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https://www.readbyqxmd.com/read/29344294/cardiomyocyte-restricted-low-density-lipoprotein-receptor-related-protein-6-lrp6-deletion-leads-to-lethal-dilated-cardiomyopathy-partly-through-drp1-signaling
#1
Zhidan Chen, Yang Li, Ying Wang, Juying Qian, Hong Ma, Xiang Wang, Guoliang Jiang, Ming Liu, Yanpeng An, Leilei Ma, Le Kang, Jianguo Jia, Chunjie Yang, Guoping Zhang, Ying Chen, Wei Gao, Mingqiang Fu, Zheyong Huang, Huiru Tang, Yichun Zhu, Junbo Ge, Hui Gong, Yunzeng Zou
Low density lipoprotein receptor-related protein 6 (LRP6), a wnt co-receptor, regulates multiple functions in various organs. However, the roles of LRP6 in the adult heart are not well understood. Methods: We observed LRP6 expression in heart with end-stage dilated cardiomyopathy (DCM) by western blot. Tamoxifen-inducible cardiac-specific LRP6 knockout mouse was constructed. Hemodynamic and echocardiographic analyses were performed to these mice. Results: Cardiac LRP6 expression was dramatically decreased in patients with end-stage dilated cardiomyopathy (DCM) compared to control group...
2018: Theranostics
https://www.readbyqxmd.com/read/29343862/angiotensin-ii-overstimulation-leads-to-an-increased-susceptibility-to-dilated-cardiomyopathy-and-higher-mortality-in-female-mice
#2
Sophie Mathieu, Nabil El Khoury, Katy Rivard, Pierre Paradis, Mona Nemer, Céline Fiset
Heart failure (HF) is associated with high mortality and affects men and women differently. The underlying mechanisms for these sex-related differences remain largely unexplored. Accordingly, using mice with cardiac-specific overexpression of the angiotensin II (ANGII) type 1 receptor (AT1R), we explored male-female differences in the manifestations of hypertrophy and HF. AT1R mice of both sexes feature electrical and Ca2+ handling alterations, systolic dysfunction, hypertrophy and develop HF. However, females had much higher mortality (21...
January 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29335596/circulating-microrna-signature-for-the-diagnosis-of-childhood-dilated-cardiomyopathy
#3
Meng Jiao, Hong-Zhao You, Xin-Ying Yang, Hui Yuan, Yu-Lin Li, Wen-Xian Liu, Mei Jin, Jie Du
Circulating miRNAs are proposed as a biomarker of heart disease. This study evaluated whether circulating miRNAs could be used as a biomarker for childhood dilated cardiomyopathy (CDCM). A total of 28 participants were enrolled in a discovery set, including patients with CDCM (n = 16) and healthy children (n = 12). The cardiac function of patients with CDCM was characterized by echocardiography and serum miRNA profiles of all participants were assessed by miRNA sequencing. After miRNA profiling, we quantitatively confirmed 148 regulated miRNAs in patients with CDCM compared with healthy subjects, and none were downregulated...
January 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29332614/serelaxin-for-infant-heart-failure-in-congenital-dilated-cardiomyopathy
#4
Patrick O Myers, Alice Bordessoule, Cécile Tissot
Serelaxin has been studied in trials in adults with acute heart failure, but not in children. We report the first compassionate use of serelaxin in an infant. A 6-month-old girl with dilated cardiomyopathy was placed on extracorporeal membrane oxygenation following cardiac arrest unresponsive to medical treatment. Extracorporeal membrane oxygenation weaning failed despite maximal ino-dilator therapy. During the 48-hour infusion of serelaxin, we observed marked improvement in brain natriuretic peptide, left ventricular systolic function, and dilatation...
