most animal models of pulmonary hypertension (PH) as well as in pulmonary arterial hypertension (PAH) patients. Accumulating evidence suggests a functional role of perivascular inflammation in the initiation and/or progression of PAH and pulmonary vascular remodeling. High levels of cytokines, chemokines, and inflammatory mediators can be detected in PAH patients and correlate with clinical outcome. Similarly, multiple immune cells, including neutrophils, macrophages, dendritic cells, mast cells, T lymphocytes, and B lymphocytes ch

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Preda, Mariana, Andrei Seferian, Etienne-Marie Jutant, et al. "Tyrosine Kinase Inhibitor–Induced Pulmonary Arterial Hypertension." Advances in Pulmonary Hypertension 17, no. 2 (2018): 69–74. http://dx.doi.org/10.21693/1933-088x-17.2.69.

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The treatment of the malignant hematological diseases has been revolutionized by the use of tyrosine kinase inhibitors (TKI): for example, imatinib in patients with chronic myeloid leukemia. Dasatinib, a second-generation TKI, has been reported to induce severe pulmonary arterial hypertension (PAH). The mechanism of PAH development is presumed to be endothelial cell toxicity through the production of mitochondrial reactive oxygen species. There are other TKIs that are reported to cause PAH, such as: ponatinib, bosutinib, lapatinib, and lorlatinib. The management of PAH due to TKIs primarily in

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Tofovic, Stevan P., and Edwin K. Jackson. "Estradiol Metabolism: Crossroads in Pulmonary Arterial Hypertension." International Journal of Molecular Sciences 21, no. 1 (2019): 116. http://dx.doi.org/10.3390/ijms21010116.

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Pulmonary arterial hypertension (PAH) is a debilitating and progressive disease that predominantly develops in women. Over the past 15 years, cumulating evidence has pointed toward dysregulated metabolism of sex hormones in animal models and patients with PAH. 17β-estradiol (E2) is metabolized at positions C2, C4, and C16, which leads to the formation of metabolites with different biological/estrogenic activity. Since the first report that 2-methoxyestradiol, a major non-estrogenic metabolite of E2, attenuates the development and progression of experimental pulmonary hypertension (PH), it has

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Mirrakhimov, Aibek E., Alaa M. Ali, Aram Barbaryan, and Suartcha Prueksaritanond. "Human Immunodeficiency Virus and Pulmonary Arterial Hypertension." ISRN Cardiology 2013 (August 21, 2013): 1–11. http://dx.doi.org/10.1155/2013/903454.

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Human immunodeficiency virus- (HIV-) related pulmonary arterial hypertension (PAH) is a rare complication of HIV infection. The pathophysiology of HIV-related PAH is complex, with viral proteins seeming to play the major role. However, other factors, such as coinfection with other microorganisms and HIV-related systemic inflammation, might also contribute. The clinical presentation of HIV-related PAH and diagnosis is similar to other forms of pulmonary hypertension. Both PAH-specific therapies and HAART are important in HIV-related PAH management. Future studies investigating the pathogenesis

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Meloche, Jolyane, Marie Le Guen, François Potus, et al. "miR-223 reverses experimental pulmonary arterial hypertension." American Journal of Physiology-Cell Physiology 309, no. 6 (2015): C363—C372. http://dx.doi.org/10.1152/ajpcell.00149.2015.

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Pulmonary arterial hypertension (PAH) is a devastating disease affecting lung vasculature. The pulmonary arteries become occluded due to increased proliferation and suppressed apoptosis of the pulmonary artery smooth muscle cells (PASMCs) within the vascular wall. It was recently shown that DNA damage could trigger this phenotype by upregulating poly(ADP-ribose)polymerase 1 (PARP-1) expression, although the exact mechanism remains unclear. In silico analyses and studies in cancer demonstrated that microRNA miR-223 targets PARP-1. We thus hypothesized that miR-223 downregulation triggers PARP-1

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Tyagi, Sanjay, and Vishal Batra. "Novel Therapeutic Approaches of Pulmonary Arterial Hypertension." International Journal of Angiology 28, no. 02 (2019): 112–17. http://dx.doi.org/10.1055/s-0039-1692140.

