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Pulmonary artery hypertension

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https://www.readbyqxmd.com/read/28919734/usefulness-of-the-desaturation-distance-ratio-from-the-six-minute-walk-test-for-patients-with-copd
#1
Yukari Fujimoto, Yutaro Oki, Masahiro Kaneko, Hideki Sakai, Shogo Misu, Takumi Yamaguchi, Yuji Mitani, Hisafumi Yasuda, Akira Ishikawa
PURPOSE: A straightforward, noninvasive method is needed to assess emphysema and pulmonary hypertension (PH) in COPD patients. The desaturation-distance ratio (DDR) is an index derived from the distance traveled and level of desaturation during a six-minute walk test (6MWT); it has previously been shown to be associated with percentage of forced expiratory volume in the first second of expiration (%FEV1.0) and percentage of diffusion capacity of the lung for carbon monoxide (%DLCO). The aim of this study was to examine the associations between DDR and emphysema and PH...
2017: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/28919201/macitentan-for-the-treatment-of-inoperable-chronic-thromboembolic-pulmonary-hypertension-merit-1-results-from-the-multicentre-phase-2-randomised-double-blind-placebo-controlled-study
#2
Hossein-Ardeschir Ghofrani, Gérald Simonneau, Andrea M D'Armini, Peter Fedullo, Luke S Howard, Xavier Jaïs, David P Jenkins, Zhi-Cheng Jing, Michael M Madani, Nicolas Martin, Eckhard Mayer, Kelly Papadakis, Dominik Richard, Nick H Kim
BACKGROUND: Macitentan is beneficial for long-term treatment of pulmonary arterial hypertension. The microvasculopathy of chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension are similar. METHODS: The phase 2, double-blind, randomised, placebo-controlled MERIT-1 trial assessed macitentan in 80 patients with CTEPH adjudicated as inoperable. Patients identified as WHO functional class II-IV with a pulmonary vascular resistance (PVR) of at least 400 dyn·s/cm(5) and a walk distance of 150-450 m in 6 min were randomly assigned (1:1), via an interactive voice/web response system, to receive oral macitentan (10 mg once a day) or placebo...
September 8, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28915063/twist1-in-hypoxia-induced-pulmonary-hypertension-through-tgf%C3%AE-smad-signaling
#3
Tadanori Mammoto, Megan Muyleart, G Ganesh Konduri, Akiko Mammoto
Pulmonary hypertension (PH) is a devastating pulmonary vascular disease characterized by aberrant muscularization of the normally non-muscularized distal pulmonary arterioles. The expression of the transcription factor, Twist1, increases in the lungs of pulmonary arterial hypertension patients. However, the mechanisms by which Twist1 controls the pathogenesis of PH remain unclear. It is becoming clear that endothelial-to-mesenchymal transition (EndMT) contributes to various vascular pathologies including PH; Twist1 is known to mediate EndMT...
September 15, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28915060/long-non-coding-rna-lnrpt-is-regulated-by-pdgf-bb-and-modulates-proliferation-of-pulmonary-artery-smooth-muscle-cells
#4
Jidong Chen, Jiao Guo, Xiaolei Cui, Yan Dai, Zhixiong Tang, Junle Qu, J Usha Raj, Qinghua Hu, Deming Gou
Pulmonary artery hypertension (PAH) is a rare and fatal disorder with extensive remodeling of pulmonary arteries mediated by hyperproliferation of pulmonary artery smooth muscle cell (PASMC). Aberrant platelet-derived growth factor (PDGF) activity can lead to hyperproliferation of PASMC, however, little is known about the role of long noncoding RNA (lncRNA) in this process. Using RNA sequencing (RNA-seq), we identified 725 lncRNAs in rat PASMC (RPASMC), 95 of which were expressed differentially in response to PDGF-BB treatment...
