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https://www.readbyqxmd.com/read/28819685/cardiomyocyte-dimethylarginine-dimethylaminohydrolase-1-ddah1-plays-an-important-role-in-attenuating-ventricular-hypertrophy-and-dysfunction
#1
Xin Xu, Ping Zhang, Dongmin Kwak, John Fassett, Wenhui Yue, Dorothee Atzler, Xinli Hu, Xiaohong Liu, Huan Wang, Zhongbing Lu, Haipeng Guo, Edzard Schwedhelm, Rainer H Böger, Peijie Chen, Yingjie Chen
Asymmetric dimethylarginine (ADMA) is an endogenous inhibitor of nitric oxide synthases that limits nitric oxide bioavailability. Dimethylarginine dimethylaminohydrolase-1 (DDAH1) exerts a critical role for ADMA degradation and plays an important role in NO signaling. In the heart, DDAH1 is observed in endothelial cells and in the sarcolemma of cardiomyocytes. While NO signaling is important for cardiac adaptation to stress, DDAH1 impact on cardiomyocyte homeostasis is not clear. Here we used the MerCreMer-LoxP model to specifically disrupt cardiomyocyte DDAH1 expression in adult mice to determine the physiological impact of cardiomyocyte DDAH1 under basal conditions and during hypertrophic stress imposed by transverse aortic constriction (TAC)...
August 17, 2017: Basic Research in Cardiology
https://www.readbyqxmd.com/read/28818204/geometry-as-a-confounder-when-assessing-ventricular-systolic-function-comparison-between-ejection-fraction-and-strain
#2
Thomas M Stokke, Nina E Hasselberg, Marit K Smedsrud, Sebastian I Sarvari, Kristina H Haugaa, Otto A Smiseth, Thor Edvardsen, Espen W Remme
BACKGROUND: Preserved left ventricular (LV) ejection fraction (EF) and reduced myocardial strain are reported in patients with hypertrophic cardiomyopathy, ischemic heart disease, diabetes mellitus, and more. OBJECTIVES: The authors performed a combined mathematical and echocardiographic study to understand the inconsistencies between EF and strains. METHODS: An analytical equation showing the relationship between EF and the 4 parameters, global longitudinal strain (GLS), global circumferential strain (GCS), wall thickness, and short-axis diameter, was derived from an elliptical LV model...
August 22, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28816235/g3bp2-is-involved-in-isoproterenol-induced-cardiac-hypertrophy-through-activating-the-nf-%C3%AE%C2%BAb-signaling-pathway
#3
Hui-Qi Hong, Jing Lu, Xiu-Li Fang, Yu-Hong Zhang, Yi Cai, Jing Yuan, Pei-Qing Liu, Jian-Tao Ye
The RasGAP SH3 domain-binding proteins (G3BPs) are a family of RNA-binding proteins that can co-ordinate signal transduction and post-transcriptional gene regulation. G3BPs have been shown to be involved in mediating a great diversity of cellular processes such as cell survival, growth, proliferation and apoptosis. But the potential roles of G3BPs in the pathogenesis and progression of cardiovascular diseases remain to be clarified. In the present study, we provide the first evidence that suggests the participation of G3BP2 in cardiac hypertrophy...
August 17, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28808947/the-sydney-heart-bank-improving-translational-research-while-eliminating-or-reducing-the-use-of-animal-models-of-human-heart-disease
#4
REVIEW
C G Dos Remedios, S P Lal, A Li, J McNamara, A Keogh, P S Macdonald, R Cooke, E Ehler, R Knöll, S B Marston, J Stelzer, H Granzier, C Bezzina, S van Dijk, F De Man, G J M Stienen, J Odeberg, F Pontén, W Linke, J van der Velden
The Sydney Heart Bank (SHB) is one of the largest human heart tissue banks in existence. Its mission is to provide high-quality human heart tissue for research into the molecular basis of human heart failure by working collaboratively with experts in this field. We argue that, by comparing tissues from failing human hearts with age-matched non-failing healthy donor hearts, the results will be more relevant than research using animal models, particularly if their physiology is very different from humans. Tissue from heart surgery must generally be used soon after collection or it significantly deteriorates...
August 14, 2017: Biophysical Reviews
https://www.readbyqxmd.com/read/28803513/mechanisms-of-unexpected-death-and-autopsy-findings-in-friedreich-ataxia
#5
Roger W Byard, John D Gilbert
A 36-year-old woman with a clinical history of Friedreich ataxia and hypertrophic cardiomyopathy was found unexpectedly dead at her home. The heart showed asymmetric left ventricular hypertrophy, with an interventricular septal thickness of 20-25 mm (the remainder of the left ventricular wall measured 15 mm). Histologically, both ventricles had irregular areas of marked myocyte hypertrophy with associated interstitial fibrosis and focal myofibre disarray. There was neuronal loss within the dentate nucleus of the cerebellum, with vacuolation and axonal loss in the dorsal columns and spinocerebellar tracts of the upper cervical spinal cord...