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AbstractPulmonary arterial hypertension (PAH) is an uncommon disease characterized progressive remodeling of pulmonary vasculature. Although treatment for PAH have improved in last two decades but the outcome remains fatal. Currently, the therapies for PAH target three well-established pathways the nitric oxide (NO) pathway, endothelin receptors, and prostanoids. There are multiple potential targets for development of newer drugs in PAH which requires meticulous research and clinical trials.

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Boucherat, Olivier, Geraldine Vitry, Isabelle Trinh, Roxane Paulin, Steeve Provencher, and Sebastien Bonnet. "The cancer theory of pulmonary arterial hypertension." Pulmonary Circulation 7, no. 2 (2017): 285–99. http://dx.doi.org/10.1177/2045893217701438.

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Pulmonary arterial hypertension (PAH) remains a mysterious killer that, like cancer, is characterized by tremendous complexity. PAH development occurs under sustained and persistent environmental stress, such as inflammation, shear stress, pseudo-hypoxia, and more. After inducing an initial death of the endothelial cells, these environmental stresses contribute with time to the development of hyper-proliferative and apoptotic resistant clone of cells including pulmonary artery smooth muscle cells, fibroblasts, and even pulmonary artery endothelial cells allowing vascular remodeling and PAH dev

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Zakiev, V., T. Martynyuk, and Y. Kotovskaya. "Current treatment of pulmonary arterial hypertension." Clinical pharmacology and therapy 35, no. 3 (2024): 14–20. http://dx.doi.org/10.32756/0869-5490-2024-3-14-20.

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Pulmonary arterial hypertension (PAH) is a rare, progressive, life-threatening disease that leads to the right ventricular heart failure and premature death. Available specific therapies act primarily as vasodilators and have a limited effect on the biological cause of the disease, that is, the uncontrolled proliferation of vascular endothelial and smooth muscle cells. Currently used drugs for the treatment of PAH, as well as potential drugs that are in various phases of clinical trials are reviewed in the article. The authors discuss also the issue of drug provision for patients with PAH.

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Singh, Inderjit, Farbod N. Rahaghi, Robert Naeije, et al. "Dynamic right ventricular–pulmonary arterial uncoupling during maximum incremental exercise in exercise pulmonary hypertension and pulmonary arterial hypertension." Pulmonary Circulation 9, no. 3 (2019): 204589401986243. http://dx.doi.org/10.1177/2045894019862435.

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Despite recent advances, the prognosis of pulmonary hypertension (PH) remains poor. While the initial insult in PH implicates the pulmonary vasculature, the functional state, exercise capacity, and survival of such patients are closely linked to right ventricular (RV) function. In the current study, we sought to investigate the effects of maximum incremental exercise on the matching of RV contractility and afterload (i.e. right ventricular–pulmonary arterial [RV–PA] coupling) in patients with exercise PH (ePH) and pulmonary arterial hypertension (PAH). End-systolic elastance (Ees), pulmonary a

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Cullivan, Sarah, Margaret Higgins, and Sean Gaine. "Diagnosis and management of pulmonary arterial hypertension." Breathe 18, no. 4 (2022): 220168. http://dx.doi.org/10.1183/20734735.0168-2022.

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Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that is characterised by elevated pressures within the pulmonary vascular tree. Recent decades have witnessed a dramatic expansion in our understanding of the pathobiology and the epidemiology of PAH, and improvements in treatment options and outcomes. The prevalence of PAH is estimated to be between 48 and 55 cases per million adults. The definition was recently amended and a diagnosis of PAH now requires evidence of a mean pulmonary artery pressure >20 mmHg, a pulmonary vascular resistance >2 Wo

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