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arrhythmogenic right ventricular cardiomyopathy

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https://www.readbyqxmd.com/read/28645928/molecular-mechanisms-in-cardiomyopathy
#1
REVIEW
Keith Dadson, Ludger Hauck, Filio Billia
Cardiomyopathies represent a heterogeneous group of diseases that negatively affect heart function. Primary cardiomyopathies specifically target the myocardium, and may arise from genetic [hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), mitochondrial cardiomyopathy] or genetic and acquired [dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM)] etiology. Modern genomics has identified mutations that are common in these populations, while in vitro and in vivo experimentation with these mutations have provided invaluable insight into the molecular mechanisms native to these diseases...
July 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28638576/a-classic-case-of-arrhythmogenic-right-ventricular-cardiomyopathy-arvc-and-literature-review
#2
Htun Latt, Thein Tun Aung, Chanwit Roongsritong, David Smith
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a relatively under-recognized hereditary cardiomyopathy. It is characterized pathologically by fibro-fatty infiltration of right ventricular (RV) myocardium and clinically by consequences of RV electrical instability. Timely intervention with device therapy and pharmacotherapy may help reduce the risk of arrhythmic events or sudden cardiac death. Here, we describe a classic case of a young adult with ARVC and a brief literature review. The patient presented with exertional palpitations and ARVC was suspected after his routine electrocardiogram (EKG) revealed symmetric T wave inversions and possible epsilon waves in right precordial leads...
March 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28633348/lifelong-arrhythmic-risk-stratification-in-arrhythmogenic-right-ventricular-cardiomyopathy-distribution-of-events-and-impact-of-periodical-reassessment
#3
Chiara Cappelletto, Davide Stolfo, Antonio De Luca, Bruno Pinamonti, Giulia Barbati, Alberto Pivetta, Marco Gobbo, Francesca Brun, Marco Merlo, Gianfranco Sinagra
Aims: The arrhythmic risk stratification of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains controversial. We evaluated the long-term distribution of life-threatening arrhythmic events assessing the impact of periodical risk reassessment. Methods and results: Ninety-eight ARVC patients with no previous major ventricular arrhythmias were retrospectively analysed. Patients were assessed at baseline, at 22 [inter-quartile range (IQR) 16-26], 49 (IQR 41-55) and 97 months (IQR 90-108)...
June 13, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28623036/-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-literature-review-and-case-report
#4
William Alejandro Camargo-Ariza, Silvia Juliana Galvis-Blanco, Tatiana Del Pilar Camacho-Enciso, Carlos Alberto Quiroz-Romero, Juan José Bermudez-Echeverry
Arrhythmogenic right ventricular cardiomyopathy/dysplasia is an inherited autosomal dominant pathology with an estimated prevalence of 1:2,500 to 1:5,000, being higher in males (3:1). It is characterized histologically by replacement of cardiomyocytes by fibro-adipose tissue, which predisposes to ventricular arrhythmias, right ventricular failure, and sudden cardiac death. The main goal of treatment is to reduce the risk of sudden death and improve the quality of life of patients. We present the case of a 23 year old woman whose clinical symptoms started with palpitations, chest pain with physical activity, syncope and headache 6 years ago during her first pregnancy...
June 13, 2017: Archivos de Cardiología de México
https://www.readbyqxmd.com/read/28596827/erratum-to-arrhythmogenic-right-ventricular-cardiomyopathy-secondary-to-adipose-infiltration-as-a-cause-of-episodic-collapse-in-a-horse
#5
Alexandra G Raftery, Nerea Cuesta-Garcia, Hal Thompson, David G M Sutton
[This corrects the article DOI: 10.1186/s13620-015-0052-3.].
