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Arrhythmogenic cardiomyopathy

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https://www.readbyqxmd.com/read/28431057/quantitative-analysis-of-pkp2-and-neighbouring-genes-in-a-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy-caused-by-heterozygous-pkp2-deletion
#1
Keiko Sonoda, Seiko Ohno, Sou Otuki, Koichi Kato, Nobue Yagihara, Hiroshi Watanabe, Takeru Makiyama, Tohru Minamino, Minoru Horie
Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease mainly caused by desmosome gene mutations. The genetic culprit, however, remains elusive in ∼50% of ARVC patients. One of the reasons for missing genetic abnormalities is the difficulty in detecting large deletions/duplications, which are called as copy number variation (CNV) by the Sanger sequencing method. This study aimed to identify CNVs in PKP2 and a part of other desmosome genes in ARVC patients. Methods and Results: The study cohort consisted of 71 ARVC probands who were diagnosed as definite or borderline cases based on 2010 Task Force Criteria...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28431055/electrocardiographic-differentiation-of-idiopathic-right-ventricular-outflow-tract-ectopy-from-early-arrhythmogenic-right-ventricular-cardiomyopathy
#2
Jan Novak, Alessandro Zorzi, Silvia Castelletti, Antonis Pantasis, Ilaria Rigato, Domenico Corrado, William Mckenna, Pier D Lambiase
Aims: The differentiation between idiopathic right ventricular outflow tract (RVOT) arrhythmias and early arrhythmogenic right ventricular cardiomyopathy (ARVC) can be challenging. We aimed to assess whether QRS morphological features and coupling interval of ventricular ectopic beats (VEBs) can improve differentiation between the two conditions. Methods and Results: Twenty desmosomal-gene mutation carriers (13 females, mean age 43 years) with no or mild ARVC phenotypic expression and 33 age- and sex-matched subjects with idiopathic RVOT arrhythmias were studied...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28431051/safety-long-term-outcomes-and-predictors-of-recurrence-after-first-line-combined-endoepicardial-ventricular-tachycardia-substrate-ablation-in-arrhythmogenic-cardiomyopathy-impact-of-arrhythmic-substrate-distribution-pattern-a-prospective-multicentre-study
#3
Antonio Berruezo, Juan Acosta, Juan Fernández-Armenta, Alonso Pedrote, Alberto Barrera, Eduardo Arana-Rueda, Andrés Ignacio Bodegas, Ignasi Anguera, Luis Tercedor, Diego Penela, David Andreu, Rosario Jesus Perea, Susana Prat-González, Lluis Mont
Background: First-line endoepicardial ventricular tachycardia (VT) ablation has been proposed for patients with arrhythmogenic cardiomyopathy (AC). This study reports procedural safety, outcomes, and predictors of recurrence. Methods and Results: Forty-one consecutive patients [12 with left ventricle (LV) involvement, 7 left-dominant] underwent first-line endoepicardial VT substrate ablation. Standard bipolar and unipolar thresholds were used to define low-voltage areas (LVA)...
April 1, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28419233/epicardial-ablation-may-not-be-necessary-in-all-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-and-frequent-ventricular-tachycardia-author-s-reply
#4
Antonio Berruezo, Juan Acosta, Juan Fernández-Armenta
No abstract text is available yet for this article.
April 16, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28402769/arrhythmogenic-right-ventricular-cardiomyopathy
#5
LETTER
Domenico Corrado, Mark S Link, Hugh Calkins
No abstract text is available yet for this article.
April 13, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28402404/long-term-outcome-of-surgical-cryoablation-for-refractory-ventricular-tachycardia-in-patients-with-non-ischemic-cardiomyopathy
#6
Jackson J Liang, Brian P Betensky, Daniele Muser, Erica S Zado, Elad Anter, Nimesh D Desai, David J Callans, Rajat Deo, David S Frankel, Mathew D Hutchinson, David Lin, Michael P Riley, Robert D Schaller, Gregory E Supple, Pasquale Santangeli, Michael A Acker, Joseph E Bavaria, Wilson Y Szeto, Prashanth Vallabhajosyula, Francis E Marchlinski, Sanjay Dixit
Aims: Limited data exist on the long-term outcome of patients (pts) with non-ischemic cardiomyopathy (NICM) and ventricular tachycardia (VT) refractory to conventional therapies undergoing surgical ablation (SA). We aimed to investigate the long-term survival and VT recurrence in NICM pts with VT refractory to radiofrequency catheter ablation (RFCA) who underwent SA. Methods and results: Consecutive pts with NICM and VT refractory to RFCA who underwent SA were included...
