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Full Text Journal Articles about
Idiopathic Pulmonary Arterial Hypertension

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Management of systemic sclerosis: the first five years.

David Roofeh, Dinesh Khanna,

PURPOSE OF REVIEW:This review provides a risk-stratified and evidence-based management for subsets of systemic sclerosis (SSc) patients in the first five years from disease onset. RECENT FINDINGS:Cardiopulmonary disease remains the primary cause of mortality in SSc patients. Morbidity and mortality in SSc-associated pulmonary arterial hypertension have improved with combination treatment, ... Read more >>

Curr Opin Rheumatol (Current opinion in rheumatology)
[2020, 32(3):228-237]

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Therapeutic Use of Extracellular Vesicles for Acute and Chronic Lung Disease.

Erin N Worthington, James S Hagood,

Multipotent mesenchymal stem cells (MSCs) possess regenerative properties and have been shown to improve outcomes and survival in acute and chronic lung diseases, but there have been some safety concerns raised related to MSC-based therapy. Subsequent studies have demonstrated that many of the regenerative effects of MSCs can be attributed ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(7):]

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Comprehensive identification of signaling pathways for idiopathic pulmonary arterial hypertension.

Bingxun Liu, Liping Zhu, Ping Yuan, Glenn Marsboom, Zhigang Hong, Jinming Liu, Peng Zhang, Qinghua Hu,

Whole exome sequencing (WES) was used in the research of familial pulmonary arterial hypertension (FPAH). CAV1 and KCNK3 were found as two novel candidate genes of FPAH. However, few pathogenic genes were identified in idiopathic pulmonary arterial hypertension (IPAH). We conducted WES in 20 unrelated IPAH patients that did not ... Read more >>

Am. J. Physiol., Cell Physiol. (American journal of physiology. Cell physiology)
[2020, :]

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Association of Rare PTGIS Variants With Susceptibility and Pulmonary Vascular Response in Patients With Idiopathic Pulmonary Arterial Hypertension.

Xiao-Jian Wang, Xi-Qi Xu, Kai Sun, Ke-Qiang Liu, Su-Qi Li, Xin Jiang, Qin-Hua Zhao, Lan Wang, Fu-Hua Peng, Jue Ye, Yan Wu, Rui Jiang, Jin Zhang, Wei Huang, Wen-Bin Wei, Yi Yan, Jing-Hui Li, Qian-Qian Liu, Sheng Li, Yong Wang, Shu-Yang Zhang, Xue Zhang, Zhi-Cheng Jing,

Importance:Idiopathic pulmonary arterial hypertension (IPAH) is a fatal disease with high heritability; however, the bone morphogenetic protein receptor 2 (BMPR2) gene only accounts for 17% of IPAH. The genetic basis of IPAH needs further investigation. Objective:To identify novel IPAH susceptibility genes other than BMPR2. Design, Setting, and Participants:This 2-stage, case-control ... Read more >>

JAMA Cardiol (JAMA cardiology)
[2020, :]

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The endothelin system as target for therapeutic interventions in cardiovascular and renal disease.

Eray Eroglu, Ismail Kocyigit, Bengt Lindholm,

Endothelins including its most abundant isoform, endothelin-1 (ET-1), are peptides acting as vasoconstrictors when binding to ETA and ETB receptors, and, in addition to their distinct roles in normal physiology, endothelins have a central role in the pathophysiology of many diseases including cardiovascular and renal diseases. Endothelin-1 (ET-1), the most ... Read more >>

Clin. Chim. Acta (Clinica chimica acta; international journal of clinical chemistry)
[2020, 506:92-106]

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Connexin-43 is a promising target for pulmonary hypertension due to hypoxaemic lung disease.

Claire Bouvard, Nafiisha Genet, Carole Phan, Baptiste Rode, Raphaël Thuillet, Ly Tu, Paul Robillard, Marilyne Campagnac, Raffaella Soleti, Eric Dumas De La Roque, Frédéric Delcambre, Laurent Cronier, Thibaud Parpaite, Elise Maurat, Patrick Berger, Jean-Pierre Savineau, Roger Marthan, Christophe Guignabert, Véronique Freund-Michel, Christelle Guibert,

The mechanisms underlying pulmonary hypertension (PH) are complex and multifactorial, and involve different cell types that are interconnected through gap junctional channels. Although connexin (Cx)-43 is the most abundant gap junction protein in the heart and lungs, and critically governs intercellular signalling communication, its contribution to PH remains unknown. The ... Read more >>

Eur. Respir. J. (The European respiratory journal)
[2020, 55(3):]

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Mild parenchymal lung disease and/or low diffusion capacity impacts survival and treatment response in patients diagnosed with idiopathic pulmonary arterial hypertension.

