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Arrhythmogenic cardiomyopathy

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https://www.readbyqxmd.com/read/28536891/myocardial-voltage-ratio-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#1
REVIEW
Andreas Müssigbrodt, Livio Bertagnolli, Elena Efimova, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Michael Döring, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
AIMS: This study aimed to analyze the influence of scar distribution between the endocardium and the epicardium in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). METHODS: Electroanatomical mapping data were derived from our ARVD/C registry. Myocardial voltage distribution between the endocardium and the epicardium was analyzed in 28 patients (18 men, 49.9 ± 13.0 years) with previous ventricular tachycardia (VT) ablation and complete right ventricular maps...
May 23, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28527814/desmoplakin-missense-and-non-missense-mutations-in-arrhythmogenic-right-ventricular-cardiomyopathy-genotype-phenotype-correlation
#2
Silvia Castelletti, Annina S Vischer, Petros Syrris, Lia Crotti, Carla Spazzolini, Alice Ghidoni, Gianfranco Parati, Sharon Jenkins, Maria-Christina Kotta, William J McKenna, Peter J Schwartz, Antonis Pantazis
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is traditionally considered as primarily affecting the right ventricle. Mutations in genes encoding desmosomal proteins account for 40-60% of cases. Genotype-phenotype correlations are scant and mostly non gene-specific. Accordingly, we assessed the genotype-phenotype correlation for desmoplakin (DSP) missense and non-missense mutations causing ARVC. METHODS AND RESULTS: We analyzed 27 ARVC patients carrying a missense or a non-missense DSP mutation, with complete clinical assessment...
May 10, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28523642/autosomal-recessive-nonsyndromic-arrhythmogenic-right-ventricular-cardiomyopathy-without-cutaneous-involvements-a-novel-mutation
#3
Mahdieh Soveizi, Bahareh Rabbani, Yousef Rezaei, Sedigheh Saedi, Nasim Najafi, Majid Maleki, Nejat Mahdieh
The arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic disease frequently associated with desmosomal mutations, mainly attributed to dominant mutations in the Plakophilin-2 (PKP2) gene. Naxos and Carvajal are the syndromic forms of ARVD/C due to recessive mutations. Herein, we report an autosomal recessive form of nonsyndromic ARVD/C caused by a mutation in the PKP2 gene. After examination and implementation of diagnostic modalities, the definite diagnosis of ARVD/C was confirmed by detection of ventricular tachycardia with a left bundle branch configuration and a superior axis, T-wave inversion in right precordial leads (i...
May 19, 2017: Annals of Human Genetics
https://www.readbyqxmd.com/read/28510712/gender-issues-in-arrhythmias-from-atrial-fibrillation-to-crt-and-arrhythmogenic-ventricular-cardiomyopathy
#4
Thomas F Lüscher
No abstract text is available yet for this article.
May 14, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28509892/arrhythmogenic-right-ventricular-dysplasia-an-under-recognized-form-of-inherited-cardiomyopathy
#5
George O Adesina, Shelly A Hall, Jose C Mendez, Susan M Joseph, Robert L Gottlieb, Parag P Kale, Amarinder S Bindra
We report a case of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD) in order to evaluate the course of an under-recognized form of cardiomyopathy with a vast array of clinical manifestations. The patient is a 49-year-old white woman transferred from an outside hospital due to dyspnea and persistent hypoxia. She had a pertinent family history that included a sister who died suddenly in her 30s from unexplained heart failure. Initial work-up for hypoxia was unrevealing. Transthoracic echocardiography revealed isolated right ventricular dysfunction with dilation and multiple trabeculations...
