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Hypertrophy cardiomyopathy

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https://www.readbyqxmd.com/read/28533215/inhibition-of-hdac3-prevents-diabetic-cardiomyopathy-in-ove26-mice-via-epigenetic-regulation-of-dusp5-erk1-2-pathway
#1
Zheng Xu, Qian Tong, Zhiguo Zhang, Shudong Wang, Yang Zheng, Qiuju Liu, Lingbo Qian, Shao-Yu Chen, Jian Sun, Lu Cai
Inhibition of total histone deacetylases (HDACs) was phenomenally associated with the prevention of diabetic cardiomyopathy (DCM). However, which specific HDAC plays the key role in DCM remains unclear. The present study was designed to determine whether DCM can be prevented by specific inhibition of HDAC3 and to elucidate the mechanisms by which inhibition of HDAC3 prevent DCM. Type 1 diabetes OVE26 and age-matched wild-type mice were given the selective HDAC3 inhibitor RGFP966 or vehicle for 3 months. These mice were then sacrificed immediately or 3 months later for cardiac function and pathological examination...
May 22, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28529009/assembly-defects-of-multiple-respiratory-chain-complexes-in-a-child-with-cardiac-hypertrophy-associated-with-a-novel-acad9-mutation
#2
Konstantina Fragaki, Annabelle Chaussenot, Audrey Boutron, Sylvie Bannwarth, Cecile Rouzier, Brigitte Chabrol, Veronique Paquis-Flucklinger
Patients carrying Acyl-CoA dehydrogenase 9 (ACAD9) mutations reported to date mainly present with severe hypertrophic cardiomyopathy and isolated complex I (CI) dysfunction. Here we report a novel ACAD9 mutation in a young girl presenting with severe hypertrophic cardiomyopathy, isolated CI deficiency and interestingly multiple respiratory chain complexes assembly defects. We show that ACAD9 analysis has to be performed in first intention in patients presenting with cardiac hypertrophy even in the presence of multiple assembly defects...
May 4, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28523323/discovery-of-a-new-mutation-in-the-desmin-gene-in-a-young-patient-with-cardiomyopathy-and-muscular-weakness
#3
Ruxandra Oana JurcuŢ, Alexandra Eugenia Bastian, Sebastian Militaru, Aura Popa, Emilia Manole, Bogdan Alexandru Popescu, Jonna Tallila, Bogdan Ovidiu Popescu, Carmen Doina Ginghină
A 25-year-old woman with a five years history of syncope, mild left ventricular hypertrophy and moderately enlarged atria, was diagnosed with third degree atrioventricular heart block alternating with atrioventricular heart block 2:1, and received a dual chamber pacemaker. After three years of evolution, she developed atrial fibrillation, marked biatrial enlargement, severely depressed longitudinal myocardial velocities, associated with mild girdle weakness and slight increase in creatine kinase level. The diagnosis of restrictive cardiomyopathy with mild skeletal myopathy imposed the screening for a common etiology...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28520945/systolic-anterior-motion-of-the-tricuspid-valve-in-a-patient-with-hypertrophic-obstructive-cardiomyopathy
#4
Emile S Farag, R Nils Planken, S Matthijs Boekholdt, Jolanda Kluin
Hypertrophic cardiomyopathy is a heterogeneous myocardial disease and is characterized by increased left ventricular wall thickness. Left ventricular outflow tract obstruction occurs in up to 70% of patients and is often caused by systolic anterior motion of the mitral valve, a paradoxical phenomenon in which the anterior mitral valve leaflet is pulled into the left ventricular outflow tract during systole. We present the first case of hypertrophic cardiomyopathy with systolic anterior motion of both the mitral and the tricuspid valves and severe hypertrophy of both ventricles...
May 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28520448/-the-role-of-cardiovascular-magnetic-resonance-imaging-in-the-diagnosis-of-hypertrophic-cardiomyopathy-part-ii
#5
Martin Pleva, Júlia Borová, Ilona Plevová, Jaroslav Januška, Margita Belicová
Hypertrophic cardiomyopathy is currently understood as a group of diseases with left ventricular hypertrophy, which are not based on adaptive mechanisms. The first part of the review details the possibility of cardiac magnetic resonance in the diagnosis of sarcomeric forms of hypertrophic cardiomyopathy, the second part will focus on the possibilities of distinguishing the sarcomeric forms from their phenocopies.Key words: cardiac magnetic resonance - hypertrophic cardiomyopathy - phenocopies.
