3,290 results match your criteria Arrhythmogenic Right Ventricular Cardiomyopathy


Sudden Death Related Cardiomyopathies - Arrhythmogenic Right Ventricular Cardiomyopathy, Arrhythmogenic Cardiomyopathy, and Exercise-Induced Cardiomyopathy.

Prog Cardiovasc Dis 2019 Apr 17. Epub 2019 Apr 17.

Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States of America; Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD, United States of America. Electronic address:

Sudden cardiac death (SCD) is a devastating possible outcome of all cardiomyopathies. The risk of SCD is increased in patients with structural heart disease and continues to increase as ventricular dysfunction worsens. There is however a subset of cardiomyopathy, so-called "arrhythmogenic cardiomyopathy" (ACM), that carries an inherent propensity for arrhythmia in all stages of the disease, even preceding ventricular dysfunction. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S00330620193007
Publisher Site
http://dx.doi.org/10.1016/j.pcad.2019.04.002DOI Listing
April 2019
1 Read

Association of Echocardiographic Parameters of Right Ventricular Remodeling and Myocardial Performance With Modified Task Force Criteria in Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy.

Circ Cardiovasc Imaging 2019 Apr;12(4):e007693

Department of Pediatrics, Labatt Family Heart Centre (G.E.P., L.G.-W., M.H., M.F., P.C., C.S., W.H., C.-P.S.F., C.M., L.M., R.H., M.K.F.), Hospital for Sick Children, University of Toronto, ON, Canada.

Background: The usefulness of echocardiographic indices, including those already used by modified Task Force Criteria (mTFC), and others such as strain imaging, to identify arrhythmogenic right ventricular cardiomyopathy (ARVC) in adolescence is not well established.

Methods: Echocardiograms from 120 adolescents investigated for ARVC (13±4 years) were retrospectively analyzed. According to the mTFC, patients were classified into definite (n=38), borderline (n=39), or possible (n=43) ARVC. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIRCIMAGING.118.007693DOI Listing

Challenge of Early Identification of Arrhythmogenic (Right Ventricular) Cardiomyopathy.

Circ Cardiovasc Imaging 2019 Apr;12(4):e009084

Division of Cardiology, Cardiothoracovascular Department, Azienda Sanitaria Universitaria Integrata of Trieste, Italy.

View Article

Download full-text PDF

Source
https://www.ahajournals.org/doi/10.1161/CIRCIMAGING.119.0090
Publisher Site
http://dx.doi.org/10.1161/CIRCIMAGING.119.009084DOI Listing
April 2019
1 Read

The Netherlands Arrhythmogenic Cardiomyopathy Registry: design and status update.

Neth Heart J 2019 Apr 17. Epub 2019 Apr 17.

Durrer Centre for Cardiovascular Research, Netherlands Heart Institute, Utrecht, The Netherlands.

Background: Clinical research on arrhythmogenic cardiomyopathy (ACM) is typically limited by small patient numbers, retrospective study designs, and inconsistent definitions.

Aim: To create a large national ACM patient cohort with a vast amount of uniformly collected high-quality data that is readily available for future research.

Methods: This is a multicentre, longitudinal, observational cohort study that includes (1) patients with a definite ACM diagnosis, (2) at-risk relatives of ACM patients, and (3) ACM-associated mutation carriers. Read More

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/s12471-019-1270-1
Publisher Site
http://dx.doi.org/10.1007/s12471-019-1270-1DOI Listing
April 2019
1 Read

Exercise-related sudden cardiac death of an American football player with arrhythmogenic right ventricular dysplasia/cardiomyopathy AND sarcoidosis.

Clin Case Rep 2019 Apr 19;7(4):686-688. Epub 2019 Feb 19.

Department of Electrophysiology, Heart Center University of Leipzig Leipzig Germany.

This case emphasizes the value of cardiac MRI and genetic testing in the early phase of ARVD/C. It also emphasizes the increased risk of SCD for patients with ARVD/C participating in competitive sports, even with immediate cardiopulmonary resuscitation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/ccr3.2071DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452462PMC

A case of desmoplakin mutation and delayed arrhythmogenic right ventricular cardiomyopathy/dysplasia after atrial septal defect closure.

J Cardiol Cases 2019 Apr 6;19(4):111-114. Epub 2019 Feb 6.