Robert A Lewis, A A Roger Thompson, Catherine G Billings, Athanasios Charalampopoulos, Charlie A Elliot, Neil Hamilton, Catherine Hill, Judith Hurdman, Smitha Rajaram, Ian Sabroe, Andy J Swift, David G Kiely, Robin Condliffe,

There are limited published data defining survival and treatment response in patients with mild lung disease and/or reduced gas transfer who fulfil diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH).Patients diagnosed with IPAH between 2001-19 were identified in the ASPIRE registry. Using pre-specified criteria based on CT imaging and spirometry, ... Read more >>

Eur. Respir. J. (The European respiratory journal)
[2020, :]

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Lipids and Ketones Dominate Metabolism at the Expense of Glucose Control in Pulmonary Arterial Hypertension: A Hyperglycemic Clamp and Metabolomics Study.

Jacob T Mey, Adithya Hari, Christopher L Axelrod, Ciarán E Fealy, Melissa L Erickson, John P Kirwan, Raed A Dweik, Gustavo A Heresi,

BACKGROUND: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 ... Read more >>

Eur. Respir. J. (The European respiratory journal)
[2020, :]

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Idiopathic Pulmonary Fibrosis (IPF) and pulmonary hypertension: Heracles meets the Hydra.

Keshava Rajagopal, Andrew J Bryant, Sandeep Sahay, Nancy Wareing, Yang Zhou, Lavannya M Pandit, Harry Karmouty-Quintana,

Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease where the additional presence of pulmonary hypertension (PH) reduces survival. In particular, the presence of coexistent pulmonary vascular disease in patients with advanced lung parenchymal disease results in worse outcomes than either diagnosis alone. This is true with respect to the ... Read more >>

Br. J. Pharmacol. (British journal of pharmacology)
[2020, :]

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Basement Membrane Remodelling Controls Endothelial Function in IPAH.

Katharina Jandl, Leigh M Marsh, Julia Hoffmann, Ayse Ceren Mutgan, Oliver Baum, Wilhelm Bloch, Helene Thekkekara-Puthenparampil, Dagmar Kolb, Katharina Sinn, Walter Klepetko, Akos Heinemann, Andrea Olschewski, Horst Olschewski, Grazyna Kwapiszewska,

The extracellular matrix (ECM) increasingly emerges as an active driver in several diseases, including idiopathic pulmonary arterial hypertension (IPAH). The basement membrane (BM) is a specialized class of ECM proteins. In pulmonary arteries, the BM is in close contact and direct proximity to vascular cells including endothelial cells. So far, ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, :]

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Pediatric heart-lung transplantation: A contemporary analysis of outcomes.

Kyle W Riggs, Jacquelyn L Chapman, Marc Schecter, Christopher Towe, Farhan Zafar, David L S Morales,

BACKGROUND:Pediatric heart-lung transplantation (HLT) is rare, and no report has analyzed patient outcomes since time of listing. We analyzed pediatric HLTs to understand risk factors for waitlist and post-HLT mortality. METHODS:All pediatric (<18 year old) HLT candidates were identified within the UNOS database (n = 573) and grouped by age, era, and by ... Read more >>

Pediatr Transplant (Pediatric transplantation)
[2020, :e13682]

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David's procedure for pulmonary artery aneurysm.

Renda Circi, Emre Boysan, Bulent Behlul Altunkeser, Nazif Aygul, Kumral Cagli, Kerim Cagli, Erol Sener,

Pulmonary artery aneurysm (PAA) is a rare entity with fatal complications. Its silent course contributes to large aneurysms with compression symptoms. We present a 39-year-old female idiopathic pulmonary arterial hypertension patient with a giant PAA causing severe pulmonary regurgitation (PR) and symptomatic left main coronary artery compression (LMCA). Since she ... Read more >>

J Card Surg (Journal of cardiac surgery)
[2020, :]

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Myocardial perfusion abnormalities in Eisenmenger syndrome.

Anunay Gupta, Chetan Patel, Saurabh Kumar Gupta, S Ramakrishnan, Anita Saxena, Shyam S Kothari,

BACKGROUND:Compared to primary pulmonary hypertension (PPH), the right ventricular (RV) contractile function is preserved for a long time in patients with Eisenmenger syndrome and is likely the most important determinant of relatively higher survival. The differences in myocardial perfusion have been purported to explain this discrepancy. The exact prevalence of ... Read more >>

Nucl Med Commun (Nuclear medicine communications)
[2020, 41(3):206-211]

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The Clinical Efficacy of Pulmonary Hypertension-Specific Agents in Idiopathic Pulmonary Fibrosis: Systematic Review and Meta-Analysis of Randomized Controlled Clinical Trials.

