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Dilated cardiomyopathy

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https://www.readbyqxmd.com/read/28731484/cpr-ecls-bvad-and-successful-heart-transplantation-within-2-months-a-single-centre-case-series-in-two-young-high-urgency-listed-patients
#1
Anna K Schmidt, Diyar Saeed, Arash Mehdiani, Bozena Sowinski, Ralf Westenfeld, Payam Akhyari, Artur Lichtenberg, Udo Boeken
INTRODUCTION: In times of organ shortage, death while on the heart waiting-list still represents a major problem. As a consequence, bridging to transplant as well as the decision when to escalate therapy play a very important role. METHODS AND RESULTS: We report on two young patients with dilated cardiomyopathy and acute decompensation who were successfully bridged to heart transplantation with both left and temporary right ventricular assist devices in just 2 months...
July 20, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28730386/erratum-to-changes-of-myocardial-gene-expression-and-protein-composition-in-patients-with-dilated-cardiomyopathy-after-immunoadsorption-with-subsequent-immunoglobulin-substitution
#2
Sabine Ameling, Gourav Bhardwaj, Elke Hammer, Daniel Beug, Leif Steil, Yvonne Reinke, Kerstin Weitmann, Markus Grube, Christiane Trimpert, Karin Klingel, Reinhard Kandolf, Wolfgang Hoffmann, Matthias Nauck, Marcus Dörr, Klaus Empen, Stephan B Felix, Uwe Völker
No abstract text is available yet for this article.
July 2017: Basic Research in Cardiology
https://www.readbyqxmd.com/read/28724804/pediatric-dilated-cardiomyopathy-hearts-display-a-unique-gene-expression-profile
#3
Philip D Tatman, Kathleen C Woulfe, Anis Karimpour-Fard, Danielle A Jeffrey, James Jaggers, Joseph C Cleveland, Karin Nunley, Matthew Rg Taylor, Shelley D Miyamoto, Brian L Stauffer, Carmen C Sucharov
Our previous work showed myocellular differences in pediatric and adult dilated cardiomyopathy (DCM). However, a thorough characterization of the molecular pathways involved in pediatric DCM does not exist, limiting the development of age-specific therapies. To characterize this patient population, we investigated the transcriptome profile of pediatric patients. RNA-Seq from 7 DCM and 7 nonfailing (NF) explanted age-matched pediatric left ventricles (LV) was performed. Changes in gene expression were confirmed by real-time PCR (RT-PCR) in 36 DCM and 21 NF pediatric hearts and in 20 DCM and 10 NF adult hearts...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28724793/a-bag3-chaperone-complex-maintains-cardiomyocyte-function-during-proteotoxic-stress
#4
Luke M Judge, Juan A Perez-Bermejo, Annie Truong, Alexandre Js Ribeiro, Jennie C Yoo, Christina L Jensen, Mohammad A Mandegar, Nathaniel Huebsch, Robyn M Kaake, Po-Lin So, Deepak Srivastava, Beth L Pruitt, Nevan J Krogan, Bruce R Conklin
Molecular chaperones regulate quality control in the human proteome, pathways that have been implicated in many diseases, including heart failure. Mutations in the BAG3 gene, which encodes a co-chaperone protein, have been associated with heart failure due to both inherited and sporadic dilated cardiomyopathy. Familial BAG3 mutations are autosomal dominant and frequently cause truncation of the coding sequence, suggesting a heterozygous loss-of-function mechanism. However, heterozygous knockout of the murine BAG3 gene did not cause a detectable phenotype...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28724792/pediatric-and-adult-dilated-cardiomyopathy-represent-distinct-pathological-entities
#5
Meghna D Patel, Jayaram Mohan, Caralin Schneider, Geetika Bajpai, Enkhsaikhan Purevjav, Charles E Canter, Jeffrey Towbin, Andrea Bredemeyer, Kory J Lavine
Pediatric dilated cardiomyopathy (DCM) is the most common indication for heart transplantation in children. Despite similar genetic etiologies, medications routinely used in adult heart failure patients do not improve outcomes in the pediatric population. The mechanistic basis for these observations is unknown. We hypothesized that pediatric and adult DCM comprise distinct pathological entities, in that children do not undergo adverse remodeling, the target of adult heart failure therapies. To test this hypothesis, we examined LV specimens obtained from pediatric and adult donor controls and DCM patients...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28724790/med12-regulates-a-transcriptional-network-of-calcium-handling-genes-in-the-heart