April 10, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28382086/catheter-ablation-of-ventricular-tachycardia-fibrillation-in-a-patient-with-right-ventricular-amyloidosis-with-initial-manifestations-mimicking-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#7
Fa-Po Chung, Yenn-Jiang Lin, Ling Kuo, Shih-Ann Chen
Differentiating arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) from other cardiomyopathies is clinically important but challenging. Although the modified Task Force Criteria can facilitate diagnosis of ARVD/C according to clinical manifestations, histopathological examination plays a pivotal role in excluding other diseases that can mimic ARVD/C. Here, we report a patient with amyloidosis that initially presented similarly to ARVD/C. The diagnosis was confirmed by endomyocardial biopsy, and catheter ablation eliminated the ventricular tachyarrhythmias through an epicardial approach...
March 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28373789/arrhythmogenic-right-ventricular-cardiomyopathy-with-biventricular-involvement-and-heart-failure-in-a-9-year-old-girl
#8
Fateh Ali Tipoo Sultan, Mehnaz Atiq Ahmed, Jamie Miller, Joseph B Selvanayagam
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is seldom recognized clinically in infancy or under the age of 10. We report a case of a 9-year-old girl with ARVC, who presented with signs and symptoms of heart failure and palpitations. Holter monitoring showed frequent premature ventricular beats and echocardiogram revealed dilated and dysfunctional right ventricle with normal tricuspid valve and no evidence of intracardiac shunt. Cardiac magnetic resonance showed classical features of ARVC with both ventricular involvements...
April 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28371837/characterization-of-the-arrhythmogenic-substrate-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-undergoing-ventricular-tachycardia-ablation
#9
Senthil Kirubakaran, Caterina Bisceglia, John Silberbauer, Teresa Oloriz, Giulia Santagostino, Miki Yamase, Giuseppe Maccabelli, Nicola Trevisi, Paolo Della Bella
Aims: Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is associated with ventricular arrhythmias, even without RV structural disease. We aimed to characterize the RV substrate using electroanatomical mapping and to define outcomes following ventricular tachycardia (VT) ablation in patients with and without RV structural abnormalities. Methods and results: Twenty-nine patients with definite or suspected ARVC undergoing VT ablation were classified as 'electrical' and 'structural' cardiomyopathy based on the absence or presence of major structural criteria...
March 20, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28365402/distinct-fibrosis-pattern-in-desmosomal-and-phospholamban-mutation-carriers-in-hereditary-cardiomyopathies
#10
Shahrzad Sepehrkhouy, Johannes M I H Gho, René van Es, Magdalena Harakalova, Nicolaas de Jonge, Dennis Dooijes, Jasper J van der Smagt, Marc P Buijsrogge, Richard N W Hauer, Roel Goldschmeding, Roel A de Weger, Folkert W Asselbergs, Aryan Vink
BACKGROUND: Desmosomal and phospholamban (PLN) mutations are associated with arrhythmogenic cardiomyopathy. Ultimately, most cardiomyopathy hearts develop significant cardiac fibrosis. OBJECTIVE: To compare the fibrosis patterns of desmosomal and p. Arg14del PLN associated cardiomyopathies with the pattern in hearts with other hereditary cardiomyopathies. METHODS: A mid-ventricular transversal slice was obtained from hearts of 30 patients with a cardiomyopathy with a known underlying mutation, and from 8 controls...
March 29, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28359489/palpitations-and-a-left-ventricular-mass-an-odd-presentation-of-left-dominant-arrhythmogenic-cardiomyopathy
#11
Sandor Toledo, Elizabeth Grigoryan, Jacobo Kirsch, Edward B Savage
A 20-year-old woman presented with palpitations. Echocardiography demonstrated a left ventricular mass involving the posterolateral apical wall and protruding into the ventricular cavity. Evaluation with magnetic resonance imaging (MRI) suggested fatty consistency with all edges well defined except the medial, which was ill defined, raising concern for an invasive liposarcoma. Open core needle biopsy demonstrated mature adipocytes infiltrating the myocardium with extensive interstitial fibrosis. The diagnosis was left-dominant arrhythmogenic cardiomyopathy...