2017: Irish Veterinary Journal
https://www.readbyqxmd.com/read/28595345/supraventricular-arrhythmias-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-associate-with-long-term-outcome-after-catheter-ablation-of-ventricular-tachycardias
#6
Andreas Müssigbrodt, Helge Knopp, Elena Efimova, Alexander Weber, Livio Bertagnolli, Sebastian Hilbert, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
Aims: This study aimed to assess the impact of supraventricular tachycardia (SVT) on long-term results of radiofrequency catheter ablation therapy of ventricular tachycardia (VT) in a large cohort of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Methods and results: Supraventricular tachycardia occurrence has been studied in patients from our ARVD/C registry (70 patients, 48 male, age 53.2 ± 14.0, 45 patients (64.3%) with previous VT ablation)...
June 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28588093/implantable-cardioverter-defibrillator-therapy-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-predictors-of-appropriate-therapy-outcomes-and-complications
#7
Gabriela M Orgeron, Cynthia A James, Anneline Te Riele, Crystal Tichnell, Brittney Murray, Aditya Bhonsale, Ihab R Kamel, Stephan L Zimmerman, Daniel P Judge, Jane Crosson, Harikrishna Tandri, Hugh Calkins
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by ventricular arrhythmias and sudden cardiac death. Once the diagnosis is established, risk stratification to determine whether implantable cardioverter-defibrillator (ICD) placement is warranted is critical. METHODS AND RESULTS: The cohort included 312 patients (163 men, age at presentation 33.6±13.9 years) with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy who received an ICD...
June 6, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28578331/whole-genome-sequence-identified-a-rare-homozygous-pathogenic-mutation-of-the-dsg2-gene-in-a-familial-arrhythmogenic-cardiomyopathy-involving-both-ventricles
#8
Yubi Lin, Qianhuan Zhang, Zhi An Zhong, Zhe Xu, Siqi He, Fang Rao, Yang Liu, Jiaojiao Tang, Feng Wang, Hui Liu, Jiajun Xie, Hongmei Wu, Shuxia Wang, Xin Li, Zhixin Shan, Chunyu Deng, Zili Liao, Hai Deng, Hongtao Liao, Yumei Xue, Wanqun Chen, Xianzhang Zhan, Bin Zhang, Shulin Wu
BACKGROUND: This study was designed to identify the pathogenic mutation in a Chinese family with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) using whole genome sequencing (WGS). METHODS AND RESULTS: Probands II:1 and II:2 underwent routine examinations for diagnosis. Genomic DNA was extracted from the peripheral blood of family members and analyzed using WGS. A total of 60,285 single-nucleotide polymorphisms (SNP) and 13,918 insertions/deletions (InDel) occurring in the exonic regions of genes and predisposing to cardiomyopathies and arrhythmias were identified...
June 3, 2017: Cardiology
https://www.readbyqxmd.com/read/28575228/right-ventricular-outflow-tract-dimensions-in-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-a-multicentre-study-comparing-echocardiography-and-cardiovascular-magnetic-resonance
#9
Alexander Gotschy, Ardan M Saguner, Markus Niemann, Sandra Hamada, Deniz Akdis, Ji-Na Yoon, Elena V Parmon, Victoria Delgado, Jeroen J Bax, Sebastian Kozerke, Corinna Brunckhorst, Firat Duru, Felix C Tanner, Robert Manka
Aims: Right ventricular outflow tract (RVOT) dilation is one of the echocardiographic criteria in the 2010 revised Task Force Criteria (TFC) of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). However, studies comparing cardiac magnetic resonance (CMR) and transthoracic echocardiography (TTE) suggest a lower diagnostic accuracy of TTE due to its operator dependence and limited reproducibility. The goal of this study was to compare the 2010 TFC measures of RVOT dilation with three alternative measures for improving the echocardiographic assessment of RVOT in patients with ARVC/D...
May 26, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28536891/myocardial-voltage-ratio-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#10
REVIEW
Andreas Müssigbrodt, Livio Bertagnolli, Elena Efimova, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Michael Döring, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
AIMS: This study aimed to analyze the influence of scar distribution between the endocardium and the epicardium in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). METHODS: Electroanatomical mapping data were derived from our ARVD/C registry. Myocardial voltage distribution between the endocardium and the epicardium was analyzed in 28 patients (18 men, 49.9 ± 13.0 years) with previous ventricular tachycardia (VT) ablation and complete right ventricular maps...
