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Arrhythmogenic cardiomyopathy

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https://www.readbyqxmd.com/read/28215569/cardiac-phenotype-and-long-term-prognosis-of-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-patients-with-late-presentation
#1
Aditya Bhonsale, Anneline S J M Te Riele, Abhishek C Sawant, Judith A Groeneweg, Cynthia A James, Brittney Murray, Crystal Tichnell, Thomas P Mast, Michelle J van der Pols, Maarten J M Cramer, Dennis Dooijes, Jeroen F van der Heijden, Harikrishna Tandri, J Peter van Tintelen, Daniel P Judge, Richard N W Hauer, Hugh Calkins
BACKGROUND: The clinical profile of Arrhythmogenic Right Ventricular Cardiomyopathy/ Dysplasia patients with late presentation (ARVC/D) is unknown. OBJECTIVE: To characterize the genotype, cardiac phenotype and long term outcomes of ARVC/D patients with late presentation (age≥ 50 years at diagnosis) METHODS: 502 patients with ARVC/D diagnosis from Johns Hopkins and Utrecht registries were studied and long term clinical outcomes ascertained. RESULTS: Late presentation was seen in 104 (21%) patients (38% PKP2 carriers) with 3% ≥ 65 years at diagnosis...
February 12, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28185710/my-approach-to-the-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy-arvc
#2
Angeliki Asimaki
No abstract text is available yet for this article.
November 2, 2016: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28183531/cardiomyocyte-hypertrophy-in-arrhythmogenic-cardiomyopathy
#3
Mustafa Gerçek, Muhammed Gerçek, Sebastian Kant, Sakine Simsekyilmaz, Astrid Kassner, Hendrik Milting, Elisa A Liehn, Rudolf E Leube, Claudia A Krusche
Arrhythmogenic cardiomyopathy (AC) is a hereditary disease leading to sudden cardiac death or heart failure. AC pathology is characterized by cardiomyocyte loss and replacement fibrosis. Our goal was to determine whether cardiomyocytes respond to AC progression by pathological hypertrophy. To this end, we examined tissue samples from AC patients with end-stage heart failure and tissue samples that were collected at different disease stages from desmoglein 2-mutant mice, a well characterized AC model. We find that cardiomyocyte diameters are significantly increased in right ventricles of AC patients...
February 6, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28167090/ablation-of-ventricular-tachycardia-in-arrhythmogenic-right-ventricular-dysplasia
#4
REVIEW
Rajeev K Pathak, Fermin C Garcia
Endocardial and epicardial electroanatomical mapping and ablation is a safe and effective therapy in the treatment of right ventricle arrhythmias occurring in the setting of arrhythmogenic right ventricular cardiomyopathy (ARVD). Careful mapping and ablation plans must be tailored for each patient based on comorbidities and ventricular tachycardia morphologies. This review focuses on the catheter ablation for ventricular arrhythmias in patients with ARVD.
March 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28167088/substrate-ablation-of-ventricular-tachycardia-late-potentials-scar-dechanneling-local-abnormal-ventricular-activities-core-isolation-and-homogenization
#5
REVIEW
David F Briceño, Jorge Romero, Carola Gianni, Sanghamitra Mohanty, Pedro A Villablanca, Andrea Natale, Luigi Di Biase
Ventricular arrhythmias are a frequent cause of mortality in patients with ischemic cardiomyopathy and nonischemic cardiomyopathy. Scar-related reentry represents the most common arrhythmia substrate in patients with recurrent episodes of sustained ventricular tachycardia (VT). Initial mapping of scar-related VT circuits is focused on identifying arrhythmogenic tissue. The substrate-based strategies include targeting late potentials, scar dechanneling, local abnormal ventricular activities, core isolation, and homogenization of the scar...
March 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28153106/isolated-subepicardial-right-ventricular-outflow-tract-scar-in-athletes-with-ventricular%C3%A2-tachycardia
#6
Jeroen Venlet, Sebastiaan R D Piers, Jan D H Jongbloed, Alexander F A Androulakis, Yoshihisa Naruse, Dennis W den Uijl, Gijsbert F L Kapel, Marta de Riva, J Peter van Tintelen, Daniela Q C M Barge-Schaapveld, Martin J Schalij, Katja Zeppenfeld
BACKGROUND: High-level endurance training has been associated with right ventricular pathological remodeling and ventricular tachycardia (VT). Although overlap with arrhythmogenic right ventricular cardiomyopathy (ARVC) has been suggested, the arrhythmogenic substrate for VTs in athletes is unknown. OBJECTIVES: The goal of this study was to evaluate whether electroanatomic scar patterns related to sustained VT can distinguish exercise-induced arrhythmogenic remodeling from ARVC and post-inflammatory cardiomyopathies...
February 7, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28146053/cardiac-channelopathies-and-sudden-death-recent-clinical-and-genetic-advances
#7
REVIEW
Anna Fernández-Falgueras, Georgia Sarquella-Brugada, Josep Brugada, Ramon Brugada, Oscar Campuzano
Sudden cardiac death poses a unique challenge to clinicians because it may be the only symptom of an inherited heart condition. Indeed, inherited heart diseases can cause sudden cardiac death in older and younger individuals. Two groups of familial diseases are responsible for sudden cardiac death: cardiomyopathies (mainly hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic cardiomyopathy) and channelopathies (mainly long QT syndrome, Brugada syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia)...