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28504961/wnt-%C3%AE-catenin-pathway-in-arrhythmogenic-cardiomyopathy
#6
REVIEW
Alessandra Lorenzon, Martina Calore, Giulia Poloni, Leon J De Windt, Paola Braghetta, Alessandra Rampazzo
Wnt/β-catenin signaling pathway plays essential roles in heart development as well as cardiac tissue homoeostasis in adults. Abnormal regulation of this signaling pathway is linked to a variety of cardiac disease conditions, including hypertrophy, fibrosis, arrhythmias, and infarction. Recent studies on genetically modified cellular and animal models document a crucial role of Wnt/β-catenin signaling in the molecular pathogenesis of arrhythmogenic cardiomyopathy (AC), an inherited disease of intercalated discs, typically characterized by ventricular arrhythmias and progressive substitution of the myocardium with fibrofatty tissue...
April 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28500252/transcriptome-profiling-reveals-novel-bmi-and-sex-specific-gene-expression-signature-for-human-cardiac-hypertrophy
#7
Mackenzie S Newman, Tina Nguyen, Michael J Watson, Robert Hull, Han-Gang Yu
BACKGROUND: How obesity or sex may affect the gene expression profiles of human cardiac hypertrophy is unknown. We hypothesized that body-mass index (BMI) and sex can affect gene expression profiles of cardiac hypertrophy. RESULTS: Human heart tissues were grouped according to sex (male, female), BMI (lean<25 kg/m(2), obese>30 kg/m(2)), or left ventricular hypertrophy (LVH) and non-LVH non-failed controls (NF). We identified 24 DE genes comparing female to male samples...
May 12, 2017: Physiological Genomics
https://www.readbyqxmd.com/read/28498411/mitochondrial-activity-and-oxidative-stress-functions-are-influenced-by-the-activation-of-ahr-induced-cyp1a1-overexpression-in-cardiomyocytes
#8
Bing Zhou, Xi Wang, Feng Li, Yingting Wang, Lei Yang, Xiaolong Zhen, Wuhong Tan
There is an endemic cardiomyopathy currently occurring in China, termed, Keshan disease (KD). The authors previously compared mitochondrial‑associated gene expression profiles of peripheral blood mononuclear cells (PBMCs) derived from KD patients and normal controls, using mitochondria‑focused cDNA microarray technology. The results detected an upregulation of the enzyme‑associated CYP1A1 gene, (ratios ≥2.0). The aryl hydrocarbon receptor (AhR) regulates the expression of numerous cytochrome P450 (CYP) genes including members of the CYP1 family; CYP1A1 and CYP1A2...
May 12, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28496025/identification-of-a-novel-gla-gene-mutation-p-ile239met-in-fabry-disease-with-a-predominant-cardiac-phenotype
#9
Beáta Csányi, Lidia Hategan, Viktória Nagy, Izabella Obál, Edina T Varga, János Borbás, Annamária Tringer, Sabrina Eichler, Tamás Forster, Arndt Rolfs, Róbert Sepp
Fabry disease (FD) is an X-linked inherited lysosomal storage disorder caused by mutations in the GLA gene, encoding for the enzyme α-galactosidase A. Although hundreds of mutations in the GLA gene have been described, many of them are variants of unknown significance. Here we report a novel GLA mutation, p.Ile239Met, identified in a large Hungarian three-generation family with FD. A 69 year-old female index patient with a clinical history of renal failure, hypertrophic cardiomyopathy, and 2nd degree AV block was screened for mutation in the GLA gene...
May 12, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28490697/-effect-of-left-ventricular-hypertrophy-and-deformation-on-cardiac-function-in-patients-with-uremia-cardiomyopathy-by-using-quantitative-speckle-tracking-technology
#10
Yi Zheng, Xiaohong Yang, Qiping Zhou, Yaoyao Huang, Fan Zhang, Liangyu Wang
To evaluate the effect of left ventricular hypertrophy and deformation on cardiac function in patients with uremic cardiomyopathy (UCM) by using the technology of two dimensional speckle tracking imaging (2D-STI).
 Methods: A total of 67 UCM patients were randomly divided into the normal cardiac function group (subgroup A, 32 cases) and the abnormal cardiac function group (subgroup B, 35 cases) according to the New York Heart Association points (NYHA-P). A total of 30 healthy subjetcs served as the control group...