January 15, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29326874/only-some-patients-with-bulbar-and-spinal-muscular-atrophy-may-develop-cardiac-disease
#5
Josef Finsterer, Claudia Stöllberger
Objectives: According to recent publications, some patients with spinal and bulbar muscular atrophy (BSMA) develop cardiac disease, manifesting as ST-segment abnormalities, Brugada-syndrome, dilative cardiomyopathy, or sudden cardiac death. Here we present neurological and cardiac data of a BSMA patient who was followed up for 10 y. Case report: In a male patient aged 47 y, BSMA was diagnosed at age 37 y upon the typical clinical presentation (postural tremor since age 12 y, dysarthria since age 15 y, muscle cramps since age 29 y, general myalgias since age 32 y, general fasciculations since age 34 y, myoclonic jerks, easy fatigability, dyspnea upon exercise since age 36 y) and a CAG-repeat expansion of 47 ± 1 repeats in the androgen-receptor gene detected at age 37 y...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29325795/insertional-mutagenesis-confounds-the-mechanism-of-the-morbid-phenotype-of-a-plnr9c-transgenic-mouse-line
#6
Alexander Kraev
BACKGROUND: A mouse line with heterozygous transgenic expression of phospholamban carrying a substitution of cysteine for arginine 9 (TgPLNR9C) under the control of α-myosin heavy chain (αMHC) promoter features dilated cardiomyopathy, heart failure and premature death. METHODS AND RESULTS: Determination of transgene chromosomal localization by conventional methods shows that in this line the transgenic array of 13 PLNR9C expression cassettes, arranged in a head-to-tail tandem orientation, has integrated into the bi-directional promoter of the αMHC (Myh6) gene and the gene for the regulatory non-coding RNA Myheart (Mhrt), both of which are known to be involved in cardiac development and pathology...
January 8, 2018: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/29323723/haplo-insufficiency-of-bcl2-associated-athanogene-3-in-mice-results-in-progressive-left-ventricular-dysfunction-%C3%AE-adrenergic-insensitivity-and-increased-apoptosis
#7
Valerie D Myers, Dhanendra Tomar, Muniswamy Madesh, JuFang Wang, Jianliang Song, Xue-Qian Zhang, Manish K Gupta, Farzaneh G Tahrir, Jennifer Gordon, Joseph M McClung, Christopher D Kontos, Kamel Khalili, Joseph Y Cheung, Arthur M Feldman
Bcl2-associated athanogene 3 (BAG3) is a 575 amino acid protein that is found predominantly in the heart, skeletal muscle and many cancers. Deletions and truncations in BAG3 that result in haplo-insufficiency have been associated with the development of dilated cardiomyopathy. To study the cellular and molecular events attributable to BAG3 haplo-insufficiency we generated a mouse in which one allele of BAG3 was flanked by loxP recombination sites (BAG3fl/+ ). Mice were crossed with α-MHC-Cre mice in order to generate mice with cardiac-specific haplo-insufficiency (cBAG3+/-) and underwent bi-weekly echocardiography to assess their cardiac phenotype...
January 11, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29323059/histological-and-morphometric-analysis-of-dilated-cardiomyopathy-with-special-reference-to-collagen-iv-expression
#8
Parul Jain, Sudheer Arava, Sandeep Seth, Sanjeev Lalwani, Ruma Ray
INTRODUCTION: Collagen distribution alterations are well known in dilated cardiomyopathy. There are also changes in microvasculature along with other histomorphorphological features. AIMS AND OBJECTIVES: To study the histomorphological features of DCM along with their quantitative correlation with LVEF. Alterations in collagen IV distribution pattern and microvasculature in DCM were also evaluated. MATERIALS AND METHODS: The present study includes 34 right ventricular endomyocardial biopsies, 7 explanted native hearts and 41 autopsy control hearts...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29320567/myocardium-of-patients-with-dilated-cardiomyopathy-presents-altered-expression-of-genes-involved-in-thyroid-hormone-biosynthesis
#9
Carolina Gil-Cayuela, Ana Ortega, Estefanía Tarazón, Luis Martínez-Dolz, Juan Cinca, José Ramón González-Juanatey, Francisca Lago, Esther Roselló-Lletí, Miguel Rivera, Manuel Portolés
BACKGROUND: The association between dilated cardiomyopathy (DCM) and low thyroid hormone (TH) levels has been previously described. In these patients abnormal thyroid function is significantly related to impaired left ventricular (LV) function and increased risk of death. Although TH was originally thought to be produced exclusively by the thyroid gland, we recently reported TH biosynthesis in the human ischemic heart. OBJECTIVES: Based on these findings, we evaluated whether the genes required for TH production are also altered in patients with DCM...