Jonghoo Lee, Jae Uk Song,

BACKGROUND:Pulmonary hypertension (PH) is common in patients with idiopathic pulmonary fibrosis (IPF) and is associated with poor outcomes. This study was performed to determine the clinical efficacy of PH-specific therapeutic agents for IPF patients. METHODS:We performed a systematic review and meta-analysis using MEDLINE, EMBASE, and the Cochrane Central Register. We ... Read more >>

J. Korean Med. Sci. (Journal of Korean medical science)
[2020, 35(8):e48]

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Ability of the COPD Assessment Test to evaluate the lung-specific quality of life in systemic sclerosis-associated interstitial lung disease.

Naoki Mugii, Fujiko Someya,

INTRODUCTION:The COPD Assessment Test (CAT) is utilised to evaluate the treatment outcome regarding the health status in idiopathic interstitial pneumonia (IIP). However, the ability of the CAT to evaluate the lung-specific quality of life in systemic sclerosis-associated interstitial lung disease (SSc-ILD) is not established. Therefore, we investigated whether CAT scores ... Read more >>

Clin Respir J (The clinical respiratory journal)
[2020, :]

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Electrical Stimulation-Guided Approach to Pulmonary Artery Catheter Ablation in Patients with Idiopathic Pulmonary Arterial Hypertension: A Pilot Feasibility Study with a 12-Month Follow-Up.

Natalia S Goncharova, Olga M Moiseeva, Heber Ivan Condori Leandro, Irina S Zlobina, Aelita V Berezina, Kirill N Malikov, Dmitry M Tashkhanov, Dmitry S Lebedev, Evgeny N Mikhaylov,

Background:Recently, transcatheter pulmonary artery (PA) ablation aiming at sympathetic denervation has been proposed in pulmonary arterial hypertension (PAH). This pilot feasibility study aimed to assess the feasibility of selective radiofrequency PA ablation based on response to high-frequency stimulation mapping. Methods:The study comprised 3 female patients with idiopathic PAH (IPAH). The ... Read more >>

Biomed Res Int (BioMed Research International)
[2020, 2020:8919515]

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Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody.

Yusho Ishii, Hiroshi Fujii, Koichiro Sugimura, Tsuyoshi Shirai, Yosuke Hoshi, Yoko Fujita, Yuko Shirota, Tomonori Ishii, Hiroaki Shimokawa, Hideo Harigae,

Systemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications. Among them, anticentriole antibody is recently reported to be highly associated with SSc-associated pulmonary arterial hypertension (SSc-PAH). In ... Read more >>

Case Rep Rheumatol (Case reports in rheumatology)
[2020, 2020:1926908]

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PPARγ is a gatekeeper for extracellular matrix and vascular cell homeostasis: beneficial role in pulmonary hypertension and renal/cardiac/pulmonary fibrosis.

Gábor Kökény, Laurent Calvier, Ekaterina Legchenko, Philippe Chouvarine, Miklós M Mózes, Georg Hansmann,

PURPOSE OF REVIEW:Pulmonary arterial hypertension (PAH) is characterized by pulmonary arterial endothelial cell (PAEC) dysfunction and apoptosis, pulmonary arterial smooth muscle cell (PASMC) proliferation, inflammation, vasoconstriction, and metabolic disturbances that include disrupted bone morphogenetic protein receptor (BMPR2)-peroxisome proliferator-activated receptor gamma (PPARγ) axis and DNA damage. Activation of PPARγ improves many ... Read more >>

Curr. Opin. Nephrol. Hypertens. (Current opinion in nephrology and hypertension)
[2020, 29(2):171-179]

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Impaired Right and Left Ventricular Longitudinal Function in Patients with Fibrotic Interstitial Lung Diseases.