January 29, 2017: Biology
https://www.readbyqxmd.com/read/28139837/long-term-follow-up-of-arrhythmogenic-right-ventricular-cardiomyopathy-patients-with-an-implantable-cardioverter-defibrillator-for-prevention-of-sudden-cardiac-death
#8
Kang Yin, Ligang Ding, Yuqiu Li, Wei Hua
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited cardiomyopathywith a high burden of ventricular arrhythmia, which is an important cause of suddencardiac death (SCD). Implantable cardioverter-defibrillator (ICD) is believed to be the most reliable management against SCD. HYPOTHESIS: Ventricular arrhythmia does not necessarily confer a poor prognosis in ARVC patients with an ICD. METHODS: A total of 39 ARVC patients (34 male) implanted with an ICD at our electrophysiologycenter and followed up continuously were included in this study...
January 31, 2017: Clinical Cardiology
https://www.readbyqxmd.com/read/28131707/the-application-of-signal-average-ecg-in-the-prediction-of-recurrences-after-catheter-ablation-of-ventricular-arrhythmias-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#9
Ying-Chieh Liao, Fa-Po Chung, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Chin-Yu Lin, Yao-Ting Chang, Ming-Hsiung Hsieh, Shih-Ann Chen
BACKGROUND: The changes of signal averaged ECG (SAECG) in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) undergoing radiofrequency catheter ablation (RFCA) of ventricular arrhythmias (VAs) remains unknown. METHODS: Between 2010 and 2014, a total of 81 ARVD/C patients underwent endocardial and/or epicardial RFCA for drug-refractory VAs. Seventy patients (mean age 46.2±14.1years, 37 males) achieving acute procedure success (negative inducibility) were enrolled...
January 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28130335/development-of-dilated-cardiomyopathy-and-impaired-calcium-homeostasis-with-cardiac-specific-deletion-of-esrr%C3%AE
#10
Glenn C Rowe, Angeliki Asimaki, Evan L Graham, Kimberly D Martin, Kenneth B Margulies, Saumya Das, Jeffrey E Saffitz, Zoltan Arany
Mechanisms underlying the development of Idiopathic Dilated Cardiomyopathy (DCM) remain poorly understood. Using transcription factor expression profiling, we identified estrogen-related receptor beta (ESRRβ), a member of the nuclear receptor family of transcription factors, as highly expressed in murine hearts and other highly oxidative striated muscle beds. Mice bearing cardiac-specific deletion of ESRRβ (MHC-ERRB KO) develop dilated cardiomyopathy and sudden death at approximately 10 months of age. Isolated adult cardiomyocytes from the MHC-ERRB KO mice showed an increase in calcium sensitivity and impaired cardiomyocyte contractility, which preceded echocardiographic cardiac remodeling and dysfunction by several months...
January 27, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28129067/radiologic-pathologic-correlation-of-primary-and-secondary-cardiomyopathies-mr-imaging-and-histopathologic-findings-in-hearts-from-autopsy-and-transplantation
#11
Hiromi Hashimura, Fumiko Kimura, Hatsue Ishibashi-Ueda, Yoshiaki Morita, Masahiro Higashi, Shintaro Nakano, Atsushi Iguchi, Kensuke Uotani, Kazuro Sugimura, Hiroaki Naito
Cardiac magnetic resonance (MR) imaging with late gadolinium enhancement (LGE) is used to detect and assess the myocardial damage seen with a variety of cardiomyopathies. Gadolinium-based contrast material accumulates in the expanded interstitial space of the myocardium. Areas with LGE correspond to replacement fibrosis, fibrofatty change, epithelioid granuloma, inflammatory cell infiltration, cardiomyocyte necrosis, and amyloid deposition-conditions that represent a focal increase in interstitial space. Areas without LGE correspond to interstitial or plexiform fibrosis, mildly degenerated cardiomyocytes, inflammatory cell infiltration, and diffuse amyloid deposition-conditions that represent diffuse increases in interstitial space...
January 27, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28122031/long-term-outcomes-of-radio-frequency-catheter-ablation-on-ventricular-tachycardias-due-to-arrhythmogenic-right-ventricular-cardiomyopathy-a-single-center-experience
#12
Wei Wei, Hongtao Liao, Yumei Xue, Xianhong Fang, Jun Huang, Yang Liu, Hai Deng, Yuanhong Liang, Zili Liao, Fangzhou Liu, Weidong Lin, Xianzhang Zhan, Shulin Wu
AIMS: To summarize our experience of radiofrequency catheter ablation (RFCA) for recurrent drug-refractory ventricular tachycardias (VTs) due to arrhythmogenic right ventricular cardiomyopathy (ARVC) in our center over the past 11 years and its related factors. METHODS AND RESULTS: We reviewed 48 adults (mean age 39.9 ± 12.9 years, range: 14 to 65) who met the present ARVC diagnostic criteria and accepted RFCA for VTs from December 2004 to April 2016. The patients received a total of 70 procedures using two ablation approaches, the endocardial approach in 52 RFCAs, and the combined epicardial and endocardial approach (the combined approach) in 18 RFCAs...