May 23, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28527814/desmoplakin-missense-and-non-missense-mutations-in-arrhythmogenic-right-ventricular-cardiomyopathy-genotype-phenotype-correlation
#11
Silvia Castelletti, Annina S Vischer, Petros Syrris, Lia Crotti, Carla Spazzolini, Alice Ghidoni, Gianfranco Parati, Sharon Jenkins, Maria-Christina Kotta, William J McKenna, Peter J Schwartz, Antonis Pantazis
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is traditionally considered as primarily affecting the right ventricle. Mutations in genes encoding desmosomal proteins account for 40-60% of cases. Genotype-phenotype correlations are scant and mostly non gene-specific. Accordingly, we assessed the genotype-phenotype correlation for desmoplakin (DSP) missense and non-missense mutations causing ARVC. METHODS AND RESULTS: We analyzed 27 ARVC patients carrying a missense or a non-missense DSP mutation, with complete clinical assessment...
May 10, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28523642/autosomal-recessive-nonsyndromic-arrhythmogenic-right-ventricular-cardiomyopathy-without-cutaneous-involvements-a-novel-mutation
#12
Mahdieh Soveizi, Bahareh Rabbani, Yousef Rezaei, Sedigheh Saedi, Nasim Najafi, Majid Maleki, Nejat Mahdieh
The arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic disease frequently associated with desmosomal mutations, mainly attributed to dominant mutations in the Plakophilin-2 (PKP2) gene. Naxos and Carvajal are the syndromic forms of ARVD/C due to recessive mutations. Herein, we report an autosomal recessive form of nonsyndromic ARVD/C caused by a mutation in the PKP2 gene. After examination and implementation of diagnostic modalities, the definite diagnosis of ARVD/C was confirmed by detection of ventricular tachycardia with a left bundle branch configuration and a superior axis, T-wave inversion in right precordial leads (i...
May 19, 2017: Annals of Human Genetics
https://www.readbyqxmd.com/read/28509892/arrhythmogenic-right-ventricular-dysplasia-an-under-recognized-form-of-inherited-cardiomyopathy
#13
George O Adesina, Shelly A Hall, Jose C Mendez, Susan M Joseph, Robert L Gottlieb, Parag P Kale, Amarinder S Bindra
We report a case of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD) in order to evaluate the course of an under-recognized form of cardiomyopathy with a vast array of clinical manifestations. The patient is a 49-year-old white woman transferred from an outside hospital due to dyspnea and persistent hypoxia. She had a pertinent family history that included a sister who died suddenly in her 30s from unexplained heart failure. Initial work-up for hypoxia was unrevealing. Transthoracic echocardiography revealed isolated right ventricular dysfunction with dilation and multiple trabeculations...
2017: Reviews in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28506445/comparison-of-features-of-fatal-versus-nonfatal-cardiac-arrest-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#14
Richa Gupta, Crystal Tichnell, Brittney Murray, Stefania Rizzo, Anneline Te Riele, Harikrishna Tandri, Daniel P Judge, Gaetano Thiene, Cristina Basso, Hugh Calkins, Cynthia A James
Once arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is diagnosed, the incidence of sudden cardiac death (SCD) is rare and prognosis is favorable, highlighting the value of early disease recognition. To inform strategies to diagnose ARVD/C before SCD, we sought to characterize clinical, genetic, and family history features of ARVD/C cases first recognized after SCD or resuscitated SCD (sudden cardiac arrest [SCA]). We identified 66 ARVD/C cases submitted to the Johns Hopkins ARVD/C Registry in whom disease was first recognized after SCD (n = 45) or SCA (n = 21) and compared their clinical, genetic, and demographic features with 352 patients (227 probands) diagnosed with ARVD/C by 2010 Task Force Criteria before any arrest...
