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Arrhythmogenic cardiomyopathy

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https://www.readbyqxmd.com/read/27909515/myocardial-biopsy-in-idiopathic%C3%A2-atrial-fibrillation-and-other-arrhythmias-nosological-diagnosis-clinical-and-morphological-parallels-and-treatment
#1
O V Blagova, A V Nedostup, E A Kogan, V A Sulimov, S A Abugov, A G Kupriyanova, V A Zaydenov, A E Donnikov, E V Zaklyazminskaya, E A Okisheva
BACKGROUND: The nosological nature of "idiopathic" arrhythmias and the effect of etiotropic and pathogenetic treatment are often unknown. METHODS AND RESULTS: 19 patients (42.6±11.3 years, 9 women) with atrial fibrillation (n = 16), supraventricular (n = 10) and ventricular (n = 4) premature beats, supraventricular (n = 2) and ventricular tachycardia (n = 1), left bundle branch block (n= 2), AV block (n = 2) without structural heart changes. Viruses were identified (polymerase chain reaction, PCR) along with measurement of anti-heart antibodies (AHA) and endomyocardial biopsy (EMB)...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27909514/influence-of-novel-electrocardiographic-features-of-provocable-brugada-ecg-in-arrhythmogenic-cardiomyopathy-and-its-exclusion-by-lead-avr
#2
REVIEW
Stefan Peters
In 19 patients (14 females, mean age 49.1 ± 11.3 years) with typical arrhythmogenic cardiomyopathy and provocable type I Brugada ECG pattern by ajmaline administration were analysed by novel electrocardiographic features as having "true" or "false" Brugada syndrome. Three patients turned out as having false Brugada syndrome, the diagnosis is pure arrhythmogenic cardiomyopathy. In 16 patients, however, true Brugada syndrome could be provoked. In these patients the diagnosis was arrhythmogenic cardiomyopathy associated by provocable Brugada syndrome...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27908349/truncating-flnc-mutations-are-associated-with-high-risk-dilated-and-arrhythmogenic-cardiomyopathies
#3
Martín F Ortiz-Genga, Sofía Cuenca, Matteo Dal Ferro, Esther Zorio, Ricardo Salgado-Aranda, Vicente Climent, Laura Padrón-Barthe, Iria Duro-Aguado, Juan Jiménez-Jáimez, Víctor M Hidalgo-Olivares, Enrique García-Campo, Chiara Lanzillo, M Paz Suárez-Mier, Hagith Yonath, Sonia Marcos-Alonso, Juan P Ochoa, José L Santomé, Diego García-Giustiniani, Jorge L Rodríguez-Garrido, Fernando Domínguez, Marco Merlo, Julián Palomino, María L Peña, Juan P Trujillo, Alicia Martín-Vila, Davide Stolfo, Pilar Molina, Enrique Lara-Pezzi, Francisco E Calvo-Iglesias, Eyal Nof, Leonardo Calò, Roberto Barriales-Villa, Juan R Gimeno-Blanes, Michael Arad, Pablo García-Pavía, Lorenzo Monserrat
BACKGROUND: Filamin C (encoded by the FLNC gene) is essential for sarcomere attachment to the plasmatic membrane. FLNC mutations have been associated with myofibrillar myopathies, and cardiac involvement has been reported in some carriers. Accordingly, since 2012, the authors have included FLNC in the genetic screening of patients with inherited cardiomyopathies and sudden death. OBJECTIVES: The aim of this study was to demonstrate the association between truncating mutations in FLNC and the development of high-risk dilated and arrhythmogenic cardiomyopathies...