Department of Cardiology, Tokyo Women's Medical University, Tokyo, Japan.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a slow-developing cardiomyopathy characterized by ventricular arrhythmias and fibrofatty replacement of the right ventricular (RV) myocardium. Its clinical diagnosis is challenging because of its variable clinical presentation and low genetic penetrance. We describe the case of a 67-year-old man who was diagnosed as having ARVC/D with a desmoplakin mutation that appeared after occlusion of an atrial septal defect (ASD). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jccase.2018.09.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451054PMC
April 2019
1 Read

Patients with Isolated Focal Right Ventricular Dyskinetic Segments: Toward a Better Understanding of This Cohort.

J Cardiovasc Imaging 2019 Apr;27(2):93-101

Division of Cardiovascular Medicine, Clemenceau Medical Center, Beirut, Lebanon.

Background: The 2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) provided guidance for the classification of patients as definitive, borderline or possible ARVC. However, many patients with clinical suspicion for ARVC have isolated RV dyskinetic segments only and partly meet cardiac magnetic resonance (CMR) imaging criteria. This subgroup of patients and the implication of this imaging finding remain not well defined. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4250/jcvi.2019.27.e16DOI Listing
April 2019
1 Read

Identification of key pathways and genes in nasopharyngeal carcinoma using bioinformatics analysis.

Oncol Lett 2019 May 8;17(5):4683-4694. Epub 2019 Mar 8.

Jiangsu Cancer Hospital, Jiangsu Institute of Cancer Research, Nanjing Medical University Affiliated Cancer Hospital, Nanjing, Jiangsu 210000, P.R. China.

Nasopharyngeal carcinoma (NPC) is one of the most common malignancies in the head and neck. The aim of the current study was to identify the key pathways and genes involved in NPC through bioinformatics analysis and to identify potential molecular mechanisms underlying NPC proliferation and progression. Three gene expression profiles (GSE12452, GSE34573 and GSE64634) were downloaded from the Gene Expression Omnibus database. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3892/ol.2019.10133DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6447945PMC
May 2019
1 Read

Incidentally identified genetic variants in arrhythmogenic right ventricular cardiomyopathy-associated genes among children undergoing exome sequencing reflect healthy population variation.

Mol Genet Genomic Med 2019 Apr 15:e593. Epub 2019 Apr 15.

Department of Pediatrics, Section of Pediatric Cardiology, Baylor College of Medicine, Houston, Texas.

Background: With expanding use of clinical whole exome sequencing (WES), genetic variants of uncertain significance are increasingly identified. As pathologic mutations in genes associated with arrhythmogenic right ventricular cardiomyopathy (ARVC) carry a risk of sudden death, determining the diagnostic relevance of incidentally identified variants associated with these genes is critical.

Methods: WES variants from a large, predominantly pediatric cohort (N = 7,066 probands) were obtained for nine ARVC-associated genes (Baylor Miraca). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mgg3.593DOI Listing
April 2019
1 Read

Prediction of ventricular arrhythmia and sudden death in arrhythmogenic right ventricular cardiomyopathy.

Eur Heart J 2019 Apr 11. Epub 2019 Apr 11.

Section of Cardiovascular Diseases, Yale School of Medicine and YNHH Heart and Vascular Center, New Haven, CT, USA.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/eurheartj/ehz195DOI Listing
April 2019
1 Read

A case of arrhythmogenic right ventricular cardiomyopathy with biventricular involvement.

Monaldi Arch Chest Dis 2019 Mar 27;89(1). Epub 2019 Mar 27.

San Giovanni Addolorata Community Hospital, Cardiology and Rehabilitation Unit, Department of Medicine, Rome.

We reported a case of a young adult male aged 18 years admitted in our institution for syncope during a basketball match. No previous symptoms were reported. Electrocardiogram (ECG) showed T-wave inversion in the anterior leads and an incomplete right bundle branch block. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4081/monaldi.2019.1009DOI Listing
March 2019
1 Read

Arrhythmogenic right ventricular cardiomyopathy or athlete's heart? Challenges in assessment of right heart morphology and function.

Monaldi Arch Chest Dis 2019 Mar 27;89(1). Epub 2019 Mar 27.

