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Arrhythmogenic cardiomyopathy

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https://www.readbyqxmd.com/read/28329361/sex-hormones-affect-outcome-in-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-from-a-stem-cell-derived-cardiomyocyte-based-model-to-clinical-biomarkers-of-disease-outcome
#1
Deniz Akdis, Ardan M Saguner, Khooshbu Shah, Chuanyu Wei, Argelia Medeiros-Domingo, Arnold von Eckardstein, Thomas F Lüscher, Corinna Brunckhorst, H S Vincent Chen, Firat Duru
Aims: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by fibrofatty infiltration of the myocardium and ventricular arrhythmias that may lead to sudden cardiac death. It has been observed that male patients develop the disease earlier and present with more severe phenotypes as compared to females. Thus, we hypothesized that serum levels of sex hormones may contribute to major arrhythmic cardiovascular events (MACE) in patients with ARVC/D. Methods and results: The serum levels of five sex hormones, sex hormone-binding globulin, high sensitivity troponin T, pro-brain natriuretic peptide, cholesterol, triglycerides, insulin, and glucose were measured in 54 ARVC/D patients (72% male)...
February 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28326674/whole-exome-sequencing-with-genomic-triangulation-implicates-cdh2-encoded-n-cadherin-as-a-novel-pathogenic-substrate-for-arrhythmogenic-cardiomyopathy
#2
Kari L Turkowski, David J Tester, J Martijn Bos, Kristina H Haugaa, Michael J Ackerman
BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is a heritable disease characterized by fibrofatty replacement of cardiomyocytes, has a prevalence of approximately 1 in 5000 individuals, and accounts for approximately 20% of sudden cardiac death in the young (≤35 years). ACM is most often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. While mutations in several genes that encode key desmosomal proteins underlie about half of all ACM, the remainder is elusive genetically...
March 21, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28326523/should-epsilon-wave-be-considered-as-a-major-diagnostic-criterion-in-arrhythmogenic-right-ventricular-cardiomyopathy
#3
Elżbieta K Biernacka, Pyotr G Platonov, Aneta Fronczak
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28319834/molecular-mechanisms-in-the-pathogenesis-of-arrhythmogenic-cardiomyopathy
#4
REVIEW
Jeffrey E Saffitz
The article is based on work presented in the Distinguished Achievement Award lecture at the Society for Cardiovascular Pathology meeting in Seattle, WA, in March 2016. It reviews our current understanding of mechanisms responsible for a highly arrhythmogenic, nonischemic cardiomyopathy. It highlights the armamentarium of powerful methods available to the experimental pathologist in efforts to define how complex cardiovascular diseases work. It concludes with acknowledgment of the need for a far more detailed approach as to how we categorize human disease, a task for which pathologists are especially well positioned...
February 27, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28315121/untangling-the-biology-of-genetic-cardiomyopathies-with-pluripotent-stem-cell-disease-models
#5
REVIEW
Jan W Buikema, Sean M Wu
PURPOSE OF REVIEW: Recently, the discovery of strategies to reprogram somatic cells into induced pluripotent stem (iPS) cells has led to a major paradigm change in developmental and stem cell biology. The application of iPS cells and their cardiac progeny has opened novel directions to study cardiomyopathies at a cellular and molecular level. This review discusses approaches currently undertaken to unravel known inherited cardiomyopathies in a dish. RECENT FINDINGS: With improved efficiency for mutation correction by genome editing, human iPS cells have now provided a platform to untangle the biology of cardiomyopathies...