#6
Kedryn K Baskin, Catherine A Makarewich, Susan M DeLeon, Wenduo Ye, Beibei Chen, Nadine Beetz, Heinrich Schrewe, Rhonda Bassel-Duby, Eric N Olson
The Mediator complex regulates gene transcription by linking basal transcriptional machinery with DNA-bound transcription factors. The activity of the Mediator complex is mainly controlled by a kinase submodule that is composed of 4 proteins, including MED12. Although ubiquitously expressed, Mediator subunits can differentially regulate gene expression in a tissue-specific manner. Here, we report that MED12 is required for normal cardiac function, such that mice with conditional cardiac-specific deletion of MED12 display progressive dilated cardiomyopathy...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28723664/detecting-the-genetic-link-between-alzheimer-s-disease-and-obesity-using-bioinformatics-analysis-of-gwas-data
#7
Qi-Shuai Zhuang, Hao Zheng, Xiao-Dan Gu, Liang Shen, Hong-Fang Ji
Alzheimer's disease (AD) represents the major form of dementia in the elderly. In recent years, accumulating evidence indicate that obesity may act as a risk factor for AD, while the genetic link between the two conditions remains unclear. This bioinformatics analysis aimed to detect the genetic link between AD and obesity on single nucleotide polymorphisms (SNPs), gene, and pathway levels based on genome-wide association studies data. A total of 31 SNPs were found to be shared by AD and obesity, which were linked to 7 genes...
July 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28716623/a-second-truncation-in-ttn-causes-early-onset-recessive-muscular-dystrophy
#8
Elizabeth Harris, Ana Töpf, Anna Vihola, Anni Evilä, Rita Barresi, Judith Hudson, Peter Hackman, Brian Herron, Daniel MacArthur, Hanns Lochmüller, Kate Bushby, Bjarne Udd, Volker Straub
Mutations in the gene encoding the giant skeletal muscle protein titin are associated with a variety of muscle disorders, including recessive congenital myopathies ±cardiomyopathy, limb girdle muscular dystrophy (LGMD) and late onset dominant distal myopathy. Heterozygous truncating mutations have also been linked to dilated cardiomyopathy. The phenotypic spectrum of titinopathies is emerging and expanding, as next generation sequencing techniques make this large gene amenable to sequencing. We undertook whole exome sequencing in four individuals with LGMD...
June 22, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28711447/the-transition-from-hypertension-to%C3%A2-heart%C3%A2-failure-contemporary-update
#9
REVIEW
Franz H Messerli, Stefano F Rimoldi, Sripal Bangalore
Longstanding hypertension ultimately leads to heart failure (HF), and as a consequence most patients with HF have a history of hypertension. Conversely, absence of hypertension in middle age is associated with lower risks for incident HF across the remaining life course. Cardiac remodeling to a predominant pressure overload consists of diastolic dysfunction and concentric left ventricular (LV) hypertrophy. When pressure overload is sustained, diastolic dysfunction progresses, filling of the concentric remodeled LV decreases, and HF with preserved ejection fraction ensues...
July 6, 2017: JACC. Heart Failure
https://www.readbyqxmd.com/read/28709811/arrhythmic-risk-stratification-in-non-ischemic-dilated-cardiomyopathy-where-do-we-stand-after-danish
#10
REVIEW
Polychronis Dilaveris, Christos-Konstantinos Antoniou, Konstantinos A Gatzoulis
Publication of the DANISH randomized trial led to considerable debate, given that it demonstrated no survival benefit stemming from current implantable cardioverter-defibrillator (ICD) allocation criteria in patients with non-ischemic dilated cardiomyopathy (NIDCM). Consequently, a thorough reconsideration of our approach to sudden cardiac death (SCD)-risk stratification appears to be in order. NIDCM encompasses a wide spectrum of disease entities, often with differing arrhythmogenicity; however, in its kernel, is still defined by the fundamentals of electrophysiology that dictate that abnormal tissue, exhibiting altered electrophysiological properties is necessary for arrhythmogenesis, but not enough, given that formation of functional circuits is required...