2018: PloS One
https://www.readbyqxmd.com/read/29316444/force-generation-via-%C3%AE-cardiac-myosin-titin-and-%C3%AE-actinin-drives-cardiac-sarcomere-assembly-from-cell-matrix-adhesions
#10
Anant Chopra, Matthew L Kutys, Kehan Zhang, William J Polacheck, Calvin C Sheng, Rebeccah J Luu, Jeroen Eyckmans, J Travis Hinson, Jonathan G Seidman, Christine E Seidman, Christopher S Chen
Truncating mutations in the sarcomere protein titin cause dilated cardiomyopathy due to sarcomere insufficiency. However, it remains mechanistically unclear how these mutations decrease sarcomere content in cardiomyocytes. Utilizing human induced pluripotent stem cell-derived cardiomyocytes, CRISPR/Cas9, and live microscopy, we characterize the fundamental mechanisms of human cardiac sarcomere formation. We observe that sarcomerogenesis initiates at protocostameres, sites of cell-extracellular matrix adhesion, where nucleation and centripetal assembly of α-actinin-2-containing fibers provide a template for the fusion of Z-disk precursors, Z bodies, and subsequent striation...
January 8, 2018: Developmental Cell
https://www.readbyqxmd.com/read/29315424/cardiac-resynchronization-therapy-in-patients-with-end-stage-hypertrophic-cardiomyopathy
#11
Ammar M Killu, Jae-Yoon Park, Jaskanwal D Sara, David O Hodge, Bernard J Gersh, Rick A Nishimura, Samuel J Asirvatham, Christopher J McLeod
Aims: A dilated/end-stage phase of hypertrophic cardiomyopathy (HCM) is rare but well-recognized. The role for cardiac resynchronization therapy (CRT) in this subset of patients remains unexplored. We aimed to clarify the impact of bi-ventricular pacing CRT in dilated/end-stage HCM. Methods and results: The Mayo Clinic HCM database was interrogated to identify patients with ejection fraction (EF) <50% and CRT. Control subjects were identified in 1:1 manner...
January 1, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29314019/white-blood-cell-differentials-in-dogs-with-congestive-heart-failure-chf-in-comparison-to-those-in-dogs-without-cardiac-disease
#12
J Hamilton-Elliott, E Ambrose, R Christley, J Dukes-McEwan
OBJECTIVES: To determine if dogs with congestive heart failure have different white blood cell differential cell counts than dogs without cardiac disease. MATERIALS AND METHODS: In total, 72 dogs with congestive heart failure and 143 controls were included in this retrospective study. Signalment, white blood cell differential counts and echocardiography data were retrieved. Basic statistical analysis was performed on white blood cell differential counts, and principal component analysis was used to compare these counts between cases and controls, with age, gender and case/control status as supplementary variables...
January 4, 2018: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/29313818/long-term-mortality-in-patients-with-severe-secondary-mitral-regurgitation-and-normal-left-ventricular-ejection-fraction-interventional-perspective
#13
Amr F Barakat, Mohammad Q Raza, Kinjal Banerjee, Rayji S Tsutsui, Amgad Mentias, Karim Abdur Rehman, Badal Thakkar, Sajjad Gul, Raquib Faruqui, Amar Krishnaswamy, Leonardo L Rodriguez, Richard Grimm, Brian Griffin, Emin Murat Tuzcu, Samir R Kapadia
AIMS: Patients with severe secondary mitral regurgitation (MR) and normal ejection fraction are being excluded from clinical trials evaluating transcatheter mitral devices. We sought to evaluate the long-term mortality with medical management alone in this patient population. METHODS AND RESULTS: We retrospectively evaluated patients diagnosed with ≥3+ mitral MR at our institution over 15 years. Only patients with ejection fraction ≥ 60% were included in the study...
January 9, 2018: EuroIntervention
https://www.readbyqxmd.com/read/29313593/a-patient-with-dilated-cardiomyopathy-and-portal-hypertension-which-beta-blocker-to-use
#14
Rathindranath Sarkar, Rudrajit Paul, Debaditya Roy, Asim Saha, Tanmay Jyoti Sau, Jayati Mondal
No abstract text is available yet for this article.