Highly Specialized Rehabilitation Hospital Motta di Livenza, Cardiac Prevention and Rehabilitation Unit.

The incidence of sudden cardiac death (SCD) in young athletes varies among studies, due to the disagreement in the definitions and the lack of information in this field. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4081/monaldi.2019.1047DOI Listing

Transcriptome analysis to identify long non coding RNA (lncRNA) and characterize their functional role in back fat tissue of pig.

Gene 2019 Apr 5;703:71-82. Epub 2019 Apr 5.

Animal Genomics and Bioinformatics Division, National Institute of Animal Science, RDA, Wanju 55365, Republic of Korea. Electronic address:

Long non coding RNAs (lncRNA) have been previously found to be involved in important cellular activities like epigenetics, implantation, cell growth etc. in pigs. However, comprehensive analysis of lncRNA in back fat tissues at different developmental stages in pigs is still lacking. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.gene.2019.04.014DOI Listing
April 2019
2 Reads
2.138 Impact Factor

T wave inversion on the electrocardiogram: when to worry and when not to.

Authors:
Filippo Stazi

Eur Heart J Suppl 2019 Mar 29;21(Suppl B):B96-B97. Epub 2019 Mar 29.

Cardiology Unit II, San Giovanni-Addolorata Hospital, Rome, Italy.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/eurheartj/suz021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439922PMC
March 2019
1 Read

Epicardial ablation in genetic cardiomyopathies: a new frontier.

Eur Heart J Suppl 2019 Mar 29;21(Suppl B):B61-B66. Epub 2019 Mar 29.

Arrhythmology and Electrophysiology Department, IRCCS Policlinico San Donato, San Donato Milanese, Milano, Italy.

Brugada syndrome (BrS) and several cardiomyopathies, including dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction (LVNC), and hypertrophic cardiomyopathy (HCM), share common genetic mutations and are associated with an arrhythmogenic substrate (AS) and increased risk of sudden cardiac death (SCD) due to malignant ventricular arrhythmias. We report a family in which a SCN5A mutation was found in both a father and daughter who presented with different phenotypes: the father with LVNC and the daughter with BrS, suggesting SCN5A may be important in cases of overlap between BrS and these various other cardiomyopathies and arrhythmias. Additionally, we report a family in which a MYBPC3 mutation was found in a father, daughter, and son, but they also presented with different phenotypes: the father with HCM and the daughter and son with BrS, suggesting patients with cardiomyopathies or BrS exhibiting sarcomeric mutations may have common genetic pathways that ultimately diverge into different phenotypes. Read More

View Article

Download full-text PDF

Source
https://academic.oup.com/eurheartjsupp/article/21/Supplement
Publisher Site
http://dx.doi.org/10.1093/eurheartj/suz028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439905PMC
March 2019
3 Reads

Microvolt T-wave alternans complemented with electrophysiologic study for prediction of ventricular tachyarrhythmias in patients with arrhythmogenic right ventricular cardiomyopathy: a long-term follow-up study.

Chin Med J (Engl) 2019 Mar 29. Epub 2019 Mar 29.

Department of Cardiology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu 210029, China.

Background: The long-term predicted value of microvolt T-wave alternans (MTWA) for ventricular tachyarrhythmia in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) remains unclear. Our study explored the characteristics of MTWA and its prognostic value when combined with an electrophysiologic study (EPS) in ARVC patients.

Methods: All patients underwent noninvasive MTWA examination with modified moving average (MMA) analysis and an EPS. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/CM9.0000000000000239DOI Listing
March 2019
3 Reads

A novel genotype-based clinicopathology classification of arrhythmogenic cardiomyopathy provides novel insights into disease progression.

Eur Heart J 2019 Mar 3. Epub 2019 Mar 3.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167A Beilishi Road, Xi Cheng District, Beijing 100037, China.

Aims: Arrhythmogenic cardiomyopathy (AC) shows large heterogeneity in its clinical, genetic, and pathological presentation. This study aims to provide a comprehensive atlas of end-stage AC and illustrate the relationships among clinical characteristics, genotype, and pathological profiles of patients with this disease.

Methods And Results: We collected 60 explanted AC hearts and performed standard pathology examinations. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/eurheartj/ehz172DOI Listing
March 2019
3 Reads

The dividing line of arrhythmogenic right ventricular cardiomyopathy and Brugada Syndrome at epicardial ablation era: limited to or beyond RVOT?