June 7, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28706255/mitsugumin-29-regulates-t-tubule-architecture-in-the-failing-heart
#11
Robert N Correll, Jeffrey M Lynch, Tobias G Schips, Vikram Prasad, Allen J York, Michelle A Sargent, Didier X P Brochet, Jianjie Ma, Jeffery D Molkentin
Transverse tubules (t-tubules) are uniquely-adapted membrane invaginations in cardiac myocytes that facilitate the synchronous release of Ca(2+) from internal stores and subsequent myofilament contraction, although these structures become disorganized and rarefied in heart failure. We previously observed that mitsugumin 29 (Mg29), an important t-tubule organizing protein in skeletal muscle, was induced in the mouse heart for the first time during dilated cardiomyopathy with heart failure. Here we generated cardiac-specific transgenic mice expressing Mg29 to model this observed induction in the failing heart...
July 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28705874/automated-quantification-of-low-amplitude-abnormal-qrs-peaks-from-high-resolution-ecg-recordings-predicts-arrhythmic-events-in-patients-with-cardiomyopathy
#12
Moloy Das, Adrian M Suszko, Sachin Nayyar, Karthik Viswanathan, Danna A Spears, George Tomlinson, Arnold Pinter, Eugene Crystal, Rupin Dalvi, Sridhar Krishnan, Vijay S Chauhan
BACKGROUND: Cardiomyopathy patients are at risk of sudden death, typically from scar-related abnormalities of electrical activation that promote ventricular tachyarrhythmias. Abnormal intra-QRS peaks may provide a measure of altered activation. We hypothesized that quantification of such QRS peaks (QRSp) in high-resolution ECGs would predict arrhythmic events in implantable cardioverter-defibrillator (ICD)-eligible cardiomyopathy patients. METHODS AND RESULTS: Ninety-nine patients with ischemic or non-ischemic dilated cardiomyopathy undergoing prophylactic ICD implantation were prospectively enrolled (age 62±11 years, left ventricular ejection fraction 27±7%)...
July 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28704447/sexual-dimorphisms-of-mrna-and-mirna-in-human-murine-heart-disease
#13
Masato Tsuji, Takanori Kawasaki, Takeru Matsuda, Tomio Arai, Satoshi Gojo, Jun K Takeuchi
BACKGROUND: Sexual dimorphisms are well recognized in various cardiac diseases such as ischemic cardiomyopathy (ICM), hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). Thorough understanding of the underlying genetic programs is crucial to optimize treatment strategies specified for each gender. By performing meta-analysis and microarray analysis, we sought to comprehensively characterize the sexual dimorphisms in the healthy and diseased heart at the level of both mRNA and miRNA transcriptome...
2017: PloS One
https://www.readbyqxmd.com/read/28702159/hodgkin-s-lymphoma-in-a-man-with-dilated-cardiomyopathy-and-paraneoplastic-ataxia-a-therapeutical-challenge
#14
Elisabetta Abruzzese, Malgorzata Monika Trawinska, Achille Gaspardone, Antonino Morocutti, Paolo de Fabritiis
Hodgkin's lymphoma is a cancer of the lymphatic system. We report the case of a man with Hodgkin's lymphoma and cardiomyopathy, for which the dilemma was whether to use the standard protocol - putting the patient at risk of worsening of heart failure, but giving him a good chance of full recovery - or not. The standard protocol was given and the patient made a full recovery without cardiac complications.
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28701682/prognostic-impact-of-segmental-wall-motion-abnormality-in-patients-with-idiopathic-dilated-cardiomyopathy
#15
Yuichiro Iida, Takayuki Inomata, Toyoji Kaida, Teppei Fujita, Yuki Ikeda, Takeru Nabeta, Shunsuke Ishii, Toshimi Koitabashi, Ichiro Takeuchi, Junya Ako
The clinical impact of left ventricular (LV) segmental wall motion abnormalities (SWMA) in patients with idiopathic dilated cardiomyopathy (IDCM) has not been well elucidated.Among 100 consecutive IDCM patients with follow-up visits, we enrolled 85 after excluding those with left bundle branch block and/or ventricular pacemaker implantation. LV wall motion was assessed using left ventriculography scored for 7 segments according to the American Heart Association classification as follows: 0, normokinesis; 1, hypokinesis; 2, akinesis; and 3, dyskinesis...