December 6, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27900866/-the-benefit-of-magnetic-resonance-for-diagnosing-cardiomyopathy-and-myocarditis
#4
Michal Fikrle, Petr Kuchynka, Martin Mašek, Jana Podzimková, Jan Kuchař, Aleš Linhart, Tomáš Paleček
Magnetic resonance is becoming an increasingly used examination in cardiology, since it greatly improves the accuracy of diagnosing of many heart diseases. At present magnetic resonance is the gold standard in assessing the volumes of the heart chambers and the systolic function of both ventricles. The possibility of detecting tissue characteristics to refine the diagnostics of different types of myocardial pathology is of essential importance. The authors summarize in the article the present knowledge about the use of magnetic resonance of the heart in the field of myocardial disease, i...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27900527/should-all-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-undergo-epicardial-catheter-ablation
#5
Andreas Müssigbrodt, Elena Efimova, Helge Knopp, Livio Bertagnolli, Nikolaos Dagres, Sergio Richter, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
BACKGROUND: Epicardial radiofrequency catheter ablation is currently considered as the therapeutic option of choice in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and recurrent ventricular tachycardia (VT). We hypothesised that inducibility of VT may guide the ablation strategy and thereby affect long-term results. Additional epicardial ablation was only performed if VT were still inducible after thorough endocardial ablation. METHODS: The objective was to examine an inducibility-guided ablation approach by comparing the long-term results between endocardial and epi-endocardial radiofrequency catheter ablation of VT in a large cohort of patients with ARVD/C...
November 30, 2016: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/27871237/a-unique-association-of-arrhythmogenic-right-ventricular-dysplasia-and-acute-myocarditis-as-assessed-by-cardiac-mri-a-case-report
#6
Andrea Ponsiglione, Marta Puglia, Carmine Morisco, Luigi Barbuto, Antonio Rapacciuolo, Mario Santoro, Letizia Spinelli, Bruno Trimarco, Alberto Cuocolo, Massimo Imbriaco
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD), is a genetic disorder of the heart, which mainly involves the right ventricle. It is characterized by hypokinetic areas at the free wall of the right ventricle (RV) or both ventricles, where myocardium is replaced by fibrous or fatty tissue. ARVD is an important cause of ventricular arrhythmias in children and young adults. Although the transmission of the disease is based on hereditary, in young adults it may not show any symptoms...
November 21, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27864249/positive-predictive-value-of-cardiovascular-diagnoses-in-the-danish-national-patient-registry-a-validation-study
#7
Jens Sundbøll, Kasper Adelborg, Troels Munch, Trine Frøslev, Henrik Toft Sørensen, Hans Erik Bøtker, Morten Schmidt
OBJECTIVE: The majority of cardiovascular diagnoses in the Danish National Patient Registry (DNPR) remain to be validated despite extensive use in epidemiological research. We therefore examined the positive predictive value (PPV) of cardiovascular diagnoses in the DNPR. DESIGN: Population-based validation study. SETTING: 1 university hospital and 2 regional hospitals in the Central Denmark Region, 2010-2012. PARTICIPANTS: For each cardiovascular diagnosis, up to 100 patients from participating hospitals were randomly sampled during the study period using the DNPR...
November 18, 2016: BMJ Open
https://www.readbyqxmd.com/read/27860063/a-comparison-of-women-and-men-undergoing-catheter-ablation-for-sustained-monomorphic-ventricular-tachycardia
#8
Samuel H Baldinger, Saurabh Kumar, Jorge Romero, Akira Fujii, Laurence M Epstein, Gregory F Michaud, Roy John, Usha B Tedrow, William G Stevenson
INTRODUCTION: Women are underrepresented in clinical studies on catheter ablation (CA) for ventricular tachycardia (VT). The role of gender as a predictor for VT recurrence after CA is unclear and complication rates have not been compared in a large cohort. METHODS AND RESULTS: We analyzed data of consecutive patients undergoing CA for sustained monomorphic VT at our center between 2005 and 2015. A total of 948 patients underwent 1314 ablation procedures: 114 patients without SHD (48% female), 486 with coronary artery disease (CAD) (9% female), 301 with non-ischemic cardiomyopathy (NICM) (22% female), and 46 with arrhythmogenic right ventricular cardiomyopathy (ARVC) (17% female)...
