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arrhythmogenic right ventricular cardiomyopathy

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https://www.readbyqxmd.com/read/28912976/electromagnetic-interference-from-swimming-pool-generator-current-causing-inappropriate-icd-discharges
#1
Edward Samuel Roberto, Thein Tun Aung, Atif Hassan, Abdul Wase
Electromagnetic interference (EMI) includes any electromagnetic field signal that can be detected by device circuitry, with potentially serious consequences: incorrect sensing, pacing, device mode switching, and defibrillation. This is a unique case of extracardiac EMI by alternating current leakage from a submerged motor used to recycle chlorinated water, resulting in false rhythm detection and inappropriate ICD discharge. A 31-year-old female with arrhythmogenic right ventricular cardiomyopathy and Medtronic dual-chamber ICD placement presented after several inappropriate ICD shocks at the public swimming pool...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#2
REVIEW
Teresa M Lee, Daphne T Hsu, Paul Kantor, Jeffrey A Towbin, Stephanie M Ware, Steven D Colan, Wendy K Chung, John L Jefferies, Joseph W Rossano, Chesney D Castleberry, Linda J Addonizio, Ashwin K Lal, Jacqueline M Lamour, Erin M Miller, Philip T Thrush, Jason D Czachor, Hiedy Razoky, Ashley Hill, Steven E Lipshultz
Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28912183/arrhythmogenic-cardiomyopathy
#3
REVIEW
Domenico Corrado, Cristina Basso, Daniel P Judge
Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed. It is a cell-to-cell junction cardiomyopathy, typically caused by genetically determined abnormalities of cardiac desmosomes, which leads to detachment of myocytes and alteration of intracellular signal transduction...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28902650/a-rare-cause-of-cyanosis-and-hypoxia-that-should-not-be-forgotten-after-implantable-cardioverter-defibrillator-implantation
#4
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/28901675/sudden-cardiac-death-in-isolated-right-ventricular-hypertrabeculation-noncompaction-cardiomyopathy
#5
Soad Bekheit, Boutros Karam, Farshid Daneshvar, Julie Zaidan, Rabih Tabet, Jonathan Spagnola, James Lafferty
Hypertrabeculation/noncompaction of the myocardium is a rare disorder that involves most commonly the left ventricle of the heart and it has been recognized as a distinct cardiomyopathy by the World Health Organization. However, it is extremely rare for this condition to involve exclusively the right ventricle. We report the cases of three patients who presented with ventricular tachyarrhythmia and sudden cardiac death. They were found to have isolated right ventricular hypertrabeculation/noncompaction on echocardiography...
September 12, 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/28895994/asymptomatic-course-of-arrhythmogenic-right-ventricular-cardiomyopathy
#6
Agnieszka Sioma, Łukasz Mazurkiewicz, Magdalena Marczak, Ewa Kucharczyk-Petryka, Jacek Grzybowski
No abstract text is available yet for this article.
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28878587/arrhythmogenic-right-ventricular-cardiomyopathy-in-an-endurance-athlete-presenting-with-ventricular-tachycardia-and-normal-right-ventricular-function
#7
Jeffrey S Hedley, Ibhar Al Mheid, Zoubin Alikhani, Maria A Pernetz, Jonathan H Kim
Arrhythmogenic right ventricular cardiomyopathy, a genetically inherited disease that results in fibrofatty replacement of normal cardiac myocytes, has been associated with sudden cardiac death in athletes. Long-term participation in endurance exercise hastens the development of both the arrhythmic and structural arrhythmogenic right ventricular cardiomyopathy phenotypes. We describe the unusual case of a 34-year-old, symptomatic, female endurance athlete who had arrhythmogenic right ventricular cardiomyopathy in the presence of a structurally normal right ventricle...