April 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28290856/-chronic-heart-failure-with-preserved-systolic-function-and-reversible-dilatation-of-cardiac-chambers
#6
O V Blagova, S V Volkov, A V Nedostup, A O Korobkov, I V Mostovoy, N G Sergushina, N V Gagarina, E A Mershina
Diagnosis of dilation (D) cardiomyopathy (CMP) requires exclusion not only of inflammatory and genetically determined forms but also of some rare diseases. This 51 year old patient with history of moderate arterial hypertension approached a cardiologist because of new onset atrial fibrillation and dyspnea. Echocardiography detected dilation of all cardiac chambers with relatively preserved ejection fraction, causing suspicion of DCMP. Among conditions excluded were coronary atherosclerosis, congenital heart defect with left to right shunt, primary pulmonary hypertension, pulmonary embolism, hypertensive heart, tachycardia induced CMP, arrhythmogenic right ventricular dysplasia, noncompaction myocardium...
June 2016: Kardiologiia
https://www.readbyqxmd.com/read/28289018/adrenergic-signaling-strengthens-cardiac-myocyte-cohesion
#7
Camilla Schinner, Franziska Vielmuth, Vera Rötzer, Matthias Hiermaier, Mariya Radeva, Thu K Co, Eva Hartlieb, Andreas Schmidt, Axel Imhof, Ahmed Messoudi, Anja Horn, Angela Schlipp, Volker Spindler, Jens Waschke
Rationale: The sympathetic nervous system is a major mediator of heart function. Intercalated discs (ICDs) composed of desmosomes, adherens junctions and gap junctions provide the structural backbone for coordinated contraction of cardiac myocytes. Objective: Gap junctions dynamically remodel to adapt to sympathetic signaling. However, it is unknown whether such rapid adaption also occurs for the adhesive function provided by desmosomes and adherens junctions. Methods and Results: Atomic force microscopy revealed that β-adrenergic signaling enhances both the number of desmoglein 2 (Dsg2) -specific interactions along cell junctions and the mean Dsg2-mediated binding forces, whereas N-cadherin-mediated interactions were not affected...
March 13, 2017: Circulation Research
https://www.readbyqxmd.com/read/28288337/remodelling-of-myocardial-intercalated-disc-protein-connexin-43-causes-increased-susceptibility-to-malignant-arrhythmias-in-arvc-d-patients
#8
Xiao Chen, Liang Chen, Zhenglian Chen, Xinshan Chen, Jiangping Song
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a primary cardiomyopathy characterised by fibrofatty replacement and ventricular arrhythmias. The occurrence of malignant arrhythmias may be associated with fatty infiltration and intercalated disk remodelling, but the specific pathological remodelling pattern is not yet clear. METHODS: Twelve explanted hearts from patients diagnosed with ARVC/D according to the 2010 Task Force Criteria and pathology examination were divided into two groups with (SVT, n=6) or without (non-SVT, n=6) recurrent sustained ventricular tachycardia (SVT) before heart transplantation...
February 27, 2017: Forensic Science International
https://www.readbyqxmd.com/read/28286759/bioinformatics-analysis-reveals-micrornas-regulating-biological-pathways-in-exercise-induced-cardiac-physiological-hypertrophy
#9
REVIEW
Jiahong Xu, Yang Liu, Yuan Xie, Cuimei Zhao, Hongbao Wang
Exercise-induced physiological cardiac hypertrophy is generally considered to be a type of adaptive change after exercise training and is beneficial for cardiovascular diseases. This study aims at investigating exercise-regulated microRNAs (miRNAs) and their potential biological pathways. Here, we collected 23 miRNAs from 8 published studies. MirPath v.3 from the DIANA tools website was used to execute the analysis, and TargetScan was used to predict the target genes. Kyoto Encyclopedia of Genes and Genomes (KEGG) and Gene Ontology (GO) analyses were performed to identify potential pathways and functional annotations associated with exercise-induced physiological cardiac hypertrophy...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28283360/phenotypic-expression-of-arvc-how-12-lead-ecg-can-predict-left-or-right-ventricle-involvement-a-familiar-case-series-and-a-review-of-literature
#10
Luca Gaido, Alberto Battaglia, Mario Matta, Carla Giustetto, Simone Frea, Massimo Imazio, Elena Richiardi, Lucia Garberoglio, Fiorenzo Gaita
AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart-muscle disease primarily affecting the right ventricle (RV) and potentially causing sudden death in young people. The natural history of the disease is firstly characterized by a concealed form progressing over a biventricular involvement. Three different cases coming from the same family are presented together with a review of the literature. METHODS AND RESULTS: Multi-parameter analysis including imaging and electrocardiographic analysis is presented since the first medical referral with follow-up ranging from 11 to 38years...