Agostino Buonauro, Ciro Santoro, Maurizio Galderisi, Angelo Canora, Regina Sorrentino, Roberta Esposito, Maria Lembo, Mario Enrico Canonico, Federica Ilardi, Valeria Fazio, Bruno Golia, Alessandro Sanduzzi, Marialuisa Bocchino,

Background: Left ventricular (LV) and right ventricular (RV) dysfunction is recognized in idiopathic pulmonary fibrosis (IPF). Little is known about cardiac involvement in non-idiopathic pulmonary fibrosis (no-IPF). This issue can be explored by advanced echocardiography. Methods: Thirty-three clinically stable and therapy-naive fibrotic IPF and 28 no-IPF patients, and 30 healthy ... Read more >>

J Clin Med (Journal of clinical medicine)
[2020, 9(2):]

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Evaluation and management of pulmonary hypertension in the emergency department setting.

Erica Simon, Rachel E Bridwell, Tim Montrief, Alex Koyfman, Brit Long,

BACKGROUND:Pulmonary hypertension (PH) is characterized by increased pulmonary vascular resistance and pulmonary arterial pressure and is associated with significant morbidity and mortality. OBJECTIVE:This narrative review evaluates PH, outlines the complex pathophysiologic derangements, and addresses the emergency department (ED) management of this patient population. DISCUSSION:Approximately 10-20% of individuals in the United ... Read more >>

Am J Emerg Med (The American journal of emergency medicine)
[2020, :]

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Pre-Anesthesia Extracorporeal Membrane Oxygenation in Two Lung Transplant Recipients with Severe Pulmonary Hypertension.

Liu Minqiang, Gao Hong, Chen Jingyu, Wang Yanjuan, Xu Bo, Wang Guilong, Wu Qiang, Hu Chunxiao,

Extracorporeal membrane oxygenation (ECMO) is a widely used cardiopulmonary support method that is usually implemented after anesthesia during the period of lung transplantation (LTx). In severe pulmonary arterial hypertension (PAH) patients, however, anesthesia induction is a high-risk phase and can result in severe cardiorespiratory failure. Herein, we describe two severe ... Read more >>

Case Rep Med (Case Reports in Medicine)
[2020, 2020:7265429]

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Clinical implications of idiopathic pulmonary arterial hypertension phenotypes defined by cluster analysis.

Roberto Badagliacca, Franz Rischard, Silvia Papa, Saad Kubba, Rebecca Vanderpool, Jason X-J Yuan, Joe G N Garcia, Sophia Airhart, Roberto Poscia, Beatrice Pezzuto, Giovanna Manzi, Cristiano Miotti, Federico Luongo, Gianmarco Scoccia, Susanna Sciomer, Roberto Torre, Francesco Fedele, Carmine Dario Vizza,

BACKGROUND:>Despite advances in drug development, life expectancy in idiopathic pulmonary arterial hypertension (IPAH) remains unacceptable. Contemporary IPAH characterization is based on criteria that may not adequately capture disease heterogeneity and may be proposed as a possible explanation for why patient outcome is still unfavorable. The aim of this study was ... Read more >>

J. Heart Lung Transplant. (The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation)
[2020, 39(4):310-320]

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Prognostic impact of right ventricular mass change in patients with idiopathic pulmonary arterial hypertension.

Julia Grapsa, Timothy C Tan, Maria Carmo Pereira Nunes, Declan P O'Regan, Giuliana Durighel, Luke S G E Howard, J Simon R Gibbs, Petros Nihoyannopoulos,

BACKGROUND:Compensatory remodelling i.e. increased right ventricular (RV) mass frequently occurs as an adaptive response to the chronic pressure overload to maintain contractile function. The prognostic value of the serial change in RV mass is unclear. AIM:The aim of our study was to examine the longitudinal changes in RV mass and ... Read more >>

Int. J. Cardiol. (International journal of cardiology)
[2020, 304:172-174]

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Pulmonary veno-occlusive disease: a probably underdiagnosed cause of pulmonary hypertension in systemic sclerosis.

Ana Catarina Duarte, Ana Cordeiro, Maria José Loureiro, Filipa Ferreira,

Pulmonary hypertension is a serious complication of systemic sclerosis and remains one of the leading causes of mortality. Pulmonary veno-occlusive disease (PVOD), recently reclassified as pulmonary arterial hypertension (PAH) with overt features of venous/capillaries involvement, is a subgroup of group 1 pulmonary hypertension, which has been rarely reported in systemic ... Read more >>

Clin. Rheumatol. (Clinical rheumatology)
[2020, :]

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Atrial flow regulator as a bridge to lung transplantation in a young patient with drug-resistant idiopathic pulmonary arterial hypertension.

Magdalena Janus, Sylwia Sławek-Szmyt, Aleksander Araszkiewicz, Tatiana Mularek-Kubzdela, Maciej Lesiak, Marek Grygier,

Kardiol Pol (Kardiologia polska)
[2020, :]

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