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https://www.readbyqxmd.com/read/28290856/-chronic-heart-failure-with-preserved-systolic-function-and-reversible-dilatation-of-cardiac-chambers
#1
O V Blagova, S V Volkov, A V Nedostup, A O Korobkov, I V Mostovoy, N G Sergushina, N V Gagarina, E A Mershina
Diagnosis of dilation (D) cardiomyopathy (CMP) requires exclusion not only of inflammatory and genetically determined forms but also of some rare diseases. This 51 year old patient with history of moderate arterial hypertension approached a cardiologist because of new onset atrial fibrillation and dyspnea. Echocardiography detected dilation of all cardiac chambers with relatively preserved ejection fraction, causing suspicion of DCMP. Among conditions excluded were coronary atherosclerosis, congenital heart defect with left to right shunt, primary pulmonary hypertension, pulmonary embolism, hypertensive heart, tachycardia induced CMP, arrhythmogenic right ventricular dysplasia, noncompaction myocardium...
June 2016: Kardiologiia
https://www.readbyqxmd.com/read/28288337/remodelling-of-myocardial-intercalated-disc-protein-connexin-43-causes-increased-susceptibility-to-malignant-arrhythmias-in-arvc-d-patients
#2
Xiao Chen, Liang Chen, Zhenglian Chen, Xinshan Chen, Jiangping Song
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a primary cardiomyopathy characterised by fibrofatty replacement and ventricular arrhythmias. The occurrence of malignant arrhythmias may be associated with fatty infiltration and intercalated disk remodelling, but the specific pathological remodelling pattern is not yet clear. METHODS: Twelve explanted hearts from patients diagnosed with ARVC/D according to the 2010 Task Force Criteria and pathology examination were divided into two groups with (SVT, n=6) or without (non-SVT, n=6) recurrent sustained ventricular tachycardia (SVT) before heart transplantation...
February 27, 2017: Forensic Science International
https://www.readbyqxmd.com/read/28220464/perspectives-and-challenges-of-pluripotent-stem-cells-in-cardiac-arrhythmia-research
#3
REVIEW
Alexander Goedel, Ilaria My, Daniel Sinnecker, Alessandra Moretti
PURPOSE OF REVIEW: The promises of human-induced pluripotent stem cells (hiPSCs) for modeling arrhythmogenic disease, but also for drug discovery and toxicity tests, are straightforward and exciting. However, the full potential of this new technology has not been fully realized yet. The purpose of this review is to provide an overview of the state-of-the-art research in arrhythmogenic disease modeling and drug discovery and an outlook of what can be expected from the second decade of hiPSC-based arrhythmia research...
March 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28215569/cardiac-phenotype-and-long-term-prognosis-of-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia-patients-with-late-presentation
#4
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 (ARVC/D) patients with late presentation is unknown. OBJECTIVE: The purpose of this study was to characterize the genotype, cardiac phenotype, and long-term outcomes of ARVC/D patients with late presentation (age ≥50 years at diagnosis). METHODS: Five hundred two patients with an ARVC/D diagnosis from Johns Hopkins and Utrecht Registries were studied and long-term clinical outcomes ascertained...
February 12, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/28214153/arrhythmogenic-right-ventricular-dysplasia-atypical-clinical-presentation
#5
José Marçalo, Luiz Menezes Falcão
A 67-year-old man was admitted to our hospital after episodes of syncope preceded by malaise and diffuse neck and chest discomfort. No family history of cardiac disease was reported. Laboratory workup was within normal limits, including D-dimers, serum troponin I and arterial blood gases. The electrocardiogram showed sinus rhythm with T-wave inversion in leads V1 to V3. Computed tomography angiography to investigate pulmonary embolism showed no abnormal findings. Transthoracic echocardiography (TTE) displayed massive enlargement of the right ventricle with intact interatrial septum and no pulmonary hypertension...
March 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28167090/ablation-of-ventricular-tachycardia-in-arrhythmogenic-right-ventricular-dysplasia
#6
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/28131707/the-application-of-signal-average-ecg-in-the-prediction-of-recurrences-after-catheter-ablation-of-ventricular-arrhythmias-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#7
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/28097329/quantifying-disease-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-key-to-advancing-therapy
#8
Wojciech Zareba
No abstract text is available yet for this article.