February 28, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28280076/identification-of-cadherin-2-cdh2-mutations-in-arrhythmogenic-right-ventricular-cardiomyopathy
#11
Bongani M Mayosi, Maryam Fish, Gasnat Shaboodien, Elisa Mastantuono, Sarah Kraus, Thomas Wieland, Maria-Christina Kotta, Ashley Chin, Nakita Laing, Ntobeko B A Ntusi, Michael Chong, Christopher Horsfall, Simon N Pimstone, Davide Gentilini, Gianfranco Parati, Tim-Matthias Strom, Thomas Meitinger, Guillaume Pare, Peter J Schwartz, Lia Crotti
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically heterogeneous condition caused by mutations in genes encoding desmosomal proteins in up to 60% of cases. The 40% of genotype-negative cases point to the need of identifying novel genetic substrates by studying genotype-negative ARVC families. METHODS AND RESULTS: Whole exome sequencing was performed on 2 cousins with ARVC. Validation of 13 heterozygous variants that survived internal quality and frequency filters was performed by Sanger sequencing...
April 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28268089/burden-of-arrhythmias-in-peripartum-cardiomyopathy-analysis-of-9841-hospitalizations
#12
Sagar Mallikethi-Reddy, Emmanuel Akintoye, Naveen Trehan, Shikha Sharma, Alexandros Briasoulis, Kavyashri Jagadeesh, Melvyn Rubenfire, Cindy L Grines, Luis Afonso
BACKGROUND: Peripartum cardiomyopathy (PPCM) is associated with significant morbidity and mortality. Arrhythmogenic causes of death have been implicated in a significant number of patients. However, there is a dearth of systematic studies evaluating the burden of arrhythmias in PPCM. METHODS: We used the Healthcare Utilization Project, Nationwide Inpatient Sample database (2007-2012) and identified 9841 hospitalizations for women aged ≥18years with a primary diagnosis of PPCM...
February 24, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28266935/genetic-causes-of-sudden-cardiac-death-in-the-young
#13
Andrea Mazzanti, Riccardo Maragna, Silvia G Priori
PURPOSE OF REVIEW: In this article, we discuss the most recent and relevant studies published in the field of inherited arrhythmogenic disorders, focusing in particular on channelopathies (Long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia) and arrhythmogenic right ventricular cardiomyopathy (ARVC). RECENT FINDINGS: We discuss the updated diagnostic criteria for channelopathies released by the European Society of Cardiology, the new results on the value of programmed electrical stimulation in patients with Brugada syndrome, and the recent evidences supporting a genotype-specific therapy for Long QT syndrome type 3...
March 6, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28263909/natural-history-of-arrhythmogenic-cardiomyopathy-redefining-the-age-range-of-clinical-presentation
#14
EDITORIAL
Domenico Corrado, Alessandro Zorzi
No abstract text is available yet for this article.
March 2, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28261825/electrical-storm-in-icd-recipients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#15
Kang Yin, Ligang Ding, Wei Hua, Shu Zhang
BACKGROUND: Implantable cardioverter defibrillator (ICD) is the most important management for prevention of sudden cardiac death (SCD) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, some patients may receive multiple ICD therapies in a short period, a condition referred as "electrical storm". OBJECTIVES: This study aimed to determine the prevalence, therapeutic options, and prognostic implications of ES in ARVC patients with an ICD...