September 15, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28914858/-bosentan-use-in-pulmonary-arterial-hypertension-russian-and-foreign-experience
#5
Z S Valieva, T V Martynyuk, I E Chazova
The results of evaluating the efficacy and safety of bosentan in patients with pulmonary arterial hypertension (PAH), as shown by the data of foreign randomized controlled trials and the authors' own experience, convincingly demonstrate that the introduction of the drug into clinical practice has led to a significant improvement of the possibilities of drug therapy in patients with this serious illness. Bosentan substantially improves physical activity in patients, reduces the severity of clinical symptoms, slows down the rates of disease progression, and prolongs survival in patients with different forms of PAH...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28914582/express-a-case-of-worsening-pulmonary-arterial-hypertension-and-pleural-effusions-by-bosutinib-after-prior-treatment-with-dasatinib
#6
Karan Seegobin, Amit Babbar, Jason Ferreira, Brittany Lyons, James Cury, Vandana Seeram
No abstract text is available yet for this article.
January 1, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28914424/new-perspectives-in-nanotherapeutics-for-chronic-respiratory-diseases
#7
Adriana Lopes da Silva, Fernanda Ferreira Cruz, Patricia Rieken Macedo Roccco, Marcelo Marcos Morales
According to the World Health Organization (WHO), hundreds of millions of people of all ages and in all countries suffer from chronic respiratory diseases, with particular negative consequences such as poor health-related quality of life, impaired work productivity, and limitations in the activities of daily living. Chronic obstructive pulmonary disease, asthma, occupational lung diseases (such as silicosis), cystic fibrosis, and pulmonary arterial hypertension are the most common of these diseases, and none of them are curable with current therapies...
September 15, 2017: Biophysical Reviews
https://www.readbyqxmd.com/read/28912263/hemodynamic-phenotyping-of-pulmonary-hypertension-in-left-heart-failure
#8
REVIEW
Robert Naeije, Mario Gerges, Jean-Luc Vachiery, Sergio Caravita, Christian Gerges, Irene M Lang
Increased pulmonary venous pressure secondary to left heart disease is the most common cause of pulmonary hypertension (PH). The diagnosis of PH due to left heart disease relies on a clinical probability assessment followed by the invasive measurements of a mean pulmonary artery pressure (PAP) ≥25 mm Hg and mean wedged PAP (PAWP) >15 mm Hg. A combination of mean PAP and mean PAWP defines postcapillary PH. Postcapillary PH is generally associated with a diastolic pulmonary pressure gradient (diastolic PAP minus mean PAWP) <7 mm Hg, a transpulmonary pressure gradient (mean PAP minus mean PAWP) <12 mm Hg, and pulmonary vascular resistance ≤3 Wood units (WU)...
September 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28912262/diastolic-pressure-difference-to-classify-pulmonary-hypertension-in-the-assessment-of-heart-transplant-candidates
#9
Stephen P Wright, Yasbanoo Moayedi, Farid Foroutan, Suhail Agarwal, Geraldine Paradero, Ana C Alba, Jay Baumwol, Susanna Mak
BACKGROUND: The diastolic pressure difference (DPD) is recommended to differentiate between isolated postcapillary and combined pre-/postcapillary pulmonary hypertension (Cpc-PH) in left heart disease (PH-LHD). However, in usual practice, negative DPD values are commonly calculated, potentially related to the use of mean pulmonary artery wedge pressure (PAWP). We used the ECG to gate late-diastolic PAWP measurements. We examined the method's impact on calculated DPD, PH-LHD subclassification, hemodynamic profiles, and mortality...
September 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28912026/influence-of-various-therapeutic-strategies-on-right-ventricular-morphology-function-and-hemodynamics-in-pulmonary-arterial-hypertension
#10
Roberto Badagliacca, Amresh Raina, Stefano Ghio, Michele D'Alto, Marco Confalonieri, Michele Correale, Marco Corda, Giuseppe Paciocco, Carlo Lombardi, Massimiliano Mulè, Roberto Poscia, Laura Scelsi, Paola Argiento, Susanna Sciomer, Raymond L Benza, Carmine Dario Vizza
BACKGROUND: In idiopathic pulmonary arterial hypertension (IPAH) treatment goals include improving right ventricular (RV) function, hemodynamics and symptoms to move patients to a low-risk category for adverse clinical outcomes. No data are available on the effect of upfront combination therapy on RV improvement as compared with monotherapy. The aim of this study was to evaluate echocardiographic RV morphology and function in patients affected by IPAH and treated with different strategies...