2017: PloS One
https://www.readbyqxmd.com/read/28120905/genotype-phenotype-relationship-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-caused-by-desmosomal-gene-mutations-a-systematic-review-and-meta-analysis
#13
Zhenyan Xu, Wengen Zhu, Cen Wang, Lin Huang, Qiongqiong Zhou, Jinzhu Hu, Xiaoshu Cheng, Kui Hong
The relationship between clinical phenotypes and desmosomal gene mutations in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is poorly characterized. Therefore, we performed a meta-analysis to explore the genotype-phenotype relationship in patients with ARVC. Any studies reporting this genotype-phenotype relationship were included. In total, 11 studies involving 1,113 patients were included. The presence of desmosomal gene mutations was associated with a younger onset age of ARVC (32.7 ± 15...
January 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28110572/-cardiomyopathy-and-ion-channel-diseases-registry-the-szeged-cardiogen-registry
#14
Péter Blazsó, Kornél Kákonyi, Tamás Forster, Róbert Sepp
The Szeged cardiomyopathy and ion channel diseases registry aims to establish a representative disease-specific registry based on the recruitment of patients with different cardiomyopathies and ion channel diseases followed at the Cardiology Center, University of Szeged. The registry collects patient data on the main forms of primary cardiomyopathies (hypertrophic, dilated, restrictive, arrhythmogenic right ventricular, left ventricular non-compact, tako-tsubo cardiomyopathy) and ion channel diseases (long QT syndrome, short QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia)...
January 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28103994/-analysis-of-microrna-expression-profile-in-serum-of-patients-with-electrical-burn-or-thermal-burn
#15
Q F Ruan, M J Jiang, Z Q Ye, C L Zhao, W G Xie
Objective: To explore the differential expression of microRNAs in the serum among patients with electrical burn or thermal burn and healthy persons and to explore the significance. Methods: In this study we included three patients with electrical burn and three patients with thermal burn, conforming to the inclusion criteria and hospitalized in our burn ward from June to August 2015, and three healthy adult volunteers. Their serum samples were separated from whole blood and divided into electrical burn group, thermal burn group, and normal control group...
January 20, 2017: Zhonghua Shao Shang za Zhi, Zhonghua Shaoshang Zazhi, Chinese Journal of Burns
https://www.readbyqxmd.com/read/28102477/potential-new-mechanisms-of-pro-arrhythmia-in-arrhythmogenic-cardiomyopathy-focus-on-calcium-sensitive-pathways
#16
REVIEW
C J M van Opbergen, M Delmar, T A B van Veen
Arrhythmogenic cardiomyopathy, or its most well-known subform arrhythmogenic right ventricular cardiomyopathy (ARVC), is a cardiac disease mainly characterised by a gradual replacement of the myocardial mass by fibrous and fatty tissue, leading to dilatation of the ventricular wall, arrhythmias and progression towards heart failure. ARVC is commonly regarded as a disease of the intercalated disk in which mutations in desmosomal proteins are an important causative factor. Interestingly, the Dutch founder mutation PLN R14Del has been identified to play an additional, and major, role in ARVC patients within the Netherlands...
January 19, 2017: Netherlands Heart Journal
https://www.readbyqxmd.com/read/28097477/spect-perfusion-abnormality-associated-with-arrhythmogenic-right-ventricular-cardiomyopathy
#17
Charles Hardebeck, Akira Wada, Gbemiga Sofowora
No abstract text is available yet for this article.
January 17, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28097329/quantifying-disease-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-key-to-advancing-therapy
#18
Wojciech Zareba
No abstract text is available yet for this article.
January 11, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28097316/evaluation-of-structural-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#19
Thomas P Mast, Cynthia A James, Hugh Calkins, Arco J Teske, Crystal Tichnell, Brittney Murray, Peter Loh, Stuart D Russell, Birgitta K Velthuis, Daniel P Judge, Dennis Dooijes, Ryan J Tedford, Jeroen F van der Heijden, Harikrishna Tandri, Richard N Hauer, Theodore P Abraham, Pieter A Doevendans, Anneline S J M Te Riele, Maarten J Cramer
Importance: Considerable research has described the arrhythmic course of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However, objective data characterizing structural progression, such as ventricular enlargement and cardiac dysfunction, in ARVD/C are relatively scarce. Objectives: To define the extent of structural progression, identify determinants of structural progression, and determine the association between structural progression and electrocardiographic (ECG) changes in patients with ARVD/C...
January 11, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28089876/antiarrhythmic-drug-therapy-in-patients-with-arrhythmogenic-dysplasia-cardiomyopathy-is-there-a-role-for-flecainide
#20
EDITORIAL
Hugh Calkins
No abstract text is available yet for this article.
January 12, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
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