Pacing Clin Electrophysiol 2019 Apr 3. Epub 2019 Apr 3.

Ankara City Hospital, Department of Cardiology, Health Sciences University, Ankara, Turkey.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/pace.13684DOI Listing
April 2019
2 Reads

Incorporation of desmocollin-2 into the plasma membrane requires N-glycosylation at multiple sites.

FEBS Open Bio 2019 Mar 28. Epub 2019 Mar 28.

Erich and Hanna Klessmann Institute for Cardiovascular Research & Development (EHKI), Heart and Diabetes Center NRW, University Hospital of the Ruhr-University Bochum, Oeynhausen, Germany.

Desmocollin-2 (DSC2) is a desmosomal protein of the cadherin family. Desmosomes are multiprotein complexes, which are involved in cell adhesion of cardiomyocytes and of keratinocytes. The molecular structure of the complete extracellular domain (ECD) of DSC2 was recently described, revealing three disulfide bridges, four N-glycosylation sites, and four O-mannosylation sites. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/2211-5463.12631DOI Listing
March 2019
2 Reads

Endo/epicardial ablation of ventricular arrhythmias with contact force-sensing catheters in arrhythmogenic right ventricular dysplasia/cardiomyopathy.

Anatol J Cardiol 2019 Mar;21(4):187-195

Department of Cardiology, Türkiye Yüksek İhtisas Training and Research Hospital; Ankara-Turkey.

Objective: To control ventricular arrhythmia in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), ablation may be required both from the endocardial and epicardial side. In this study, we analyzed the results of contact force-sensing (CFS) catheters in the endo/epicardial ablation of ventricular arrhythmias in ARVD/C.

Methods: We included 17 patients with ARVD/C, 5 of whom had premature ventricular contractions (PVC), and the rest of them were admitted with a ventricular tachycardia (VT) storm, between September 2014 and October 2016. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.14744/AnatolJCardiol.2018.58534DOI Listing
March 2019
1 Read

Comparison of the Arrhythmogenic Substrate between Men and Women with Nonischemic Cardiomyopathy.

Heart Rhythm 2019 Mar 27. Epub 2019 Mar 27.

Electrophysiology Section, Cardiovascular Division, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. Electronic address:

Background: Outcomes of ventricular tachycardia (VT) ablation in structural heart disease have been reported to differ by sex. Whether this is due to differences in the underlying arrhythmogenic substrates among patients with nonischemic cardiomyopathy (NICM) remains unclear.

Objectives: The aim of this study is to discover the characteristics of arrhythmogenic substrates between genders in nonischemic cardiomyopathy patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.hrthm.2019.03.024DOI Listing
March 2019
3 Reads

Letter by Cheung et al Regarding Article, "Concealed Arrhythmogenic Right Ventricular Cardiomyopathy in Sudden Unexplained Cardiac Death Events".

Circ Genom Precis Med 2019 Mar;12(3):e002447

Heart Rhythm Services, Division of Cardiology, University of British Columbia, Vancouver, Canada.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIRCGEN.118.002447DOI Listing
March 2019
1 Read

Response by Ingles and Semsarian to Letter Regarding Article, "Concealed Arrhythmogenic Right Ventricular Cardiomyopathy in Sudden Unexplained Cardiac Death Events".

Circ Genom Precis Med 2019 Mar;12(3):e002475

Agnes Ginges Centre for Molecular Cardiology at Centenary Institute and Faculty of Health and Medical Sciences, The University of Sydney, Australia. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIRCGEN.119.002475DOI Listing
March 2019
1 Read

A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy.

Eur Heart J 2019 Mar 27. Epub 2019 Mar 27.

Division of Cardiology, Department of Medicine, Johns Hopkins Hospital, Carnegie 568D, 600 N. Wolfe St. Baltimore, MD 21287, USA.

Aims: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients.

Methods And Results: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/eurheartj/ehz103DOI Listing
March 2019
7 Reads

Partial Pericardial Agenesis Mimicking Arrhythmogenic Right Ventricular Cardiomyopathy.

Clin J Sport Med 2019 Mar 8. Epub 2019 Mar 8.