April 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28487469/phosphoinositide-3-kinase-p110%C3%AE-gene-delivery-limits-diabetes-induced-cardiac-nadph-oxidase-and-cardiomyopathy-in-a-mouse-model-with-established-diastolic-dysfunction
#11
Darnel Prakoso, Miles J DeBlasio, Chengxue Qin, Sarah Rosli, Helen Kiriazis, Hongwei Qian, Xiao-Jun Du, Kate L Weeks, Paul Gregorevic, Julie R McMullen, Rebecca H Ritchie
Phosphoinositide 3-kinase [PI3K (p110α)] is able to negatively regulate the diabetes-induced increase in NADPH oxidase in the heart. Patients affected by diabetes exhibit significant cardiovascular morbidity and mortality, at least in part due to a cardiomyopathy characterised by oxidative stress and left ventricular (LV) dysfunction. Thus, PI3K (p110α) may represent a novel approach to protect the heart from diabetes-induced cardiac oxidative stress and dysfunction. In the current study, we investigated the therapeutic potential of a delayed intervention with cardiac-targeted PI3K gene therapy, administered to mice with established diabetes-induced LV diastolic dysfunction...
May 9, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28483204/usefulness-of-non-anteroseptal-region-left-ventricular-hypertrophy-using-cardiac-magnetic-resonance-to-predict-repeat-alcohol-septal-ablation-for-refractory-obstructive-hypertrophic-cardiomyopathy
#12
Mitsunobu Kitamura, Yasuo Amano, Morimasa Takayama, Junsuke Shibuya, Junya Matsuda, Hideto Sangen, Shunichi Nakamura, Hitoshi Takano, Kuniya Asai, Shinichiro Kumita, Wataru Shimizu
We evaluated a cohort of patients treated with alcohol septal ablation (ASA) to identify predictive factors for repeat ASA. We compared 15 patients who underwent repeat ASA procedures (group R) with 69 patients not requiring repeat procedures (group S) in terms of clinical parameters and morphologic cardiac magnetic resonance. Group R showed higher number of hypertrophic segments (thickness ≥15 mm) in the basal left ventricular level (2.8 ± 1.7 vs 1.7 ± 0.8, p = 0.009) than group S. In the multivariate analysis, diuretics use (adjusted odds ratio 5...
April 12, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28482693/risk-factors-for-sudden-cardiac-death-in-childhood-hypertrophic-cardiomyopathy-a-systematic-review-and-meta-analysis
#13
Gabrielle Norrish, Nicoletta Cantarutti, Eleni Pissaridou, Deborah A Ridout, Giuseppe Limongelli, Perry M Elliott, Juan Pablo Kaski
Aims To perform a systematic literature review and meta-analysis of clinical risk factors for sudden cardiac death (SCD) in childhood hypertrophic cardiomyopathy. Methods Medline and PubMed databases were searched for original articles published in English from 1963 through to December 2015 that included patients under 18 years of age with a primary or secondary end-point of either SCD or SCD-equivalent events (aborted cardiac arrest or appropriate implantable cardioverter-defibrillator discharge) or cardiovascular death (CVD)...
January 1, 2017: European Journal of Preventive Cardiology
https://www.readbyqxmd.com/read/28473928/dka-induced-takotsubo-cardiomyopathy-in-patient-with-known-hocm
#14
Ayla Gordon, Gina LaCapra, Roberto Roberti
The first published case of Diabetic Ketoacidosis-induced Takotsubo cardiomyopathy was in 2009. Our patient is the 1st reported case of Diabetic Ketoacidosis- (DKA-) induced Takotsubo cardiomyopathy (TC) in a patient with known hypertrophic cardiomyopathy (HOCM) in the United States. In the literature, there are only two examples linking DKA to TC; however, this report focuses on the biochemical and physiological causes of TC in a patient with known HOCM and new-onset DKA. TC in previously diagnosed HOCM poses particular complications...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28473471/are-targeted-therapies-for-diabetic-cardiomyopathy-on-the-horizon
#15
REVIEW
Mitchel Tate, David J Grieve, Rebecca H Ritchie
Diabetes increases the risk of heart failure approximately 2.5-fold, independent of coronary artery disease and other comorbidities. This process, termed diabetic cardiomyopathy, is characterized by initial impairment of left ventricular (LV) relaxation followed by LV contractile dysfunction. Post-mortem examination reveals that human diastolic dysfunction is closely associated with LV damage, including cardiomyocyte hypertrophy, apoptosis and fibrosis, with impaired coronary microvascular perfusion. The pathophysiological mechanisms underpinning the characteristic features of diabetic cardiomyopathy remain poorly understood, although multiple factors including altered lipid metabolism, mitochondrial dysfunction, oxidative stress, endoplasmic reticulum (ER) stress, inflammation, as well as epigenetic changes, are implicated...