September 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29311597/putative-functional-genes-in-idiopathic-dilated-cardiomyopathy
#15
Nishanth Ulhas Nair, Avinash Das, Uri Amit, Welles Robinson, Seung Gu Park, Mahashweta Basu, Alex Lugo, Jonathan Leor, Eytan Ruppin, Sridhar Hannenhalli
Idiopathic dilated cardiomyopathy (DCM) is a complex disorder with a genetic and an environmental component involving multiple genes, many of which are yet to be discovered. We integrate genetic, epigenetic, transcriptomic, phenotypic, and evolutionary features into a method - Hridaya, to infer putative functional genes underlying DCM in a genome-wide fashion, using 213 human heart genomes and transcriptomes. Many genes identified by Hridaya are experimentally shown to cause cardiac complications. We validate the top predicted genes, via five different genome-wide analyses: First, the predicted genes are associated with cardiovascular functions...
January 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29311288/coronary-embolism-among-st-segment-elevation-myocardial-infarction-patients-mechanisms-and-management
#16
Batric Popovic, Nelly Agrinier, Nidhal Bouchahda, Samuel Pinelli, Charles Henry Maigrat, Pierre Adrien Metzdorf, Christine Selton Suty, Yves Juillière, Edoardo Camenzind
BACKGROUND: Coronary artery embolism (CE) is recognized as an important nonatherosclerotic cause of ST-segment-elevation myocardial infarction. The objective was to describe clinical characteristics and long-term outcomes and to identify risks factors of CE in a large consecutive series of ST-segment-elevation myocardial infarction patients. METHODS AND RESULTS: We studied 1232 consecutive patients who presented with de novo ST-segment-elevation myocardial infarction...
January 2018: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/29305677/coupling-interval-variability-of-premature-ventricular-contractions-in-patients-with-different-underlying-pathology-an-insight-into-the-arrhythmia-mechanism
#17
Lennart J de Vries, Mihran Martirosyan, Ron T van Domburg, Sip A Wijchers, Tamas Géczy, Tamas Szili-Torok
PURPOSE: Coupling interval (CI) variability of premature ventricular contractions (PVCs) is influenced by the underlying arrhythmia mechanism. The aim of this study was to compare CI variability of PVCs in different myocardial disease entities, in order to gain insight into their arrhythmia mechanism. METHODS: Sixty-four patients with four underlying pathologies were included: idiopathic (n = 16), non-ischemic dilated cardiomyopathy (NIDCM) (n = 16), familial cardiomyopathy (PLN/LMNA) (n = 16), and post-MI (n = 16)-associated PVCs...
January 5, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/29304022/muscle-specific-mis-splicing-and-heart-disease-exemplified-by-rbm20
#18
REVIEW
Maimaiti Rexiati, Mingming Sun, Wei Guo
Alternative splicing is an essential post-transcriptional process to generate multiple functional RNAs or proteins from a single transcript. Progress in RNA biology has led to a better understanding of muscle-specific RNA splicing in heart disease. The recent discovery of the muscle-specific splicing factor RNA-binding motif 20 (RBM20) not only provided great insights into the general alternative splicing mechanism but also demonstrated molecular mechanism of how this splicing factor is associated with dilated cardiomyopathy...
January 5, 2018: Genes
https://www.readbyqxmd.com/read/29301712/extracellular-volume-in-dilated%C3%A2-cardiomyopathy-interstitial-fibrosis-and%C3%A2-more
#19
EDITORIAL
Eloisa Arbustini, Valentina Favalli, Nupoor Narula
No abstract text is available yet for this article.
January 2018: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29297825/medical-management-of-dilated-cardiomyopathy-in-livingstone-fruit-bats-pteropus-livingstonii
#20
Rowena Killick, Alberto Rodriguez Barbon, Michelle Barrows, Andrew Routh, Richard Saunders, Charlotte Day, P G Cert Exotic Animal Studies, Adam Naylor, Nicolette Hayward, David Sewell, Kieran Borgeat, Aimee L Drane, Lois Wilkie
Eleven cases of dilated cardiomyopathy have been diagnosed and treated in captive Livingstone fruit bats ( Pteropus livingstonii) in the United Kingdom over the past 7 yr. All but one case received treatment with a diuretic plus an angiotensin-converting enzyme inhibitor (ACEI), and, or pimobendan. One case is still under treatment with pimobendan alone, following diagnosis before onset of clinical signs. Diuretic treatment consisted of furosemide at a dose rate of 0.5-5 mg/kg, one to three times daily, and, or spironolactone at a dose rate of 1-4 mg/kg, once or twice daily...
December 2017: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
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