August 26, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28911936/exercise-training-in-patients-with-pulmonary-and-systemic-hypertension-a-unique-therapy-for-two-different-diseases
#11
REVIEW
Massimo Leggio, Augusto Fusco, Giorgio Limongelli, Luca Sgorbini
Pulmonary hypertension is a potentially life-threatening condition. Given its evolving definition, the incidence and prevalence of the disease is difficult to define, but registries suggest an increased global awareness. The management of patients with pulmonary arterial hypertension is highly specialised and requires multi-disciplinary input from a range of healthcare professionals, including cardiologists, respiratory physicians, rheumatologists, rehabilitation physicians and cardio-pulmonary physiotherapists...
September 11, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28911898/challenge-of-pregnancy-in-patients-with-pre-capillary-pulmonary-hypertension-veno-arterial-extracorporeal-membrane-oxygenation-as-an-innovative-support-for-delivery
#12
Patrizio Vitulo, Marta Beretta, Gennaro Martucci, Cesar Mario Hernandez Baravoglia, Giuseppe Romano, Alessandro Bertani, Lavinia Martino, Adriana Callari, Giovanna Panarello, Michele Pilato, Antonio Arcadipane
No abstract text is available yet for this article.
April 13, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28910144/ask1-inhibition-halts-disease-progression-in-preclinical-models-of-pulmonary-arterial-hypertension
#13
Grant R Budas, Mario Boehm, Baktybek Kojonazarov, Gayathri Viswanathan, Xia Tian, Swathi Veeroju, Tatyana Novoyatleva, Friedrich Grimminger, Ford Hinojosa-Kirschenbaum, Hossein A Ghofrani, Norbert Weissmann, Werner Seeger, John T Liles, Ralph T Schermuly
RATIONALE Progression of pulmonary arterial hypertension (PAH) is associated with pathologic remodeling of the pulmonary vasculature and the right ventricle (RV). Oxidative stress drives the remodeling process through activation of mitogenactivated protein kinases (MAPKs) which stimulate apoptosis, inflammation and fibrosis. OBJECTIVES We investigated whether pharmacological inhibition of the redoxsensitive apical MAPK Apoptosis Signal-Regulating Kinase 1 (ASK1) can halt the progression of pulmonary vascular and RV remodeling...
September 14, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28906388/retrospective-cause-analysis-of-troponin-i-elevation-in-non-cad-patients-special-emphasis-on-sepsis
#14
Chien-Wen Yang, Huijun Li, Lisa Thomas, Manuel Ramos, Po-Hong Liu, Thomas Roe, Ravinder Valadri, Michael C Kiel, Vincent Yi-Fong Su, Qi Shi
BACKGROUND: Troponin I is one of the most commonly tested biochemical markers in the emergency room (ER) and in the hospital setting. Besides coronary artery disease (CAD), demand ischemia with underlying tachycardia, anemia, hypertensive emergency, congestive heart failure, kidney disease, sepsis, and pulmonary embolism have also been reported to cause troponin I elevations. Few reports have excluded patients with CAD, and no study has summarized the proportion of these factors relative to an increased troponin I level...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28905517/the-prognostic-significance-of-tricuspid-valve-regurgitation-in-pulmonary-arterial-hypertension
#15
Libo Chen, Carolyn M Larsen, Rachel J Le, Heidi M Connolly, Sorin V Pislaru, Joseph G Murphy, Michael D McGoon, Robert P Frantz, Garvan C Kane
INTRODUCTION: Tricuspid valve regurgitation (TR) is a frequent finding in patients with pulmonary arterial hypertension (PAH). However, its prognostic significance and relation to PAH, while suspected, are poorly understood. We assessed 727 consecutive patients with newly diagnosed PAH who underwent transthoracic echocardiographic evaluation of tricuspid valve function. OBJECTIVES: The study objective was to determine the association of TR presence and severity with patient characteristics, pulmonary artery hemodynamics, and outcome...
