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Arrhythmogenic right ventricular dysplasia

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https://www.readbyqxmd.com/read/29033518/a-rare-experience-of-infective-residual-sheath-in-the-wall-of-the-innominate-vein-after-extraction-of-implantable-defibrillators
#1
Yuan-Hao Liu, Wei-Shiang Lin, Yi-Chang Lin, Chih-Yuan Lin, Chien-Sung Tsai, Yi-Ting Tsai
BACKGROUND: Cardiac device-related infective endocarditis is an uncommon but potentially fatal complication. Therefore, cardiac devices should be removed as soon as a device-related infection is suspected. CASE REPORT: A 56-year-old male with a history of arrhythmogenic right ventricular dysplasia with implantable cardioverter-defibrillators (ICDs) 7 years earlier and re-implantation of ICDs due to dysfunction 18 months ago presented with erosion of the ICD pocket with Pseudomonas bacteremia...
July 2017: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/29029613/right-precordial-directed-electrocardiographical-markers-identify-arrhythmogenic-right-ventricular-cardiomyopathy-in-the-absence-of-conventional-depolarization-or-repolarization-abnormalities
#2
Daniel Cortez, Anneli Svensson, Jonas Carlson, Sharon Graw, Nandita Sharma, Francesca Brun, Anita Spezzacatene, Luisa Mestroni, Pyotr G Platonov
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries a risk of sudden death. We aimed to assess whether vectorcardiographic (VCG) parameters directed toward the right heart and a measured angle of the S-wave would help differentiate ARVD/C with otherwise normal electrocardiograms from controls. METHODS: Task Force 2010 definite ARVD/C criteria were met for all patients. Those who did not fulfill Task Force depolarization or repolarization criteria (-ECG) were compared with age and gender-matched control subjects...
October 13, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29016773/multichannel-electrocardiogram-diagnostics-for-the-diagnosis-of-arrhythmogenic-right-ventricular-dysplasia
#3
Ricards Marcinkevics, James O'Neill, Hannah Law, Eleftheria Pervolaraki, Andrew Hogarth, Craig Russell, Berthold Stegemann, Arun V Holden, Muzahir H Tayebjee
Aims: The identification of arrhythmogenic right ventricular dysplasia (ARVD) from 12-channel standard electrocardiogram (ECG) is challenging. High density ECG data may identify lead locations and criteria with a higher sensitivity. Methods and results: Eighty-channel ECG recording from patients diagnosed with ARVD and controls were quantified by magnitude and integral measures of QRS and T waves and by a measure (the average silhouette width) of differences in the shapes of the normalized ECG cycles...
August 29, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28979908/epicardial-ablation-of-ventricular-tachycardia-in-a-patient-with-arrhythmogenic-right-ventricular-dysplasia-after-failed-conventional-endocardial-ablation-a-case-for-remote-navigation-with-functional-image-integration
#4
Sabine Ernst, Karine Roy, Eric Lim, Glyn Thomas
Arrhythmogenic right ventricular dysplasia (ARVD) is an inheritable heart muscle disease that predominantly affects the right ventricle (RV) and predisposes to ventricular arrhythmias and sudden cardiac death (SCD)(1). The natural history is predominantly related to ventricular electric instability which may lead to arrhythmic SCD, mostly in young people and athletes(2,3), but may progress with significant RV muscle disease and left-ventricular (LV) involvement and can result in right or biventricular heart failure(4)...
December 30, 2016: Global Cardiology Science & Practice
https://www.readbyqxmd.com/read/28948146/cardiac-sarcoidosis-mimicking-arrhythmogenic-right-ventricular-dysplasia-in-a-patient-presenting-with-monomorphic-ventricular-tachycardia
#5
Ross Biggs, Brijesh Patel, Matthew W Martinez, Matthew McCambridge, Susan Kim, Norman Marcus
No abstract text is available yet for this article.
September 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28940781/right-ventricular-scar-related-ventricular-tachycardia-in-non-ischemic-cardiomyopathy-electrophysiological-characteristics-mapping-and-ablation-of-underlying-heart-disease
#6
Saurabh Kumar, Samuel H Baldinger, Sunil Kapur, Jorge Romero, Nishaki K Mehta, Saagar Mahida, Akira Fujii, Usha B Tedrow, William G Stevenson
BACKGROUND: Right ventricular (RV)-scar related ventricular tachycardia (VT) is often due to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) or cardiac sarcoidosis (CS), but some patients whose clinical course has not been described do not fulfill diagnostic criteria for these diseases. We sought to characterize the electrophysiologic substrate and catheter ablation outcomes of such patients, termed RV cardiomyopathy of unknown source (RCUS). METHODS AND RESULTS: Data of 100 consecutive patients who presented with RV cardiomyopathy and/or RV-related VT for ablation was reviewed (51 ARVC/D, 22 CS; 27 RCUS)...