July 13, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28701297/genomic-analysis-of-an-infant-with-intractable-diarrhea-and-dilated-cardiomyopathy
#16
Dale L Bodian, Thierry Vilboux, Suchitra K Hourigan, Callie L Jenevein, Haresh Mani, Kathleen C Kent, Alina Khromykh, Benjamin D Solomon, Natalie S Hauser
We describe a case of an infant presenting with intractable diarrhea who subsequently developed dilated cardiomyopathy, for whom a diagnosis was not initially achieved despite extensive clinical testing, including panel-based genetic testing. Research-based whole genome sequences of the proband and both parents were analyzed by the SAVANNA pipeline, a variant prioritization strategy integrating features of variants, genes, and phenotypes, which was implemented using publicly available tools. Although the intestinal morphological abnormalities characteristic of congenital tufting enteropathy (CTE) were not observed in the initial clinical gastrointestinal tract biopsies of the proband, an intronic variant, EPCAM c...
July 12, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/28698364/loss-of-mouse-cardiomyocyte-talin-1-and-talin-2-leads-to-%C3%AE-1-integrin-reduction-costameric-instability-and-dilated-cardiomyopathy
#17
Ana Maria Manso, Hideshi Okada, Francesca M Sakamoto, Emily Moreno, Susan J Monkley, Ruixia Li, David R Critchley, Robert S Ross
Continuous contraction-relaxation cycles of the heart require strong and stable connections of cardiac myocytes (CMs) with the extracellular matrix (ECM) to preserve sarcolemmal integrity. CM attachment to the ECM is mediated by integrin complexes localized at the muscle adhesion sites termed costameres. The ubiquitously expressed cytoskeletal protein talin (Tln) is a component of muscle costameres that links integrins ultimately to the sarcomere. There are two talin genes, Tln1 and Tln2. Here, we tested the function of these two Tln forms in myocardium where Tln2 is the dominant isoform in postnatal CMs...
July 11, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28696268/personalizing-risk-stratification-for-sudden-death-in-dilated-cardiomyopathy-the-past-present-and-future
#18
REVIEW
Brian P Halliday, John G F Cleland, Jeffrey J Goldberger, Sanjay K Prasad
Results from the DANISH Study (Danish Study to Assess the Efficacy of ICDs in Patients With Non-Ischemic Systolic Heat Failure on Mortality) suggest that for many patients with dilated cardiomyopathy (DCM), implantable cardioverter-defibrillators do not increase longevity. Accurate identification of patients who are more likely to die of an arrhythmia and less likely to die of other causes is required to ensure improvement in outcomes and wise use of resources. Until now, left ventricular ejection fraction has been used as a key criterion for selecting patients with DCM for an implantable cardioverter-defibrillator for primary prevention purposes...
July 11, 2017: Circulation
https://www.readbyqxmd.com/read/28695818/progress-in-molecular-genetic-study-of-mitochondrial-cardiomyopathy
#19
Ruiqi Zhuge, Rong Zhou, Xinhai Ni
Mitochondria plays a key role in providing ATP for the energy-consuming cardiac tissues. Mitochondrial cardiomyopathy is a myocardial condition characterized by abnormal heart structure and/or function secondary to genetic defects involving the mitochondrial respiratory chain. The typical cardiac manifestations of mitochondrial cardiomyopathy include hypertrophic and dilated cardiomyopathy,while left ventricular myocardial noncompaction is less common. Recent research has suggested that most mitochondrial diseases result from mitochondrial DNA mutation,which can be found in genes that encode ancillary proteins needed for genetic transcription (tRNA),in genes that encode subunits of the electron transport chain complexes,or in genes that control the activities of the mitochondria called D-loop zone...
June 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28694354/myocardial-upregulation-of-cathepsin-d-by-ischemic-heart-disease-promotes-autophagic-flux-and-protects-against-cardiac-remodeling-and-heart-failure
#20
Penglong Wu, Xun Yuan, Faqian Li, Jianhua Zhang, Wei Zhu, Meng Wei, Jingbo Li, Xuejun Wang
BACKGROUND: Lysosomal dysfunction is implicated in human heart failure for which ischemic heart disease is the leading cause. Altered myocardial expression of CTSD (cathepsin D), a major lysosomal protease, was observed in human heart failure, but its pathophysiological significance has not been determined. METHODS AND RESULTS: Western blot analyses revealed an increase in the precursor but not the mature form of CTSD in myocardial samples from explanted human failing hearts with ischemic heart disease, which is recapitulated in chronic myocardial infarction produced via coronary artery ligation in Ctsd(+/+) but not Ctsd(+/-) mice...
July 2017: Circulation. Heart Failure
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