January 1, 2017: Medicine, Science, and the Law
https://www.readbyqxmd.com/read/28802509/value-of-strain-imaging-and-maximal-oxygen-consumption-in-patients-with-hypertrophic-cardiomyopathy
#6
Kegan J Moneghetti, Davide Stolfo, Jeffrey W Christle, Yukari Kobayashi, Gherardo Finocchiaro, Gianfranco Sinagra, Jonathan Myers, Euan A Ashley, Francois Haddad, Matthew T Wheeler
Longitudinal strain (LS) has been shown to be predictive of outcome in hypertrophic cardiomyopathy (HC). Percent predicted peak oxygen uptake (ppVO2), among other cardiopulmonary exercise testing (CPX) metrics, is a strong predictor of prognosis in HC. However, there has been limited investigation into the combination of LS and CPX metrics. This study sought to determine how LS and parameters of exercise performance contribute to prognosis in HC. One hundred and thirty-one consecutive patients with HC who underwent CPX and stress echocardiography were included...
July 17, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28802159/increased-%C3%AE-catenin-accumulation-and-nuclear-translocation-are-associated-with-concentric-hypertrophy-in-cardiomyocytes
#7
Cheng-Yu Lee, Wei Wen Kuo, Rathinasamy Baskaran, Cecilia Hsuan Day, Pei Ying Pai, Chao Hung Lai, Yu-Feng Chen, Ray-Jade Chen, Viswanadha Vijaya Padma, Chih Yang Huang
Defective Wnt/β-Catenin signaling, activated under various pathological conditions, can result in cardiac and vascular abnormalities. In the present study, the possible role of β-catenin over expression during cardiac hypertrophy was investigated. Ten samples from hearts of human patients with acute infarction, and granulation tissue from 20 patients and 10 from normal ones were collected in order to investigate roles of β-catenin in cardiac hypertrophy. H9c2 cardiomyoblast cells and Wistar rat primary neonatal cardiomyocytes were overexpressed with β-catenin...
July 12, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28800400/mechanism-of-tfam-mediated-cardiomyocyte-protection
#8
George H Kunkel, Pankaj Chaturvedi, Nicholas Theilen, Rohit Nair, Suresh C Tyagi
Although mitochondrial transcription factor A (TFAM) is a protective component of mitochondrial DNA and a regulator of calcium and reactive oxygen species (ROS) production, the mechanism remains unclear. In heart failure (HF), TFAM is significantly decreased and cardiomyocyte instability ensues. TFAM inhibits Nuclear Factor of Activated T cells (NFAT), which reduces ROS production; additionally, TFAM transcriptionally activates SERCA2a to decrease free calcium. Therefore, decreasing TFAM vastly increases protease expression and hypertrophic factors, leading to cardiomyocyte functional decline...
August 11, 2017: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/28799247/the-valosin-containing-protein-is-a-novel-repressor-of-cardiomyocyte-hypertrophy-induced-by-pressure-overload
#9
Ning Zhou, Ben Ma, Shaunrick Stoll, Tristan T Hays, Hongyu Qiu
Hypertension-induced left ventricular hypertrophy (LVH) is an independent risk factor for heart failure. Regression of LVH has emerged as a major goal in the treatment of hypertensive patients. Here, we tested our hypothesis that the valosin-containing protein (VCP), an ATPase associate protein, is a novel repressor of cardiomyocyte hypertrophy under the pressure overload stress. Left ventricular hypertrophy (LVH) was determined by echocardiography in 4-month male spontaneously hypertensive rats (SHRs) vs. age-matched normotensive Wistar Kyoto (WKY) rats...
August 11, 2017: Aging Cell
https://www.readbyqxmd.com/read/28793143/intraoperative-diagnosis-of-anderson-fabry-disease-in-patients-with-obstructive-hypertrophic-cardiomyopathy-undergoing-surgical-myectomy
#10
Franco Cecchi, Maria Iascone, Niccolò Maurizi, Laura Pezzoli, Irene Binaco, Elena Biagini, Maria Laura Fibbi, Iacopo Olivotto, Federico Pieruzzi, Ana Fruntelata, Lucian Dorobantu, Claudio Rapezzi, Paolo Ferrazzi
Importance: Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective: To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations...
