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Arrhythmogenic cardiomyopathy

Tomofumi Nakamura, Shinichi Tanigawa, Benjamin Schaeffer, Rahul G Muthalaly, Ammar M Killu, Usha B Tedrow, Roy M John, Michael O Sweeney, William G Stevenson
No abstract text is available yet for this article.
June 2018: HeartRhythm Case Reports
Antonio Berruezo, Elena Efimova, Juan Acosta, Beatriz Jáuregui
No abstract text is available yet for this article.
June 2018: HeartRhythm Case Reports
Amr Idris, Syed Raza Shah, Ki Park
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiovascular disease that predisposes to ventricular arrhythmias potentially leading to sudden cardiac death (SCD). ARVC varies considerably with multiple clinical presentations, ranging from no symptoms to cardiac arrhythmias to SCD. ARVC prevalence is not well known, but the estimated prevalence in the general population is 1:5000. Diagnosis of ARVC can be made by using the Revised European Society of Cardiology criteria for ARVC that includes ventricular structural and functional changes, ECG abnormalities, arrhythmias, family and genetic factors...
2018: Journal of Community Hospital Internal Medicine Perspectives
M Juhani Junttila, Lauri Holmström, Katri Pylkäs, Tuomo Mantere, Kari Kaikkonen, Katja Porvari, Marja-Leena Kortelainen, Lasse Pakanen, Risto Kerkelä, Robert J Myerburg, Heikki V Huikuri
BACKGROUND: Myocardial fibrosis is a common postmortem finding among young individuals with sudden cardiac death. Because there is no known single cause, we tested the hypothesis that some cases of myocardial fibrosis in the absence of identifiable causes (primary myocardial fibrosis [PMF]) are associated with genetic variants. METHODS: Tissue was obtained at autopsy from 4031 consecutive individuals with sudden cardiac death in Northern Finland, among whom PMF was the only structural finding in 145 subjects with sudden cardiac death...
June 19, 2018: Circulation
Marina Cerrone
No abstract text is available yet for this article.
June 16, 2018: Journal of the American Heart Association
Weijia Wang, Gabriela Orgeron, Crystal Tichnell, Brittney Murray, Jane Crosson, Oliver Monfredi, Julia Cadrin-Tourigny, Harikrishna Tandri, Hugh Calkins, Cynthia A James
BACKGROUND: Prior studies have shown a close link between exercise and development of arrhythmogenic right ventricular cardiomyopathy. How much exercise restriction reduces ventricular arrhythmia (VA), how genotype modifies its benefit, and whether it reduces risk sufficiently to defer implantable cardioverter-defibrillator (ICD) placement in arrhythmogenic right ventricular cardiomyopathy are unknown. METHODS AND RESULTS: We interviewed 129 arrhythmogenic right ventricular cardiomyopathy patients (age: 34...
June 16, 2018: Journal of the American Heart Association
Ray E Hershberger, Michael M Givertz, Carolyn Y Ho, Daniel P Judge, Paul F Kantor, Kim L McBride, Ana Morales, Matthew R G Taylor, Matteo Vatta, Stephanie M Ware
PURPOSE: The purpose of this document is to provide updated guidance for the genetic evaluation of cardiomyopathy and for an approach to manage secondary findings from cardiomyopathy genes. The genetic bases of the primary cardiomyopathies (dilated, hypertrophic, arrhythmogenic right ventricular, and restrictive) have been established, and each is medically actionable; in most cases established treatments or interventions are available to improve survival, reduce morbidity, and enhance quality of life...
June 14, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Lingmin Wu, Jingru Bao, Erpeng Liang, Siyang Fan, Lihui Zheng, Zhongpeng Du, Gang Chen, Ligang Ding, Shu Zhang, Yan Yao
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardium disorder that predominantly affects the ventricle. Little is known about atrial involvement. This study aimed to assess atrial involvement, especially the role of genotype on atrium in ARVC. METHODS: The incidence, characterization and predictors of atrial involvement were investigated. Nine known ARVC-causing genes were screened and the correlation between genotype and atrial involvement was assessed...
June 13, 2018: Journal of Cardiovascular Electrophysiology
Weijia Wang, Julia Cadrin Tourigny, Aditya Bhonsale, Crystal Tichnell, Brittney Murray, Oliver Monfredi, Jonathan Chrispin, Jane Crosson, Harikrishna Tandri, Cynthia A James, Hugh Calkins
BACKGROUND: Implantable defibrillators (ICD) are an important therapy for arrhythmogenic right ventricular cardiomyopathy (ARVC) patients at high risk of sudden death. Given the high appropriate ICD therapy rate, some have argued that the mere act of implanting an ICD inflates the malignant arrhythmia rate in ARVC. OBJECTIVE: To report the arrhythmic course of ARVC patients without ICDs at the fulfillment of the 2010 Task Force Criteria and explore predictors of malignant ventricular arrhythmias...