August 2017: Texas Heart Institute Journal
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/28865556/normative-reference-values-of-right-heart-in-competitive-athletes-a-systematic-review-and-meta-analysis
#9
REVIEW
Flavio D'Ascenzi, Antonio Pelliccia, Marco Solari, Pietro Piu, Ferdinando Loiacono, Francesca Anselmi, Stefano Caselli, Marta Focardi, Marco Bonifazi, Sergio Mondillo
Training-induced right ventricular (RV) enlargement is frequent in athletes. Unfortunately, RV dilatation is also a common phenotypic expression and one of the diagnostic criteria of arrhythmogenic RV cardiomyopathy (ARVC). The current echocardiographic reference values derived from the general population can overestimate the presence of RV dilatation in athletes. We performed a meta-analysis of the literature to derive the proper reference values for assessing RV enlargement in competitive athletes. We conducted systematic review of English-language studies in the MEDLINE, Scopus, and Cochrane databases investigating RV size and function by echocardiography and by cardiac magnetic resonance (CMR) in competitive athletes...
September 2017: Journal of the American Society of Echocardiography
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
#10
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/28857927/arrhythmogenic-right-ventricular-cardiomyopathy-and-cardiac-microvascular-disease-a-rare-association-or-a-possible-link
#11
Giovanni B Forleo, Francesco Summaria, Domenico G Della Rocca, Chiara Lanzillo, Fabio Ticconi, Domenico Sergi, Luca Santini, Francesco Romeo
No abstract text is available yet for this article.
October 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28855434/isolated-late-activation-detected-by-magnetocardiography-predicts-future-lethal-ventricular-arrhythmic-events-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#12
Yoshitaka Kimura, Hiroshi Takaki, Yuko Y Inoue, Yasutaka Oguchi, Tomomi Nagayama, Takahiro Nakashima, Shoji Kawakami, Satoshi Nagase, Takashi Noda, Takeshi Aiba, Wataru Shimizu, Shiro Kamakura, Masaru Sugimachi, Satoshi Yasuda, Hiroaki Shimokawa, Kengo Kusano
BACKGROUND: Risk stratification of ventricular arrhythmias is vital to the optimal management in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We hypothesized that 64-channel magnetocardiography (MCG) would be useful to detect isolated late activation (ILA) by overcoming the limitations of conventional noninvasive predictors of ventricular tachyarrhythmias, including epsilon waves, late potential (LP), and right ventricular ejection fraction (RVEF), in ARVC patients...
August 30, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28843747/arrhythmogenic-right-ventricular-cardiomyopathy-arvc-in-a-young-female-athlete-at-36-weeks-gestation-a-case-report
#13
Hae Yoon Grace Choung, Monika Vyas, Daniel Jacoby, Brian West
A 26year old east African professional athlete presented to the obstetric clinic for a routine visit at 36 weeks gestation. She had a history of Right Ventricular Outflow Tract - Ventricular Tachycardia (RVOT-VT) with an episode of cardiac arrest in the past, and had been treated with ablation 4 years earlier. Her current visit was uneventful, her pregnancy progressing normally. Following the visit she went to a local restaurant where she suffered a cardiac arrest that was unresponsive to therapy. Chest compressions were continued from the time of her collapse until an emergency caesarian section was performed, delivering a healthy female infant...
July 25, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28818065/case-reports-of-two-pedigrees-with-recessive-arrhythmogenic-right-ventricular-cardiomyopathy-associated-with-homozygous-thr335ala-variant-in-dsg2
#14
Sami Qadri, Olli Anttonen, Juho Viikilä, Eija H Seppälä, Samuel Myllykangas, Tero-Pekka Alastalo, Miia Holmström, Tiina Heliö, Juha W Koskenvuo
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease, involving changes in ventricular myocardial tissue and leading to fatal arrhythmias. Mutations in desmosomal genes are thought to be the main cause of ARVC. However, the exact molecular genetic etiology of the disease still remains largely inconclusive, and this along with large variabilities in clinical manifestations complicate clinical diagnostics. CASE PRESENTATION: We report two families (n = 20) in which a desmoglein-2 (DSG2) missense variant c...