Department of Cardiovascular, Neural and Metabolic Sciences, Istituto Auxologico Italiano, IRCCS, San Luca Hospital, Milan, Italy.

Absence of the pericardium is a rare congenital disease in which the fibroserum membrane covering the heart is partially or totally absent. It is characterized by few echocardiography (ECG) and imaging features that can mislead the diagnosis to an inherited cardiac disease, such as arrhythmogenic right ventricular cardiomyopathy. Although it has often a benign course, this congenital defect should be identified as in some cases herniation and strangulation can be life-threatening and cause sudden cardiac death. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/JSM.0000000000000733DOI Listing
March 2019
3 Reads

In vitro analysis of arrhythmogenic cardiomyopathy associated desmoglein-2 (DSG2) mutations reveals diverse glycosylation patterns.

J Mol Cell Cardiol 2019 Mar 15;129:303-313. Epub 2019 Mar 15.

Erich and Hanna Klessmann Institute for Cardiovascular Research and Development, Heart and Diabetes Center NRW, University Hospital of the Ruhr-University Bochum, Bad Oeynhausen, Germany.

Arrhythmogenic right ventricular cardiomyopathy is a heritable cardiac disease causing severe ventricular arrhythmias, heart failure and sudden cardiac death. It is mainly caused by mutations in genes encoding several structural proteins of the cardiac desmosomes including the DSG2 gene encoding the desmosomal cadherin desmoglein-2. Although the molecular structure of the extracellular domain of desmoglein-2 is known, it remains an open question, how mutations in DSG2 contribute to the pathogenesis of arrhythmogenic right ventricular cardiomyopathy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.yjmcc.2019.03.014DOI Listing
March 2019
1 Read

Bradyarrhythmias in Arrhythmogenic Right Ventricular Cardiomyopathy.

Am J Cardiol 2019 Feb 23. Epub 2019 Feb 23.

Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, PR China. Electronic address:

Less is known about bradyarrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC). This cross-sectional study aimed to assess the prevalence and clinical significance of bradyarrhythmias in ARVC. From May 1995 to December 2017, bradyarrhythmias including sick sinus syndrome, atrioventricular block, and intraventricular conductional block (ICB) were investigated in 522 ARVC patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.amjcard.2019.02.032DOI Listing
February 2019
3 Reads

The echocardiographic assessment of the right ventricle in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia compared with athletes and matched controls.

Echocardiography 2019 04 18;36(4):666-670. Epub 2019 Mar 18.

Harry Perkins Institute of Medical Research and Fiona Stanley Hospital, The University of Western Australia, Perth, Western Australia, Australia.

Background: There are discrepancies in the quantitative echocardiographic criteria for the right ventricle (RV) between the revised task force criteria (TFC) for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) and the guidelines for RV assessment endorsed by American Society of Echocardiography (ASE). Importantly, these criteria do not take into account potential adaptation of the RV to exercise. The goal of this study was to compare the revised TFC quantitative echocardiographic parameters in patients with ARVC/D, athletes and matched controls. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/echo.14308DOI Listing
April 2019
1 Read

Identification of aberrantly methylated differentially expressed genes in prostate carcinoma using integrated bioinformatics.

Cancer Cell Int 2019 5;19:51. Epub 2019 Mar 5.

1Department of Urology, Chinese PLA General Hospital, Beijing, China.

Background: Methylation plays a key role in the aetiology and pathogenesis of prostate cancer (PCa). This study aimed to identify aberrantly methylated differentially expressed genes (DEGs) and pathways in PCa and explore the underlying mechanisms of tumourigenesis.

Methods: Expression profile (GSE29079) and methylation profile (GSE76938) datasets were obtained from the Gene Expression Omnibus (GEO). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12935-019-0763-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402097PMC
March 2019
1 Read

Biventricular myocardial strain analysis using cardiac magnetic resonance feature tracking (CMR-FT) in patients with distinct types of right ventricular diseases comparing arrhythmogenic right ventricular cardiomyopathy (ARVC), right ventricular outflow-tract tachycardia (RVOT-VT), and Brugada syndrome (BrS).

Clin Res Cardiol 2019 Mar 13. Epub 2019 Mar 13.

Department of Clinical Radiology, University Hospital Muenster, Muenster, Germany.