June 12, 2018: Journal of Cardiovascular Electrophysiology
Duarte Martins, Caroline Ovaert, Diala Khraiche, Nathalie Boddaert, Damien Bonnet, Francesca Raimondi
INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease with an elusive association with myocardial inflammation. A myocarditis phenotype has been well established without systematic evidence of a viral trigger. We intend to study the relationship between myocardial inflammation detected by Cardiac magnetic resonance (CMR) and ARVC in a paediatric population. METHODS: Retrospective case series of all patients <18 years admitted to two CMR units for clinical suspicion of myocarditis from March 2012 to June 2017 who had genetic testing for inherited cardiomyopathies including analysis for known ARVC genes...
May 31, 2018: International Journal of Cardiology
John R Giudicessi, Michael J Ackerman
BACKGROUND: Sudden cardiac arrest (SCA) may be the sentinel expression of a sudden cardiac death-predisposing genetic heart disease (GHD). Although shown to underlie many unexplained SCAs in the young, the contribution of GHDs to sentinel SCA has never been quantified across the age spectrum. Thus, we sought to determine the contribution of GHDs in single-center referral cohort of non-ischemic SCA survivors. METHODS AND RESULTS: Retrospective analysis of 3037 patients was used to identify all individuals who experienced a sentinel event of SCA...
May 30, 2018: International Journal of Cardiology
Steven D Hicks, Paige Jacob, Frank A Middleton, Omar Perez, Zofia Gagnon
Micro-ribonucleic acids (miRNAs) mediate adaptive responses to exercise and may serve as biomarkers of exercise intensity/capacity. Expression of miRNAs is altered in skeletal muscle, plasma, and saliva following exertion. Females display unique physiologic responses to endurance exercise, and miRNAs respond to pathologic states in sex-specific patterns. However sex-specific miRNA responses to exercise remain unexplored. This study utilized high-throughput RNA sequencing to measure changes in salivary RNA expression among 25 collegiate runners following a single long-distance run...
June 8, 2018: Physiological Genomics
Josep Brugada, Gerhard Hindricks
No abstract text is available yet for this article.
June 1, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
C A Martin, M Orini, N T Srinivasan, J Bhar-Amato, S Honarbakhsh, A W Chow, M D Lowe, R Ben-Simon, P M Elliott, P Taggart, P D Lambiase
BACKGROUND: The re-entry vulnerability index (RVI) is a recently proposed activation-repolarization metric designed to quantify tissue susceptibility to re-entry. This study aimed to test feasibility of an RVI-based algorithm to predict the earliest endocardial activation site of ventricular tachycardia (VT) during electrophysiological studies and occurrence of haemodynamically significant ventricular arrhythmias in follow-up. METHODS: Patients with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) (n = 11), Brugada Syndrome (BrS) (n = 13) and focal RV outflow tract VT (n = 9) underwent programmed stimulation with unipolar electrograms recorded from a non-contact array in the RV...
May 20, 2018: International Journal of Cardiology
David Prior
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a recognised cause of sudden cardiac death during exercise in young athletes. Competitive exercise is also known to accelerate progression of ARVC and exercise restriction is an important part of disease management. Regular endurance training can induce physiological changes detectable on electrocardiography and imaging which may overlap with pathological findings caused by ARVC, thus making differentiation of athlete's heart from ARVC difficult in some cases...
May 19, 2018: Heart, Lung & Circulation
J S Foo, C H Koh, A Sahlén, H C Tang, C P Lim
Congenital absence of pericardium is a rare condition with electrocardiogram, chest X-ray, and echocardiographic findings which may mimic those of other cardiac conditions. We present a case of a 19-year-old asymptomatic female with incidental cardiomegaly on chest X-ray and electrocardiographic and echocardiographic changes, which meet the revised task force criteria for definite arrhythmogenic right ventricular cardiomyopathy but subsequently confirmed to have congenital partial absence of pericardium on cardiac magnetic resonance imaging...
2018: Case Reports in Medicine
Ibrahim El-Battrawy, Martin Borggrefe, Siegfried Lang, Xiaobo Zhou, Ibrahim Akin
No abstract text is available yet for this article.
May 28, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
A Denis, T Pambrun, R Martin, N Derval, F Sacher
No abstract text is available yet for this article.
May 28, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Ozcan Ozeke, Serkan Cay, Firat Ozcan, Serkan Topaloglu, Dursun Aras
No abstract text is available yet for this article.
May 28, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Leonardo Calò, Annamaria Martino, Emilia Goanta, Yasuo Okumura, Ermenegildo de Ruvo
Right bundle branch block is relatively frequent in the general population and is generally considered benign in healthy individuals. However, it has been associated with increased cardiovascular risk and all-cause mortality in various cohort studies. Indeed, the presence of right bundle branch block in subjects affected by cardiomyopathies or channelopathies is not rare and may conceal underlying electrocardiographic changes, thus making the diagnosis of such conditions more challenging. The purpose of this review is to analyze the prevalence and clinical significance of right bundle branch block in subjects affected by Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy...
May 26, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
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