August 17, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28804588/first-reported-case-of-arrhythmogenic-right-ventricular-cardiomyopathy-in-oman
#15
Hatim Al Lawati, Humoud Al Dhuhli
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic disorder characterized by fatty degeneration of the right ventricular myocardium with variable involvement of the left ventricle. The condition is associated with exercise-mediated ventricular tachycardia and is one of the recognized causes of sudden cardiac death in the young and in athletes. Here, we report the first confirmed case of ARVC in Oman and present its electrocardiographic, echocardiographic features, and radiological findings on gated, contrast-enhanced cardiac computed tomography...
July 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28772045/translational-registry-for-cardiomyopathies-torch-rationale-and-first-results
#16
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28771538/a-novel-noninvasive-surface-ecg-analysis-using-interlead-qrs-dispersion-in-arrhythmogenic-right-ventricular-cardiomyopathy
#17
Wan-Hsin Hsieh, Chin-Yu Lin, Abigail Louise D Te, Men-Tzung Lo, Cheng-I Wu, Fa-Po Chung, Yi-Chung Chang, Shih-Lin Chang, Chen Lin, Li-Wei Lo, Yu-Feng Hu, Jo-Nan Liao, Yun-Yu Chen, Shih-Jie Jhuo, Sunu Budhi Raharjo, Yenn-Jiang Lin, Shih-Ann Chen
BACKGROUND: This study investigated the feasibility of using the precordial surface ECG lead interlead QRS dispersion (IQRSD) in the identification of abnormal ventricular substrate in arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: Seventy-one consecutive patients were enrolled and reclassified into 4 groups: definite ARVC with epicardial ablation (Group 1), ARVC with ventricular tachycardia (VT, Group 2), idiopathic right ventricular outflow tract VT without ARVC (Group 3), and controls without VT (Group 4)...
2017: PloS One
https://www.readbyqxmd.com/read/28767663/contribution-of-exome-sequencing-for-genetic-diagnostic-in-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia
#18
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/28765973/differential-expression-of-b-type-natriuretic-peptide-between-left-and-right-ventricles-with-particular-regard-to-sudden-cardiac-death
#19
Zhi-Peng Cao, Jia-Jia Xue, Yuan Zhang, Mei-Hui Tian, Ying Xiao, Yu-Qing Jia, Bao-Li Zhu
The aim of the present study was to investigate the differential expression of B‑type natriuretic peptide (BNP) between the left and right ventricle (RV) in sudden cardiac death (SCD). A total of 26 forensic autopsy cases of sudden death (survival time <30 min, postmortem interval <48 h or frozen within 6 h following death) in the present institute were examined. The cases consisted of acute ischemic heart disease (AIHD, n=15) with/without apparent myocardial necrosis as a sign of infarction (acute myocardial infarction, n=6; ischemic heart disease, IHD, n=9), and arrhythmogenic right ventricular cardiomyopathy (ARVC/D, n=5), in addition to traffic accidents and high falls without any pre existing heart disease as control (C, total n=6)...
October 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28750076/targeted-next-generation-sequencing-detects-novel-gene-phenotype-associations-and-expands-the-mutational-spectrum-in-cardiomyopathies
#20
Cinzia Forleo, Anna Maria D'Erchia, Sandro Sorrentino, Caterina Manzari, Matteo Chiara, Massimo Iacoviello, Andrea Igoren Guaricci, Delia De Santis, Rita Leonarda Musci, Antonino La Spada, Vito Marangelli, Graziano Pesole, Stefano Favale
Cardiomyopathies are a heterogeneous group of primary diseases of the myocardium, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC), with higher morbidity and mortality. These diseases are genetically diverse and associated with rare mutations in a large number of genes, many of which overlap among the phenotypes. To better investigate the genetic overlap between these three phenotypes and to identify new genotype-phenotype correlations, we designed a custom gene panel consisting of 115 genes known to be associated with cardiomyopathic phenotypes and channelopathies...
2017: PloS One
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