April 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28342631/systematic-review-impact-of-the-new-task-force-criteria-in-the-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy
#12
Giuseppe Femia, Raymond W Sy, Rajesh Puranik
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disorder of cardiac desmosomes associated with ventricular arrhythmias and sudden cardiac death. The clinical diagnosis is problematic and relies on a complex criteria composed of clinical and non-clinical parameters. In 2010, the original 1994 Task Force Criteria (TFC) was revised with particular attention given to the imaging parameters. METHODS: Five retrospective studies compared the diagnostic concordance between the 1994 and 2010 TFC...
March 19, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28341588/targeted-next-generation-sequencing-of-51-genes-involved-in-primary-electrical-disease
#13
Dorien Proost, Johan Saenen, Geert Vandeweyer, Annelies Rotthier, Maaike Alaerts, Emeline M Van Craenenbroeck, Joachim Van Crombruggen, Geert Mortier, Wim Wuyts, Christiaan Vrints, Jurgen Del Favero, Bart Loeys, Lut Van Laer
Primary electrical disease (PED) is characterized by cardiac arrhythmias, which can lead to sudden cardiac death in the absence of detectable structural heart disease. PED encompasses a diversity of inherited syndromes, predominantly Brugada syndrome, early repolarization syndrome, long QT syndrome, short QT syndrome, arrhythmogenic right ventricular cardiomyopathy, and catecholaminergic polymorphic ventricular tachycardia. To overcome the diagnostic challenges imposed by the clinical and genetic heterogeneity of PED, we developed a targeted gene panel for next-generation sequencing of 51 PED genes...
May 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28339842/epicardial-ablation-may-not-be-necessary-in-all-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-and-frequent-ventricular-tachycardia
#14
A Müssigbrodt, E Efimova, H Knopp, L Bertagnolli, N Dagres, S Richter, D Husser, A Bollmann, G Hindricks, A Arya
No abstract text is available yet for this article.
February 28, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28339476/transgenic-mice-overexpressing-desmocollin-2-dsc2-develop-cardiomyopathy-associated-with-myocardial-inflammation-and-fibrotic-remodeling
#15
Andreas Brodehl, Darrell D Belke, Lauren Garnett, Kristina Martens, Nelly Abdelfatah, Marcela Rodriguez, Catherine Diao, Yong-Xiang Chen, Paul M K Gordon, Anders Nygren, Brenda Gerull
BACKGROUND: Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder leading to ventricular arrhythmias and heart failure, mainly as a result of mutations in cardiac desmosomal genes. Desmosomes are cell-cell junctions mediating adhesion of cardiomyocytes; however, the molecular and cellular mechanisms underlying the disease remain widely unknown. Desmocollin-2 is a desmosomal cadherin serving as an anchor molecule required to reconstitute homeostatic intercellular adhesion with desmoglein-2...
2017: PloS One
https://www.readbyqxmd.com/read/28336907/new-task-force-criteria-provide-evolution-in-diagnosis-of-arrhythmogenic-cardiomyopathy-in-patients-without-typical-progression-of-the-disease
#16
Mariana S Parahuleva, Jens Figiel, Holger Ahrens, Bernhard Schieffer, Dimitar Divchev, Ulrich Lüsebrink
BACKGROUND The original Task Force Criteria from 1994 for the clinical diagnosis of ARVC were highly specific and based on structural, histological, EKG, and familial features of disease. However, recommendations for clinical diagnosis and management of ARVC are sparse and lacked sensitivity for early disease. CASE REPORT Ventricular electrical instability and sudden cardiac death are the hallmarks of ARVC, and are often present before structural abnormalities. In this case report, we describe a patient who had detectable electrical abnormalities and structural changes that remained unchanged for over 10 years...
March 24, 2017: American Journal of Case Reports
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
#17
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
#18
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
#19
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
#20
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
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