April 13, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28496511/evaluation-of-coronary-arteries-in-non-ischemic-cardiomyopathies-a-case-report
#15
Farveh Vakilian, Mahmood Mohamadzadeh Shabestari, Ahmad Amin, Soheila Chamanian, Toktam Moghiman
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a congenital cardiac disease with myocardial involvement, most probably right ventricular (RV) dysfunction, accounting for 20% of sudden cardiac deaths. Characterized by the fibro-fatty infiltration of the RV free wall, ARVD/C presents in adolescents with ventricular arrhythmias and heart failure symptoms and as biventricular failure in adults. The coronary risk in these patients is not clear. We present an incidental finding: the left anterior descending artery cut-off in a middle-aged man with ARVD/C...
October 3, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28492156/electrical-storm-or-naxos-syndrome-in-an-adult-causing-recurrent-syncope
#16
Muhammad Furrakh Maqbool, Muhammad Sajid, Ahmed Noeman
Among the rare and well-known causes of sudden cardiac death by malignant arrthymias is a condition called arrhythmogenic right ventricular cardiomyopathy. It commonly presents with right ventricular dilatation, dysfunction and ventricular tachycardia of left bundle branch morphology due to fibro-fatty infiltration of right ventricle in second to fifth decade of life, making it an unrecognized and important cause of sudden cardiac death. Two rare variants of arrhythmogenic right ventricular cardiomyopathy are Carvajal syndrome and Naxos syndrome...
April 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28491739/incremental-value-of-electroanatomical-mapping-for-the-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy-in-a-patient-with-sustained-ventricular-tachycardia
#17
Simon A Castro, Rajeev K Pathak, Daniele Muser, Pasquale Santangeli, Anjali Owens, Francis Marchlinski, Fermin C Garcia
No abstract text is available yet for this article.
November 2016: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28491693/congenital-heart-disease-confounding-the-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy
#18
Omid Kiamanesh, Maryam Farhan, Shubhayan Sanatani, Lars Grosse-Wortmann, Walter Duncan, Robert M Hamilton
No abstract text is available yet for this article.
July 2016: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28488109/-epicardial-ablation-of-ventricular-tachycardias
#19
REVIEW
E Ene, P Halbfaß, K Nentwich, K Sonne, M Roos, S Fodor, L Lehmkuhl, F Gietzen, S Barth, K Hamm, T Deneke
Ventricular tachycardias (VT) in patients with structural heart diseases have predominantly a scar-associated reentry mechanism so that substrate-based ablation approaches also have to be used in nearly all procedures. In many VT cases-especially in nonischemic cardiomyopathy (NICM) and arrhythmogenic right ventricular cardiomyopathy-a critical epicardial substrate can be identified as an essential component of the reentry circuit so that for the ablation-based modification of the substrate in these cases an epicardial approach is necessary...
May 9, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28471438/electronic-health-record-phenotype-in-subjects-with-genetic-variants-associated-with-arrhythmogenic-right-ventricular-cardiomyopathy-a-study-of-30-716-subjects-with-exome-sequencing
#20
Christopher M Haggerty, Cynthia A James, Hugh Calkins, Crystal Tichnell, Joseph B Leader, Dustin N Hartzel, Christopher D Nevius, Sarah A Pendergrass, Thomas N Person, Marci Schwartz, Marylyn D Ritchie, David J Carey, David H Ledbetter, Marc S Williams, Frederick E Dewey, Alexander Lopez, John Penn, John D Overton, Jeffrey G Reid, Matthew Lebo, Heather Mason-Suares, Christina Austin-Tse, Heidi L Rehm, Brian P Delisle, Daniel J Makowski, Vishal C Mehra, Michael F Murray, Brandon K Fornwalt
PurposeArrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart disease. Clinical follow-up of incidental findings in ARVC-associated genes is recommended. We aimed to determine the prevalence of disease thus ascertained.MethodsIndividuals (n = 30,716) underwent exome sequencing. Variants in PKP2, DSG2, DSC2, DSP, JUP, TMEM43, or TGFβ3 that were database-listed as pathogenic or likely pathogenic were identified and evidence-reviewed. For subjects with putative loss-of-function (pLOF) variants or variants of uncertain significance (VUS), electronic health records (EHR) were reviewed for ARVC diagnosis, diagnostic criteria, and International Classification of Diseases (ICD-9) codes...
May 4, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
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