Objectives: As underlying heart diseases of right ventricular tachyarrhythmias, ARVC causes wall-motion abnormalities based on fibrofatty myocardial degeneration, while RVOT-VT and BrS are thought to lack phenotypic MR characteristics. To examine whether cardiac magnetic resonance (CMR) feature tracking (FT) in addition to ARVC objectively facilitates detection of myocardial functional impairments in RVOT-VT and BrS.

Methods: Cine MR datasets of four retrospectively enrolled, age-matched study groups [n = 65; 16 ARVC, 26 RVOT-VT, 9 BrS, 14 healthy volunteers (HV)] were independently assessed by two distinctly experienced investigators regarding myocardial function using CMR-FT. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00392-019-01450-wDOI Listing
March 2019
3 Reads

Athletic Training and Arrhythmogenic Right Ventricular Cardiomyopathy.

Int J Sports Med 2019 Mar 13. Epub 2019 Mar 13.

Faculty of Medicine, University of Coimbra, Coimbra, Portugal.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and sudden death in the young and in competitive athletes. The deleterious role of exercise in the natural history of ARVC is clear. Even in the absence of a demonstrated arrhythmogenic substrate, family history or mutations of ARVC, intense physical exercise may in some individuals lead to the development of right ventricular dysfunction and arrhythmogenicity. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1055/a-0750-5848DOI Listing
March 2019
1 Read

The aberrant expression or disruption of desmocollin2 in human diseases.

Int J Biol Macromol 2019 Mar 6;131:378-386. Epub 2019 Mar 6.

Department of Pharmacology, Key Laboratory of Chemical Biology (Ministry of Education), School of Pharmaceutical Sciences, Shandong University, Jinan 250012, China. Electronic address:

The desmosome is a member of intercellular junctions that named 'anchoring junction', which contributes to the integrity and homeostasis of tissue structure and function of multicellular living organisms. As an important component of the desmosome and the most widely distributed isoform of desmocollins (DSCs), desmocollin2 (DSC2) has been demonstrated to be essential for the unity of epithelial cells, and served as a vital regulator in signaling processes such as epithelial morphogenesis, differentiation, wound healing, cell apoptosis, migration, and proliferation. Recent studies suggested that the aberrant expression or disruption of DSC2 might lead to some disorders, including heart disorders, certain cancers, and some other human diseases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijbiomac.2019.03.041DOI Listing
March 2019
2 Reads

Arrhythmogenic Ventricular Cardiomyopathy Associated With Fibromuscular Dysplasia of Ostial Right Main Coronary Artery.

Am J Forensic Med Pathol 2019 Mar 5. Epub 2019 Mar 5.

Medico-Legal and Pathological-Anatomical Department of Health Care Surveillance Authority, Košice, Slovak Republic.

In this article, we report the autopsy findings of a 23-year-old woman, who was found unconscious at home by her relatives. During the transportation to the hospital, the woman was handed over to the ambulance personnel, who were the first to provide cardiopulmonary resuscitation. In the hospital, after an hour-lasting asystole, the heart activity was restored. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/PAF.0000000000000469DOI Listing
March 2019
1 Read

Anti-arrhythmic therapy in patients with non-ischemic cardiomyopathy.

Pharmacol Res 2019 May 4;143:27-32. Epub 2019 Mar 4.

Division of Cardiology, McGill University Health Center, Montreal, Canada. Electronic address:

Implantable cardiac defibrillators (ICD) are the foundation of therapy for the prevention of sudden cardiac death. While ICDs prevent SCD, they do not prevent the occurrence of ventricular arrhythmias which are usually symptomatic. Though catheter ablation has been successful in substrate modification of ventricular tachycardia in patients with ischemic cardiomyopathy, there is much less evidence to support its use in non-ischemic cardiomyopathy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.phrs.2019.03.004DOI Listing
May 2019
8 Reads

[Canalopathies, arrhythmogenic right ventricular cardiomyopathy and artefacts].

Herzschrittmacherther Elektrophysiol 2019 Mar 1;30(1):51-71. Epub 2019 Mar 1.

Rhythmologie und Elektrophysiologie, Klinik für Kardiologie und Angiologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00399-019-0611-5DOI Listing
March 2019
1 Read

Sleep Fainting: A Neurocardiogenic Entity.