August 9, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28790436/three-tf-co-expression-modules-regulate-pressure-overload-cardiac-hypertrophy-in-male-mice
#11
Yao-Ming Chang, Li Ling, Ya-Ting Chang, Yu-Wang Chang, Wen-Hsiung Li, Arthur Chun-Chieh Shih, Chien-Chang Chen
Pathological cardiac hypertrophy, a dynamic remodeling process, is a major risk factor for heart failure. Although a number of key regulators and related genes have been identified, how the transcription factors (TFs) dynamically regulate the associated genes and control the morphological and electrophysiological changes during the hypertrophic process are still largely unknown. In this study, we obtained the time-course transcriptomes at five time points in four weeks from male murine hearts subjected to transverse aorta banding surgery...
August 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28777121/raf1-variants-causing-biventricular-hypertrophic-cardiomyopathy-in-two-preterm-infants-further-phenotypic-delineation-and-review-of-literature
#12
Danielle Thompson, Jessica Patrick-Esteve, Jeffrey W Surcouf, Dana Rivera, Bianca Castellanos, Pooja Desai, Christian Lilje, Yves Lacassie, Michael Marble, Regina Zambrano
Noonan syndrome (NS) is an autosomal dominant disorder characterized by distinctive facial features, short neck, short stature, congenital heart defects, pectus deformities, and variable developmental delays. NS is genetically heterogeneous as pathogenic variants in several genes involved in the Ras/mitogen-activated protein kinase pathway have been associated with a NS phenotype. Overall, 50% of patients harbor pathogenic variants in PTPN11, whereas 3-17% of patients have variants in RAF1. We present two premature neonates with progressive biventricular hypertrophy found to have RAF1 variants in the CR2 domain...
August 3, 2017: Clinical Dysmorphology
https://www.readbyqxmd.com/read/28772045/translational-registry-for-cardiomyopathies-torch-rationale-and-first-results
#13
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28771943/overexpression-of-integrin-%C3%AE-11-induces-cardiac-fibrosis-in-mice
#14
Andreas Romaine, Ida W Sørensen, Cédric Zeltz, Ning Lu, Pugazendhi Murugan Erusappan, Arne Olav Melleby, Lili Zhang, Bård Bendiksen, Emma Louise Robinson, Jan Magnus Aronsen, Kate M Herum, Håvard Danielsen, Ivar Sjaastad, Geir Christensen, Donald Gullberg
AIM: To understand the role of the collagen-binding integrin α11 in vivo we have used a classical approach of creating a mouse strain overexpressing integrin α11. A transgenic mouse strain overexpressing α11 in muscle tissues was analysed in the current study with special reference to the heart tissue. METHODS: We generated and phenotyped integrin α11 transgenic (TG) mice by echocardiography, magnetic resonance imaging and histology. Wild-type (WT) mice were subjected to aortic banding (AB) and the expression of integrin α11 was measured in flow cytometry sorted cardiomyocytes and non-myocytes...
August 3, 2017: Acta Physiologica
https://www.readbyqxmd.com/read/28771545/augmented-sphingosine-1-phosphate-receptor-1-signaling-in-cardiac-fibroblasts-induces-cardiac-hypertrophy-and-fibrosis-through-angiotensin-ii-and-interleukin-6
#15
Sei-Ichiro Ohkura, Soichiro Usui, Shin-Ichiro Takashima, Noriko Takuwa, Kazuaki Yoshioka, Yasuo Okamoto, Yutaka Inagaki, Naotoshi Sugimoto, Teppei Kitano, Masayuki Takamura, Takashi Wada, Shuichi Kaneko, Yoh Takuwa
Cardiac fibroblasts, together with cardiomyocytes, occupy the majority of cells in the myocardium and are involved in myocardial remodeling. The lysophospholipid mediator sphigosine-1-phosphate (S1P) regulates functions of cardiovascular cells through multiple receptors including S1PR1-S1PR3. S1PR1 but not other S1P receptors was upregulated in angiotensin II-induced hypertrophic hearts. Therefore, we investigated a role of S1PR1 in fibroblasts for cardiac remodeling by employing transgenic mice that overexpressed S1PR1 under the control of α-smooth muscle actin promoter...