September 21, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28902650/a-rare-cause-of-cyanosis-and-hypoxia-that-should-not-be-forgotten-after-implantable-cardioverter-defibrillator-implantation
#7
Uğur Canpolat, Nihan Bahadır, Levent Şahiner, Kudret Aytemir
Transvenous pacemaker or implantable cardioverter defibrillator (ICD) implantation procedures are usually performed under local anesthetic, and prilocaine is the most common agent to be used. The data regarding methemoglobinemia after cardiac device implantation are scarce. Thus, presently described is the case of a 47-year-old female patient with arrhythmogenic right ventricular cardiomyopathy/dysplasia who underwent ICD implantation for secondary prophylaxis and developed cyanosis as a result of prilocaine-associated methemoglobinemia...
September 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28874384/heart-failure-is-common-and-under-recognized-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia
#8
Nisha A Gilotra, Aditya Bhonsale, Cynthia A James, Anneline S J Te Riele, Brittney Murray, Crystal Tichnell, Abhishek Sawant, Chin Siang Ong, Daniel P Judge, Stuart D Russell, Hugh Calkins, Ryan J Tedford
BACKGROUND: Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D. METHODS AND RESULTS: Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. HF was present in 142 patients (49%): 113 had isolated RV involvement and 29 had evidence of LV dysfunction...
September 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28863780/feature-tracking-cmr-reveals-abnormal-strain-in-preclinical-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-a-multisoftware-feasibility-and-clinical-implementation-study
#9
Mimount Bourfiss, Davis M Vigneault, Mounes Aliyari Ghasebeh, Brittney Murray, Cynthia A James, Crystal Tichnell, Firdaus A Mohamed Hoesein, Stefan L Zimmerman, Ihab R Kamel, Hugh Calkins, Harikrishna Tandri, Birgitta K Velthuis, David A Bluemke, Anneline S J M Te Riele
BACKGROUND: Regional right ventricular (RV) dysfunction is the hallmark of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), but is currently only qualitatively evaluated in the clinical setting. Feature Tracking Cardiovascular Magnetic Resonance (FT-CMR) is a novel quantitative method that uses cine CMR to calculate strain values. However, most prior FT-CMR studies in ARVD/C have focused on global RV strain using different software methods, complicating implementation of FT-CMR in clinical practice...
September 1, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28810115/current-guidelines-on-syncope
#10
Maheedhar Gedela, Naveen Rajpurohit, Kashif A Shaikh, Muhammad Omar, Scott Pham
Syncope is a very commonly encountered clinical problem in general practice and in the emergency department. In the evaluation of syncope, it is important to identify the specific cause to determine the treatment, to estimate the precise risk to a patient, and to reduce recurrence. Sometimes, making a diagnosis of syncope is difficult, as different mechanisms may often coexist. Syncope causes a significant impact on quality of life due to associated risk of physical injury. In particular, syncope can be a precursor to sudden cardiac death in patients with underlying cardiac disease...
November 2016: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28797161/masked-inherited-primary-arrhythmia-syndromes-in-sudden-cardiac-death-patients-accompanied-by-coronary-vasospasm
#11
Ki Hong Lee, Hyung Wook Park, Jeong Nam Eun, Jeong Gwan Cho, Nam Sik Yoon, Mi Ran Kim, Yo Han Ku, Hyukjin Park, Seung Hun Lee, Jeong Han Kim, Min Chul Kim, Woo Jin Kim, Hyun Kuk Kim, Jae Yeong Cho, Keun-Ho Park, Doo Sun Sim, Hyun Ju Yoon, Kye Hun Kim, Young Joon Hong, Ju Han Kim, Youngkeun Ahn, Myung Ho Jeong, Jong Chun Park
BACKGROUND/AIMS: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. METHODS: We analyzed 74 consecutive patients (3...
September 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28767663/contribution-of-exome-sequencing-for-genetic-diagnostic-in-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia
#12
Joel Fedida, Veronique Fressart, Philippe Charron, Elodie Surget, Tiphaine Hery, Pascale Richard, Erwan Donal, Boris Keren, Guillaume Duthoit, Françoise Hidden-Lucet, Eric Villard, Estelle Gandjbakhch
BACKGROUND: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited cardiomyopathy mainly caused by heterozygous desmosomal gene mutations, the major gene being PKP2. The genetic cause remains unknown in ~50% of probands with routine desmosomal gene screening. The aim of this study was to assess the diagnostic accuracy of whole exome sequencing (WES) in ARVC/D with negative genetic testing. METHODS: WES was performed in 22 patients, all without a mutation identified in desmosomal genes...