Cureus 2018 Dec 18;10(12):e3751. Epub 2018 Dec 18.

Cardiology, Aga Khan University Hospital, Karachi, PAK.

Fainting is a common clinical presentation, with vagally mediated (neurocardiogenic) causes being the most common for syncope presentation to the emergency room, and for hospital admissions. Classic teaching is that upright posture is a prerequisite for vagally mediated syncope (VMS) and that syncope in the supine position has more sinister causes. We present five patients, three males and two females, with a mean age of 44. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7759/cureus.3751DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6388817PMC
December 2018
2 Reads

Catheter Ablation of Ventricular Tachycardia in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Sequential Approach.

J Am Heart Assoc 2019 Mar;8(5):e010365

1 Department of Cardiology Asklepios Klinik St. Georg Hamburg Germany.

Background It has been suggested that endocardial and epicardial ablation of ventricular tachycardia ( VT ) improves outcome in arrhythmogenic right ventricular cardiomyopathy/dysplasia. We investigated our sequential approach for VT ablation in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia in a single center. Methods and Results We included 47 patients (44±16 years) with definite (81%) or borderline (19%) arrhythmogenic right ventricular cardiomyopathy/dysplasia between 1998 and 2016. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/JAHA.118.010365DOI Listing
March 2019
2 Reads

Intensive recreational athletes in the prospective multinational ICD Sports Safety Registry: Results from the European cohort.

Eur J Prev Cardiol 2019 Feb 27:2047487319834852. Epub 2019 Feb 27.

22 Yale University, New Haven, USA.

Background: In the ICD Sports Safety Registry, death, arrhythmia- or shock-related physical injury did not occur in athletes who continue competitive sports after implantable cardioverter-defibrillator (ICD) implantation. However, data from non-competitive ICD recipients is lacking. This report describes arrhythmic events and lead performance in intensive recreational athletes with ICDs enrolled in the European recreational arm of the Registry, and compares their outcome with those of the competitive athletes in the Registry. Read More

View Article

Download full-text PDF

Source
http://journals.sagepub.com/doi/10.1177/2047487319834852
Publisher Site
http://dx.doi.org/10.1177/2047487319834852DOI Listing
February 2019
10 Reads

Transcatheter left atrial decompression in patients with dilated cardiomyopathy: bridging to cardiac transplantation or recovery.

Cardiol Young 2019 Mar 26;29(3):355-362. Epub 2019 Feb 26.

1Hessen Pediatric Heart Center,Justus Liebig University Clinic Giessen,Giessen,Germany.

Background: Left atrial congestion results from backward failure in dilated cardiomyopathy. We aimed to evaluate feasibility and efficacy of percutaneous atrioseptostomy to create a restrictive atrial septum defect in management of dilated cardiomyopathy.Methods and resultsFrom June 2009 to December 2016, 27 interventions comprised left atria decompressions in 22 dilated cardiomyopathy patients; 9 females; age: 24 days to 36. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1017/S1047951118002433DOI Listing
March 2019
10 Reads
0.857 Impact Factor

Possibly Late-Onset Arrhythmogenic Right Ventricular Cardiomyopathy: Unique Triglyceride Deposition by Analysis of Lipid Contents.

Clin Med Insights Case Rep 2019 15;12:1179547619828715. Epub 2019 Feb 15.

Department of Pathology and Laboratory Medicine, Kanazawa Medical University, Uchinada, Japan.

We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man's death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/1179547619828715DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378422PMC
February 2019

Epsilon wave: A review of historical aspects.

Indian Pacing Electrophysiol J 2019 Mar - Apr;19(2):63-67. Epub 2019 Feb 21.

Sarver Heart Center, The University of Arizona Health Sciences Hospital, Tucson, AZ, USA.

The epsilon wave of the electrocardiogram (ECG) together with fragmented QRS (fQRS), the terminal conduction delay, incomplete right bundle branch block (IRBBB) and complete/advanced RBBB (CRBBB) of peripheral origin are part of a spectrum of ventricular depolarization abnormalities of arrhythmogenic cardiomyopathy(AC). Although the epsilon wave is considered a major diagnostic criterion for AC since 2010 (AC Task Force Criteria), its diagnostic value is limited because it is a sign of the later stage of the disease. It would be more appropriate to say that the epsilon wave is a "hallmark" of AC, but is of low diagnostic sensitivity. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ipej.2019.02.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6450822PMC
February 2019
8 Reads

Arrhythmogenic cardiomyopathies (ACs): diagnosis, risk stratification and management.