2017: PloS One
https://www.readbyqxmd.com/read/28771489/additional-value-of-screening-for-minor-genes-and-copy-number-variants-in-hypertrophic-cardiomyopathy
#16
Irene Mademont-Soler, Jesus Mates, Raquel Yotti, Maria Angeles Espinosa, Alexandra Pérez-Serra, Ana Isabel Fernandez-Avila, Monica Coll, Irene Méndez, Anna Iglesias, Bernat Del Olmo, Helena Riuró, Sofía Cuenca, Catarina Allegue, Oscar Campuzano, Ferran Picó, Carles Ferrer-Costa, Patricia Álvarez, Sergio Castillo, Pablo Garcia-Pavia, Esther Gonzalez-Lopez, Laura Padron-Barthe, Aranzazu Díaz de Bustamante, María Teresa Darnaude, José Ignacio González-Hevia, Josep Brugada, Francisco Fernandez-Aviles, Ramon Brugada
INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is the most prevalent inherited heart disease. Next-generation sequencing (NGS) is the preferred genetic test, but the diagnostic value of screening for minor and candidate genes, and the role of copy number variants (CNVs) deserves further evaluation. METHODS: Three hundred and eighty-seven consecutive unrelated patients with HCM were screened for genetic variants in the 5 most frequent genes (MYBPC3, MYH7, TNNT2, TNNI3 and TPM1) using Sanger sequencing (N = 84) or NGS (N = 303)...
2017: PloS One
https://www.readbyqxmd.com/read/28770830/resveratrol-alleviates-diabetic-cardiomyopathy-in-rats-by-improving-mitochondrial-function-through-pgc-1%C3%AE-deacetylation
#17
Wei-Jin Fang, Chun-Jiang Wang, Yang He, Yu-Lu Zhou, Xiang-Dong Peng, Shi-Kun Liu
Recent evidence shows that resveratrol (RSV) may ameliorate high-glucose-induced cardiac oxidative stress, mitochondrial dysfunction and myocardial fibrosis in diabetes. However, the mechanisms by which RSV regu¬lates mitochondrial function in diabetic cardiomyopathy have not been fully elucidated. Mitochondrial dysfunction contributes to cardiac dysfunction in diabetic patients, which is associated with dysregulation of peroxisome proliferator-activated receptor gamma coactivator-1α (PGC-1α). In this study we examined whether resveratrol alleviated cardiac dysfunction in diabetes by improving mitochondrial function via SIRT1-mediated PGC-1α deacetylation...
August 3, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28759639/g-protein-coupled-receptor-kinase-2-promotes-cardiac-hypertrophy
#18
Philipp Schlegel, Julia Reinkober, Eric Meinhardt, Henrike Tscheschner, Erhe Gao, Sarah M Schumacher, Ancai Yuan, Johannes Backs, Patrick Most, Thomas Wieland, Walter J Koch, Hugo A Katus, Philip W Raake
The increase in protein activity and upregulation of G-protein coupled receptor kinase 2 (GRK2) is a hallmark of cardiac stress and heart failure. Inhibition of GRK2 improved cardiac function and survival and diminished cardiac remodeling in various animal heart failure models. The aim of the present study was to investigate the effects of GRK2 on cardiac hypertrophy and dissect potential molecular mechanisms. In mice we observed increased GRK2 mRNA and protein levels following transverse aortic constriction (TAC)...
2017: PloS One
https://www.readbyqxmd.com/read/28752727/assessment-of-left-atrial-function-in-patients-with-hypertrophic-cardiomyopathy-using-two-dimensional-strain-a-comparison-with-volume-derived-values
#19
Jun Huang, Zi-Ning Yan, Yi-Fei Rui, Li Fan, Dan Shen, Dong-Liang Chen
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a common heart disease. Left atrial (LA) function plays an important role in the diastolic function in patients with HCM. In this study, two-dimensional speckle tracking imaging (2D-STI) was used to assess left atrial (LA) function in patients with HCM. METHODS: Thirty-four patients with HCM and thirty-four age- and gender-matched normal subjects were studied. The LA volume-derived index was measured using 2D ultrasonic images...
July 27, 2017: Minerva Cardioangiologica
https://www.readbyqxmd.com/read/28747036/long-term-outcome-of-nonobstructive-versus-obstructive-hypertrophic-cardiomyopathy-a-systematic-review-and-meta-analysis
#20
Francesco Pelliccia, Vincenzo Pasceri, Giuseppe Limongelli, Camillo Autore, Cristina Basso, Domenico Corrado, Massimo Imazio, Claudio Rapezzi, Gianfranco Sinagra, Giuseppe Mercuro
BACKGROUND: Prognosis of hypertrophic cardiomyopathy (HCM) is particularly heterogeneous. Patients with nonobstructive HCM (NOCM) are thought to be at relatively low-risk as compared with obstructive HCM (HOCM) with no need of major treatment options. However, available evidence of NOCM comes mainly from tertiary centers where a referral bias is likely to occur. Aim of this study was to perform a systematic review and meta-analysis of the published literature on hypertrophic cardiomyopathy (HCM) in order to outline differences in presenting features and long-term outcome between NOCM and HOCM...
September 15, 2017: International Journal of Cardiology
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