March 1, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28097316/evaluation-of-structural-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#9
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...
March 1, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28089876/antiarrhythmic-drug-therapy-in-patients-with-arrhythmogenic-dysplasia-cardiomyopathy-is-there-a-role-for-flecainide
#10
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
https://www.readbyqxmd.com/read/28069705/multilevel-analyses-of-scn5a-mutations-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-suggest-non-canonical-mechanisms-for-disease-pathogenesis
#11
Anneline S J M Te Riele, Esperanza Agullo-Pascual, Cynthia A James, Alejandra Leo-Macias, Marina Cerrone, Mingliang Zhang, Xianming Lin, Bin Lin, Nara L Sobreira, Nuria Amat-Alarcon, Roos F Marsman, Brittney Murray, Crystal Tichnell, Jeroen F van der Heijden, Dennis Dooijes, Toon A B van Veen, Harikrishna Tandri, Steven J Fowler, Richard N W Hauer, Gordon Tomaselli, Maarten P van den Berg, Matthew R G Taylor, Francesca Brun, Gianfranco Sinagra, Arthur A M Wilde, Luisa Mestroni, Connie R Bezzina, Hugh Calkins, J Peter van Tintelen, Lei Bu, Mario Delmar, Daniel P Judge
AIMS: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is often associated with desmosomal mutations. Recent studies suggest an interaction between the desmosome and sodium channel protein Nav1.5. We aimed to determine the prevalence and biophysical properties of mutations in SCN5A (the gene encoding Nav1.5) in ARVD/C. METHODS AND RESULTS: We performed whole-exome sequencing in six ARVD/C patients (33% male, 38.2 ± 12.1 years) without a desmosomal mutation...
January 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28062531/epsilon-wave-on-an-electronic-loop-in-a-case-of-arrhythmogenic-right-ventricular-dysplasia-with-myocarditis-an-updated-definition-of-the-epsilon-wave
#12
Guy Hugues Fontaine, Guillaume Duthoit, Guoliang Li, L Andreoletti, Estelle Gandjbakhch, Robert Frank
A young man presented with a history of myocarditis with palpitations and dizziness. He had implantation of a loop recorder that showed repetitive short episodes of VT. In addition, there were fragmented potentials immediately following the large and sharp electrograms (EGMs) before as well as after episodes of VT suggesting an Epsilon wave. This signal can be observed in multiple cardiac conditions including coronary artery disease. It was originally recorded on the epicardium as well as on the endocardium...
January 6, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28032368/clinical-presentation-and-echocardiographic-characteristics-of%C3%A2-uhl-s-anomaly
#13
Christos G Mihos, Maiteder Larrauri-Reyes, Evin Yucel, Orlando Santana
Uhl's anomaly is a rare cardiac malformation that results in partial or complete absence of the right ventricular myocardium. It most commonly presents in prenatal or newborn infants; however, it may also be found in some adults as advanced right-sided heart failure. Differential diagnoses include arrhythmogenic right ventricular dysplasia and Ebstein's anomaly. Herein, we describe the clinical presentation of Uhl's anomaly in a previously undiagnosed middle-aged adult, and review the echocardiographic criteria used to diagnose and differentiate this rare, albeit important, myocardial disorder...
December 29, 2016: Echocardiography
https://www.readbyqxmd.com/read/27932238/gender-differences-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-clinical-manifestations-electrophysiological-properties-substrate-characteristics-and-prognosis-of-radiofrequency-catheter-ablation
#14
Chin-Yu Lin, Fa-Po Chung, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Yao-Ting Chang, Yun-Yu Chen, Rohit Walia, Abigail Louise D Te, Shinya Yamada, Shih-Ann Chen
BACKGROUND: Gender differences in the penetrance and clinical expression of genetic mutations have been reported in patients with arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C). Our study aimed at clarifying the impact of gender on ventricular substrates and clinical outcomes after radiofrequency catheter ablation (RFCA). METHODS: Patients with ARVD/C underwent RFCA for drug-refractory ventricular arrhythmias (VAs) were consecutively enrolled...
January 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27920450/catheter-ablation-for-ventricular-tachycardia-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-a-systematic-review-and-meta-analysis
#15
H Jiang, X -L Zhang, Q -L Yang, S -H Zhu, H -S Yu, B Xu, W Huang
No abstract text is available yet for this article.