November 18, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27858926/-heart-failure-role-of-cardiovascular-imaging
#9
Rienzi Díaz Navarro
The usefulness of echocardiography and the new noninvasive cardiac techniques in assessing heart failure is analyzed. The usefulness of non-invasive CT coronary angiography, as well as the growing applications of magnetic resonance imaging (MRI) in the study of ischemic heart disease, cardiomyopathy and arrhythmogenic right ventricular dysplasia is considered. For this puspose, some clinical cases are used. The combined use of these techniques, especially in patients in whom the etiology of heart failure is ischemic heart disease or cardiomyopathy is emphasized...
October 11, 2016: Medwave
https://www.readbyqxmd.com/read/27855809/arrhythmogenic-right-ventricular-cardiomyopathy-better-tools-for-detecting-early-disease-and-progression
#10
EDITORIAL
Francis E Marchlinski, Thor Edvardsen
No abstract text is available yet for this article.
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27855808/right-ventricular-imaging-and-computer-simulation-for-electromechanical-substrate-characterization-in-arrhythmogenic-right-ventricular-cardiomyopathy
#11
Thomas P Mast, Arco J Teske, John Walmsley, Jeroen F van der Heijden, René van Es, Frits W Prinzen, Tammo Delhaas, Toon A van Veen, Peter Loh, Pieter A Doevendans, Maarten J Cramer, Joost Lumens
BACKGROUND: Previous studies suggested that electrical abnormalities precede overt structural disease in arrhythmogenic right ventricular cardiomyopathy (ARVC). Abnormal RV deformation has been reported in early ARVC without structural abnormalities. The pathophysiological mechanisms underlying these abnormalities remain unknown. OBJECTIVES: The authors used imaging and computer simulation to differentiate electrical from mechanical tissue substrates among ARVC clinical stages...
November 15, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27834139/microrna-130a-regulation-of-desmocollin-2-in-a-novel-model-of-arrhythmogenic-cardiomyopathy
#12
Stefan R Mazurek, Tyler Calway, Cynthia Harmon, Priyanka Farrell, Gene H Kim
BACKGROUND: MicroRNAs are small noncoding RNA molecules that play a critical role in regulating physiological and disease processes. Recent studies have now recognized microRNAs as an important player in cardiac arrhythmogenesis. Molecular insight into arrhythmogenic cardiomyopathy (AC) has primarily focused on mutations in desmosome proteins. To our knowledge, models of AC due to microRNA dysregulation have not been reported. Previously, we reported on miR-130a mediated down-regulation of Connexin43...
November 9, 2016: MicroRNA
https://www.readbyqxmd.com/read/27828830/right-ventricular-arrhythmogenic-cardiomyopathy-genetic-and-mr-for-modern-clinical-diagnosis
#13
Maddalena Graziosi, Claudio Rapezzi
No abstract text is available yet for this article.
November 8, 2016: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/27825581/usefulness-of-combined-functional-assessment-by-cardiac-magnetic-resonance-and-tissue-characterization-versus-task-force-criteria-for-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy
#14
Giovanni Donato Aquaro, Andrea Barison, Giancarlo Todiere, Chrysanthos Grigoratos, Lamia Ait Ali, Gianluca Di Bella, Michele Emdin, Pierluigi Festa
Current task force criteria (TFC) of cardiac magnetic resonance (CMR) for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC/D) were generated by comparing probands (mean age of 44 years) to healthy participants of the multi-ethnic study of atherosclerosis (mean age of 60 years). These age differences may be a selection bias because right ventricular end-diastolic volume index decreases 4.6% per decade. Moreover, fat infiltration and late gadolinium enhancement were not included. We evaluated the diagnostic accuracy of TFC using the same methodology used by the task force but comparing probands and age- and gender-matched healthy controls and considering also other morphofunctional and tissue abnormalities detected by CMR...
December 1, 2016: American Journal of Cardiology
https://www.readbyqxmd.com/read/27805903/disease-implication-of-hyper-hippo-signalling
#15
Shu-Ping Wang, Lan-Hsin Wang
The Hippo signalling pathway regulates cellular proliferation, apoptosis and differentiation, thus exerting profound effects on cellular homeostasis. Inhibition of Hippo signalling has been frequently implicated in human cancers, indicating a well-known tumour suppressor function of the Hippo pathway. However, it is less certain whether and how hyperactivation of the Hippo pathway affects biological outcome in living cells. This review describes current knowledge of the regulatory mechanisms of the Hippo pathway, mainly focusing on hyperactivation of the Hippo signalling nexus...