Heart 2019 Feb 21. Epub 2019 Feb 21.

UCL Institute of Cardiovascular Science, University College London, London, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/heartjnl-2017-311160DOI Listing
February 2019
1 Read

High risk of heart failure associated with desmoglein-2 mutations compared to plakophilin-2 mutations in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

Eur J Heart Fail 2019 Feb 21. Epub 2019 Feb 21.

Centre de Référence Pour les Maladies Cardiaques Héréditaires, APHP, Hôpital de la Pitié Salpêtrière, Paris, France.

Background: Previous studies suggested that genetic status affects the clinical course of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients. The aim of this study was to compare the outcome of desmoglein-2 (DSG2) mutation carriers to those who carry the plakophilin-2 (PKP2) mutation, the most common ARVC/D-associated gene.

Methods And Results: Consecutive ARVC/D patients carrying a pathogenic mutation in PKP2 or DSG2 were selected from a national ARVC/D registry. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/ejhf.1423DOI Listing
February 2019

Late presentation of arrhythmogenic right ventricular cardiomyopathy in an octogenarian associated with a pathogenic variant in the plakophilin 2 gene: a case report.

BMC Cardiovasc Disord 2019 Feb 19;19(1):41. Epub 2019 Feb 19.

Division of Cardiovascular Medicine, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma, Omiya, Saitama, 330-8503, Japan.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease characterized by fibrofatty replacement and ventricular arrhythmias. ARVC is believed to be a disease of the young, with most cases being diagnosed before the age of 40 years. We report here a case of newly diagnosed ARVC in an octogenarian associated with a pathogenic variant in the plakophilin 2 gene (PKP2). Read More

View Article

Download full-text PDF

Source
https://bmccardiovascdisord.biomedcentral.com/articles/10.11
Publisher Site
http://dx.doi.org/10.1186/s12872-019-1018-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6381698PMC
February 2019
6 Reads

How ARVC-Related Mutations Destabilize Desmoplakin: An MD Study.

Biophys J 2019 Mar 30;116(5):831-835. Epub 2019 Jan 30.

Interdisciplinary Center for Scientific Computing, Heidelberg University, Mathematikon, Heidelberg, Germany; Heidelberg Institute for Theoretical Studies, Heidelberg, Germany. Electronic address:

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a familial heart disease linked to mutations in several desmosomal proteins, but the specific effects of these mutations on the molecular level are poorly understood. Among the many documented ARVC-related genetic variants, a striking hotspot of nine mutations has been identified in the plakin domain of desmoplakin. This hotspot can be found at the meeting point of three different subdomains of desmoplakin: two spectrin repeats and a Src homology 3 domain. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.bpj.2019.01.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6403075PMC
March 2019
1 Read

Atrial dysplasia in the atria of humans without cardiovascular disease.

J Investig Med 2019 Feb 14. Epub 2019 Feb 14.

University Heart Center Zürich, Zürich, Switzerland.

Research on atrial histology of humans without cardiovascular disease is scarce. Therefore, our aim was to study human atrial histology in subjects without cardiovascular disease. Histology of the right atrium, left atrium or atrial septum was studied in eight patients (one newborn infant and seven adults) who died of a non-cardiac cause and who were not known to suffer from any cardiovascular pathology. Read More

View Article

Download full-text PDF

Source
http://jim.bmj.com/lookup/doi/10.1136/jim-2018-000916
Publisher Site
http://dx.doi.org/10.1136/jim-2018-000916DOI Listing
February 2019
6 Reads

Heart failure in patients with arrhythmogenic right ventricular cardiomyopathy: Genetic characteristics.

Int J Cardiol 2019 Jan 27. Epub 2019 Jan 27.

Cardiomyopathy Service, Royal Brompton Hospital, London, United Kingdom.

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disorder. The incidence of heart failure (HF) in ARVC has been reported at 5-13%. We aimed to define the genotype and disease progression of ARVC patients with HF. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijcard.2019.01.065DOI Listing
January 2019
1 Read