2017: PloS One
https://www.readbyqxmd.com/read/28706583/cardiac-damage-in-athlete-s-heart-when-the-supernormal-heart-fails
#13
REVIEW
Andreina Carbone, Antonello D'Andrea, Lucia Riegler, Raffaella Scarafile, Enrica Pezzullo, Francesca Martone, Raffaella America, Biagio Liccardo, Maurizio Galderisi, Eduardo Bossone, Raffaele Calabrò
Intense exercise may cause heart remodeling to compensate increases in blood pressure or volume by increasing muscle mass. Cardiac changes do not involve only the left ventricle, but all heart chambers. Physiological cardiac modeling in athletes is associated with normal or enhanced cardiac function, but recent studies have documented decrements in left ventricular function during intense exercise and the release of cardiac markers of necrosis in athlete's blood of uncertain significance. Furthermore, cardiac remodeling may predispose athletes to heart disease and result in electrical remodeling, responsible for arrhythmias...
June 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28705047/galectin-3-correlates-with-arrhythmogenic-right-ventricular-cardiomyopathy-and-predicts-the-risk-of-ventricular-arrhythmias-in-patients-with-implantable-defibrillators
#14
Fahrettin Oz, Imran Onur, Ali Elitok, Evin Ademoglu, Ibrahim Altun, Ahmet Kaya Bilge, Kamil Adalet
Background Arrhythmogenic right ventricular dysplasia (ARVD) is a heritable disorder characterized by fibro-fatty replacement of right ventricular myocytes, increased risk of ventricular arrhythmias, and sudden cardiac death. Galectin-3 (GAL3) is known to play an important role in a number of fibrotic conditions, including cardiac fibrosis. Many studies have focused on the association between GAL3 levels and cardiac fibrosis in heart failure. However, the role of GAL3 in the pathogenesis of ARVD and ventricular arrhythmias has not yet been evaluated thoroughly...
July 14, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28650483/disease-model-discovery-from-3-328-gene-knockouts-by-the-international-mouse-phenotyping-consortium
#15
Terrence F Meehan, Nathalie Conte, David B West, Julius O Jacobsen, Jeremy Mason, Jonathan Warren, Chao-Kung Chen, Ilinca Tudose, Mike Relac, Peter Matthews, Natasha Karp, Luis Santos, Tanja Fiegel, Natalie Ring, Henrik Westerberg, Simon Greenaway, Duncan Sneddon, Hugh Morgan, Gemma F Codner, Michelle E Stewart, James Brown, Neil Horner, Melissa Haendel, Nicole Washington, Christopher J Mungall, Corey L Reynolds, Juan Gallegos, Valerie Gailus-Durner, Tania Sorg, Guillaume Pavlovic, Lynette R Bower, Mark Moore, Iva Morse, Xiang Gao, Glauco P Tocchini-Valentini, Yuichi Obata, Soo Young Cho, Je Kyung Seong, John Seavitt, Arthur L Beaudet, Mary E Dickinson, Yann Herault, Wolfgang Wurst, Martin Hrabe de Angelis, K C Kent Lloyd, Ann M Flenniken, Lauryl M J Nutter, Susan Newbigging, Colin McKerlie, Monica J Justice, Stephen A Murray, Karen L Svenson, Robert E Braun, Jacqueline K White, Allan Bradley, Paul Flicek, Sara Wells, William C Skarnes, David J Adams, Helen Parkinson, Ann-Marie Mallon, Steve D M Brown, Damian Smedley
Although next-generation sequencing has revolutionized the ability to associate variants with human diseases, diagnostic rates and development of new therapies are still limited by a lack of knowledge of the functions and pathobiological mechanisms of most genes. To address this challenge, the International Mouse Phenotyping Consortium is creating a genome- and phenome-wide catalog of gene function by characterizing new knockout-mouse strains across diverse biological systems through a broad set of standardized phenotyping tests...
August 2017: Nature Genetics
https://www.readbyqxmd.com/read/28645928/molecular-mechanisms-in-cardiomyopathy
#16
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/28623036/-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-literature-review-and-case-report
#17
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 disease, with an estimated prevalence of 1:2,500 to 1:5,000, being higher in males (3:1). It is characterised histologically by the substitution of cardiomyocytes for fibrous-adipose tissue, which predisposes to ventricular arrhythmias, right ventricular failure, and sudden cardiac death. The main aim of treatment is to reduce the risk of sudden death and improve the quality of life of patients. The case is presented 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/28595345/supraventricular-arrhythmias-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-associate-with-long-term-outcome-after-catheter-ablation-of-ventricular-tachycardias
#18
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
#19
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
#20
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...
2017: Cardiology
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