September 14, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28904690/-mixed-connective-tissue-disease-prevalence-and-clinical-characteristics-in-african-black-study-of-7-cases-in-gabon-and-review-of-the-literature
#16
Landry Missounga, Josaphat Iba Ba, Ingrid Rosalie Nseng Nseng Ondo, Maria Ines Carine Nziengui Madjinou, Doris Malekou, Emeline Gracia Mouendou Mouloungui, Emmanuel Ecke Nzengue, Jean Bruno Boguikouma, Moussavou Kombila
The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28904266/clinical-impact-of-main-pulmonary-artery-dilatation-on-outcome-in-pediatric-idiopathic-and-heritable-pulmonary-arterial-hypertension
#17
Satoshi Ikehara, Shinichi Takatsuki, Tomotaka Nakayama, Kazuyuki Naoi, Hiroyuki Matsuura, Tsutomu Saji
BACKGROUND: Few studies have investigated the clinical impact of pulmonary artery (PA) dilatation on outcomes in pediatric pulmonary arterial hypertension (PAH).Methods and Results:This study investigated the clinical outcomes of idiopathic or heritable PAH in 66 children aged <18 years at diagnosis. Main PA/thorax (MPA/T) ratio was measured on chest radiography in PAH patients. Patients were divided into 2 groups based on MPA/T ratio, and compared with a control group of 166 age- and gender-matched healthy children...
September 13, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28904255/predictors-of-favorable-responses-to-immunosuppressive-treatment-in-pulmonary-arterial-hypertension-associated-with-connective-tissue-disease
#18
Hidekata Yasuoka, Yuichiro Shirai, Yuichi Tamura, Tsutomu Takeuchi, Masataka Kuwana
BACKGROUND: The potential efficacy of immunosuppressive (IS) treatment has been reported in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD), but its positioning in the treatment algorithm remains uncertain. The aim of this study was to identify predictors of favorable responses to first-line IS treatment.Methods and Results:This single-center retrospective study included 30 patients with PAH accompanied by systemic lupus erythematosus (SLE), mixed CTD (MCTD), or primary Sjögren's syndrome (SS) who received first-line IS treatment alone or in combination with pulmonary vasodilators...
September 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28904206/a-disease-associated-frameshift-mutation-in-caveolin-1-disrupts-caveolae-formation-and-function-through-introduction-of-a-de-novo-er-retention-signal
#19
Courtney A Copeland, Bing Han, Ajit Tiwari, Eric D Austin, James E Loyd, James D West, Anne K Kenworthy
Caveolin-1 (CAV1) is an essential component of caveolae and is implicated in numerous physiological processes. Recent studies have identified heterozygous mutations in the CAV1 gene in patients with pulmonary arterial hypertension (PAH), but the mechanisms by which these mutations impact caveolae assembly and contribute to disease remain unclear. To address this question, we examined the consequences of a familial PAH-associated frameshift mutation in CAV1, P158PfsX22, on caveolae assembly and function. We show that C-terminus of the CAV1 P158 protein contains a functional ER-retention signal that inhibits ER exit and caveolae formation and accelerates CAV1 turnover in Cav1(-/-) MEFs...
September 13, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28904006/the-crash-report-emergency-management-dilemmas-facing-acute-physicians-in-patients-with-pulmonary-arterial-hypertension
#20
Laura C Price, Konstantinos Dimopoulos, Philip Marino, Rafael Alonso-Gonzalez, Colm McCabe, Aleksander Kemnpy, Lorna Swan, Maria Boutsikou, Ahmed Al Zahrani, Gerry J Coghlan, Benjamin E Schreiber, Luke S Howard, Rachel Davies, Mark Toshner, Joanna Pepke-Zaba, Alistair C Church, Andrew Peacock, Paul A Corris, James L Lordan, Sean Gaine, Robin Condliffe, David G Kiely, Stephen John Wort
Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies. A committee of physicians from nationally designated PH centres identified the 'most common' emergency clinical scenarios encountered in patients with PAH...
September 12, 2017: Thorax
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