March 6, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28256248/whole-exome-sequencing-identifies-a-novel-mutation-of-desmocollin-2-in-a-chinese-family-with-arrhythmogenic-right-ventricular-cardiomyopathy
#16
Ji-Shi Liu, Liang-Liang Fan, Jing-Jing Li, Rong Xiang
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare heart disorder characterized by myocyte loss and fibro-fatty tissue replacement. With the progress of ARVC, patient can present serious ventricular arrhythmias, heart failure, and even sudden cardiac death. Previous studies have revealed that the generation and development of ARVC are related to structural changes of desmosomes. To date, at least 5 genes associated with desmosomes have been identified in patients with ARVC, including Desmoplakin, Plakophilin 2, Desmoglein 2, Desmocollin 2, and Junction plakoglobin...
February 10, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28255936/sudden-arrhythmic-death-during-exercise-a-post-mortem-genetic-analysis
#17
Oscar Campuzano, Olallo Sanchez-Molero, Anna Fernandez, Irene Mademont-Soler, Monica Coll, Alexandra Perez-Serra, Jesus Mates, Bernat Del Olmo, Ferran Pico, Laia Nogue-Navarro, Georgia Sarquella-Brugada, Anna Iglesias, Sergi Cesar, Esther Carro, Juan Carlos Borondo, Josep Brugada, Josep Castellà, Jordi Medallo, Ramon Brugada
BACKGROUND: Sudden cardiac death is a natural and unexpected death that occurs within 1 h of the first symptom. Most sudden cardiac deaths occur during exercise, mostly as a result of myocardial infarction. After autopsy, some cases, especially in the young, are diagnosed as cardiomyopathies or remain without a conclusive cause of death. In both situations, genetic alterations may explain the arrhythmia. OBJECTIVE: Our aim was to identify a genetic predisposition to sudden cardiac death in a cohort of post-mortem cases of individuals who died during exercise, with a structurally normal heart, and were classified as arrhythmogenic death...
March 3, 2017: Sports Medicine
https://www.readbyqxmd.com/read/28253841/identification-of-established-arrhythmogenic-right-ventricular-cardiomyopathy-mutation-in-a-patient-with-the-contrasting-phenotype-of-hypertrophic-cardiomyopathy
#18
Matthew Neil Bainbridge, Lili Li, Yanli Tan, Benjamin Y Cheong, Ali J Marian
BACKGROUND: Advances in the nucleic acid sequencing technologies have ushered in the era of genetic-based "precision medicine". Applications of the genetic discoveries to practice of medicine, however, are hindered by phenotypic variability of the genetic variants. The report illustrates extreme pleiotropic phenotypes associated with an established causal mutation for hereditary cardiomyopathy. CASE PRESENTATION: We report a 61-year old white female who presented with syncope and echocardiographic and cardiac magnetic resonance (CMR) imaging findings consistent with the diagnosis of hypertrophic cardiomyopathy (HCM)...
March 3, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28215569/cardiac-phenotype-and-long-term-prognosis-of-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-patients-with-late-presentation
#19
Aditya Bhonsale, Anneline S J M Te Riele, Abhishek C Sawant, Judith A Groeneweg, Cynthia A James, Brittney Murray, Crystal Tichnell, Thomas P Mast, Michelle J van der Pols, Maarten J M Cramer, Dennis Dooijes, Jeroen F van der Heijden, Harikrishna Tandri, J Peter van Tintelen, Daniel P Judge, Richard N W Hauer, Hugh Calkins
BACKGROUND: The clinical profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients with late presentation is unknown. OBJECTIVE: The purpose of this study was to characterize the genotype, cardiac phenotype, and long-term outcomes of ARVC/D patients with late presentation (age ≥50 years at diagnosis). METHODS: Five hundred two patients with an ARVC/D diagnosis from Johns Hopkins and Utrecht Registries were studied and long-term clinical outcomes ascertained...
February 12, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28185710/my-approach-to-the-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy-arvc
#20
Angeliki Asimaki
No abstract text is available yet for this article.
November 2, 2016: Trends in Cardiovascular Medicine
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