October 2016: Open Biology
https://www.readbyqxmd.com/read/27802374/genetic-variations-leading-to-familial-dilated-cardiomyopathy
#16
REVIEW
Kae Won Cho, Jongsung Lee, Youngjo Kim
Cardiomyopathy is a major cause of death worldwide. Based on pathohistological abnormalities and clinical manifestation, cardiomyopathies are categorized into several groups: hypertrophic, dilated, restricted, arrhythmogenic right ventricular, and unclassified. Dilated cardiomyopathy, which is characterized by dilation of the left ventricle and systolic dysfunction, is the most severe and prevalent form of cardiomyopathy and usually requires heart transplantation. Its etiology remains unclear. Recent genetic studies of single gene mutations have provided significant insights into the complex processes of cardiac dysfunction...
October 2016: Molecules and Cells
https://www.readbyqxmd.com/read/27788994/incessant-left-ventricular-tachycardia-of-unusual-etiology
#17
Praloy Chakraborty, H S Isser, Sudheer Arava, Kausik Mandal
Apart from Coronary artery disease, left ventricular tachycardia may result from cardiac sarcoidosis, left ventricular tumor, chagas disease and idiopathic left ventricular tachycardia. We report a rare case of incessant left ventricular tachycardia resulting from left dominant arrhythmogenic cardiomyopathy.
May 2016: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/27781325/beyond-the-storm-comparison-of-clinical-factors-arrhythmogenic-substrate-and-catheter-ablation-outcomes-in-structural-heart-disease-patients-with-versus-those-without-a-history-of-ventricular-tachycardia-storm
#18
Saurabh Kumar, Akira Fujii, Sunil Kapur, Jorge Romero, Nishaki K Mehta, Shinichi Tanigawa, Laurence M Epstein, Bruce A Koplan, Gregory F Michaud, Roy M John, William G Stevenson, Usha B Tedrow
AIMS: Catheter ablation can be lifesaving in ventricular tachycardia (VT) storm, but the underlying substrate in patients with storm is not well characterized. We sought to compare the clinical factors, substrate and outcomes differences in patients with sustained monomorphic VT who present for catheter ablation with VT storm versus those with a non-storm presentation. METHODS: Consecutive ischemic (ICM; n = 554) or non-ischemic cardiomyopathy patients (NICM; n = 369) with a storm vs...
October 26, 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27771401/combination-of-ecg-and-echocardiography-for-identification-of-arrhythmic-events-in-early-arvc
#19
Ida S Leren, Jørg Saberniak, Trine F Haland, Thor Edvardsen, Kristina H Haugaa
OBJECTIVES: The aim of this study was to investigate early markers of arrhythmic events (AEs) and improve risk stratification in early arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND: AEs are frequent in patients with ARVC, but risk stratification in subjects with early ARVC is challenging. METHODS: Early ARVC disease was defined as possible or borderline ARVC diagnosis according to the ARVC Task Force Criteria 2010. We performed resting and signal averaged electrocardiogram (ECG)...
October 14, 2016: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/27769725/structural-and-molecular-pathology-of%C3%A2-the%C3%A2-atrium-in-boxer-arrhythmogenic-right-ventricular-cardiomyopathy
#20
J Vila, R Pariaut, N S Moïse, E M Oxford, P R Fox, C A Reynolds, C Saelinger
OBJECTIVE: To investigate the expression and distribution of desmosomal and gap junction proteins of the intercalated disc in the atria of boxers with arrhythmogenic right ventricular cardiomyopathy (ARVC). ANIMALS: Nineteen control dogs and 13 boxers with histopathologically confirmed ARVC. METHODS: Right and left atrial samples were examined using immunofluorescence and Western blots. The intercalated disc proteins investigated included total and phosphorylated connexin43 (Cx43 and pCx43), connexin45, connexin40, plakoglobin, plakophilin-2, desmoplakin, and N-cadherin...
October 18, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
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