2016: Acta Cardiologica
https://www.readbyqxmd.com/read/27909515/myocardial-biopsy-in-idiopathic%C3%A2-atrial-fibrillation-and-other-arrhythmias-nosological-diagnosis-clinical-and-morphological-parallels-and-treatment
#16
O V Blagova, A V Nedostup, E A Kogan, V A Sulimov, S A Abugov, A G Kupriyanova, V A Zaydenov, A E Donnikov, E V Zaklyazminskaya, E A Okisheva
BACKGROUND: The nosological nature of "idiopathic" arrhythmias and the effect of etiotropic and pathogenetic treatment are often unknown. METHODS AND RESULTS: 19 patients (42.6±11.3 years, 9 women) with atrial fibrillation (n = 16), supraventricular (n = 10) and ventricular (n = 4) premature beats, supraventricular (n = 2) and ventricular tachycardia (n = 1), left bundle branch block (n= 2), AV block (n = 2) without structural heart changes. Viruses were identified (polymerase chain reaction, PCR) along with measurement of anti-heart antibodies (AHA) and endomyocardial biopsy (EMB)...
June 2016: Journal of Atrial Fibrillation
https://www.readbyqxmd.com/read/27900527/should-all-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-undergo-epicardial-catheter-ablation
#17
Andreas Müssigbrodt, Elena Efimova, Helge Knopp, Livio Bertagnolli, Nikolaos Dagres, Sergio Richter, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
BACKGROUND: Epicardial radiofrequency catheter ablation is currently considered as the therapeutic option of choice in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and recurrent ventricular tachycardia (VT). We hypothesised that inducibility of VT may guide the ablation strategy and thereby affect long-term results. Additional epicardial ablation was only performed if VT were still inducible after thorough endocardial ablation. METHODS: The objective was to examine an inducibility-guided ablation approach by comparing the long-term results between endocardial and epi-endocardial radiofrequency catheter ablation of VT in a large cohort of patients with ARVD/C...
March 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/27871237/a-unique-association-of-arrhythmogenic-right-ventricular-dysplasia-and-acute-myocarditis-as-assessed-by-cardiac-mri-a-case-report
#18
Andrea Ponsiglione, Marta Puglia, Carmine Morisco, Luigi Barbuto, Antonio Rapacciuolo, Mario Santoro, Letizia Spinelli, Bruno Trimarco, Alberto Cuocolo, Massimo Imbriaco
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD), is a genetic disorder of the heart, which mainly involves the right ventricle. It is characterized by hypokinetic areas at the free wall of the right ventricle (RV) or both ventricles, where myocardium is replaced by fibrous or fatty tissue. ARVD is an important cause of ventricular arrhythmias in children and young adults. Although the transmission of the disease is based on hereditary, in young adults it may not show any symptoms...
November 21, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27858926/-heart-failure-role-of-cardiovascular-imaging
#19
Rienzi Díaz Navarro
The usefulness of echocardiography and the new noninvasive cardiac techniques in assessing heart failure is analyzed. The usefulness of non-invasive CT coronary angiography, as well as the growing applications of magnetic resonance imaging (MRI) in the study of ischemic heart disease, cardiomyopathy and arrhythmogenic right ventricular dysplasia is considered. For this puspose, some clinical cases are used. The combined use of these techniques, especially in patients in whom the etiology of heart failure is ischemic heart disease or cardiomyopathy is emphasized...
October 11, 2016: Medwave
https://www.readbyqxmd.com/read/27790050/arrhythmogenic-right-ventricular-dysplasia-in-neuromuscular-disorders
#20
REVIEW
Josef Finsterer, Claudia Stöllberger
OBJECTIVES: Arrhythmogenic right ventricular dysplasia (ARVD) is a rare, genetic disorder predominantly affecting the right ventricle. There is increasing evidence that in some cases, ARVD is due to mutations in genes, which have also been implicated in primary myopathies. This review gives an overview about myopathy-associated ARVD and how these patients can be managed. METHODS: A literature review was done using appropriate search terms. RESULTS: The myopathy, which is most frequently associated with ARVD, is the myofibrillar myopathy due to desmin mutations...
2016: Clinical Medicine Insights. Cardiology
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