May 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28457738/acromegalic-cardiomyopathy-with-malignant-arrhythmogenic-pattern-successfully-treated-with-mechanical-circulatory-support-and-heart-transplantation
#16
Sara Doimo, Daniela Miani, Nicoletta Finato, Mauro Driussi, Gianfranco Sinagra, Ugolino Livi, Alessandro Proclemer
Cardiovascular involvement is common in acromegaly and can lead to development of acromegalic cardiomyopathy, characterized by concentric biventricular hypertrophy with a progressive impairment of diastolic and systolic function. The onset of heart failure and arrhythmias are related to poor prognosis. We report on a case of a 48-year-old man with acromegalic cardiomyopathy caused by pituitary adenoma. Despite the successful trans-sphenoidal resection of the tumour, the patient was rehospitalized for ventricular arrhythmic storms that led to cardiogenic shock, which required mechanical hemodynamic support with intra-aortic balloon pump, venoarterial extracorporeal membrane oxygenation, and urgent heart transplantation...
February 21, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28454798/the-design-of-the-valsartan-for-attenuating-disease-evolution-in-early-sarcomeric-hypertrophic-cardiomyopathy-vanish-trial
#17
Carolyn Y Ho, John J V McMurray, Allison L Cirino, Steven D Colan, Sharlene M Day, Akshay S Desai, Steven E Lipshultz, Calum A MacRae, Ling Shi, Scott D Solomon, E John Orav, Eugene Braunwald
Hypertrophic cardiomyopathy (HCM) is often caused by sarcomere gene mutations, resulting in left ventricular hypertrophy (LVH), myocardial fibrosis, and increased risk of sudden cardiac death and heart failure. Studies in mouse models of sarcomeric HCM demonstrated that early treatment with an angiotensin receptor blocker (ARB) reduced development of LVH and fibrosis. In contrast, prior human studies using ARBs for HCM have targeted heterogeneous adult cohorts with well-established disease. The VANISH trial is testing the safety and feasibility of disease-modifying therapy with an ARB in genotyped HCM patients with early disease...
May 2017: American Heart Journal
https://www.readbyqxmd.com/read/28453725/serelaxin-treatment-promotes-adaptive-hypertrophy-but-does-not-prevent-heart-failure-in-experimental-peripartum-cardiomyopathy
#18
Justus Nonhoff, Melanie Ricke-Hoch, Mirco Mueller, Britta Stapel, Tobias Pfeffer, Martina Kasten, Michaela Scherr, Constantin von Kaisenberg, Johann Bauersachs, Arash Haghikia, Denise Hilfiker-Kleiner
Aims: Peripartum cardiomyopathy (PPCM) is a systolic left ventricular dysfunction developing in the peripartum phase in previously healthy women. Relaxin-2 is a pregnancy hormone with potential beneficial effects in heart failure patients. We evaluated Relaxin-2 as a potential diagnostic marker and/or a therapeutic agent in PPCM. Methods and results: In healthy peripartum women, serum Relaxin-2 levels (measured by ELISA in the second half of pregnancy) were elevated showing a decreasing trend in the first postpartum week and returned to non-pregnant levels thereafter...
May 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28450932/echocardiographic-characterization-of-hypertrophic-cardiomyopathy-in-chinese-patients-with-myosin-binding-protein-c3-mutations
#19
Bei Zhao, Shouli Wang, Jinsong Chen, Yali Ji, Jing Wang, Xiaoli Tian, Guang Zhi
Hypertrophic cardiomyopathy (HCM) is a common autosomal dominant cardiac disease, affecting 1 in 500 people. Myosin-binding protein C3 (MyBPC3) gene mutations are the most common genetic cause of HCM. However, the prevalence of the MyBPC3 gene mutation in Chinese patients with HCM, and their echocardiographic characteristics, remain unknown. In the present study, 48 Chinese patients with HCM were sequenced to identify the MyBPC3 gene and were characterized by their clinical features using 2-dimensional echocardiography and real-time 3-dimensional echocardiography...
March 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28446882/modified-low-dose-triiodo-l-thyronine-therapy-safely-improves-function-following-myocardial-ischemia-reperfusion-injury
#20
Viswanathan Rajagopalan, Youhua Zhang, Christine Pol, Clifford Costello, Samantha Seitter, Ann Lehto, Olga V Savinova, Yue-Feng Chen, A Martin Gerdes
Background: We have shown that thyroid hormones (THs) are cardioprotective and can be potentially used as safe therapeutic agents for diabetic cardiomyopathy and permanent infarction. However, no reliable, clinically translatable protocol exists for TH treatment of myocardial ischemia-reperfusion (IR) injury. We hypothesized that modified low-dose triiodo-L-thyronine (T3) therapy would confer safe therapeutic benefits against IR injury. Methods: Adult female rats underwent left coronary artery ligation for 60 min or sham surgeries...
2017: Frontiers in Physiology
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