2017: Reviews in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28506445/comparison-of-features-of-fatal-versus-nonfatal-cardiac-arrest-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#6
Richa Gupta, Crystal Tichnell, Brittney Murray, Stefania Rizzo, Anneline Te Riele, Harikrishna Tandri, Daniel P Judge, Gaetano Thiene, Cristina Basso, Hugh Calkins, Cynthia A James
Once arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is diagnosed, the incidence of sudden cardiac death (SCD) is rare and prognosis is favorable, highlighting the value of early disease recognition. To inform strategies to diagnose ARVD/C before SCD, we sought to characterize clinical, genetic, and family history features of ARVD/C cases first recognized after SCD or resuscitated SCD (sudden cardiac arrest [SCA]). We identified 66 ARVD/C cases submitted to the Johns Hopkins ARVD/C Registry in whom disease was first recognized after SCD (n = 45) or SCA (n = 21) and compared their clinical, genetic, and demographic features with 352 patients (227 probands) diagnosed with ARVD/C by 2010 Task Force Criteria before any arrest...
April 13, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28504961/wnt-%C3%AE-catenin-pathway-in-arrhythmogenic-cardiomyopathy
#7
REVIEW
Alessandra Lorenzon, Martina Calore, Giulia Poloni, Leon J De Windt, Paola Braghetta, Alessandra Rampazzo
Wnt/β-catenin signaling pathway plays essential roles in heart development as well as cardiac tissue homoeostasis in adults. Abnormal regulation of this signaling pathway is linked to a variety of cardiac disease conditions, including hypertrophy, fibrosis, arrhythmias, and infarction. Recent studies on genetically modified cellular and animal models document a crucial role of Wnt/β-catenin signaling in the molecular pathogenesis of arrhythmogenic cardiomyopathy (AC), an inherited disease of intercalated discs, typically characterized by ventricular arrhythmias and progressive substitution of the myocardium with fibrofatty tissue...
April 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28496511/evaluation-of-coronary-arteries-in-non-ischemic-cardiomyopathies-a-case-report
#8
Farveh Vakilian, Mahmood Mohamadzadeh Shabestari, Ahmad Amin, Soheila Chamanian, Toktam Moghiman
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a congenital cardiac disease with myocardial involvement, most probably right ventricular (RV) dysfunction, accounting for 20% of sudden cardiac deaths. Characterized by the fibro-fatty infiltration of the RV free wall, ARVD/C presents in adolescents with ventricular arrhythmias and heart failure symptoms and as biventricular failure in adults. The coronary risk in these patients is not clear. We present an incidental finding: the left anterior descending artery cut-off in a middle-aged man with ARVD/C...
October 3, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28492156/electrical-storm-or-naxos-syndrome-in-an-adult-causing-recurrent-syncope
#9
Muhammad Furrakh Maqbool, Muhammad Sajid, Ahmed Noeman
Among the rare and well-known causes of sudden cardiac death by malignant arrthymias is a condition called arrhythmogenic right ventricular cardiomyopathy. It commonly presents with right ventricular dilatation, dysfunction and ventricular tachycardia of left bundle branch morphology due to fibro-fatty infiltration of right ventricle in second to fifth decade of life, making it an unrecognized and important cause of sudden cardiac death. Two rare variants of arrhythmogenic right ventricular cardiomyopathy are Carvajal syndrome and Naxos syndrome...
April 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28491739/incremental-value-of-electroanatomical-mapping-for-the-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy-in-a-patient-with-sustained-ventricular-tachycardia
#10
Simon A Castro, Rajeev K Pathak, Daniele Muser, Pasquale Santangeli, Anjali Owens, Francis Marchlinski, Fermin C Garcia
No abstract text is available yet for this article.
November 2016: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28491693/congenital-heart-disease-confounding-the-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy
#11
Omid Kiamanesh, Maryam Farhan, Shubhayan Sanatani, Lars Grosse-Wortmann, Walter Duncan, Robert M Hamilton
No abstract text is available yet for this article.
July 2016: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28488109/-epicardial-ablation-of-ventricular-tachycardias
#12
REVIEW
E Ene, P Halbfaß, K Nentwich, K Sonne, M Roos, S Fodor, L Lehmkuhl, F Gietzen, S Barth, K Hamm, T Deneke
Ventricular tachycardias (VT) in patients with structural heart diseases have predominantly a scar-associated reentry mechanism so that substrate-based ablation approaches also have to be used in nearly all procedures. In many VT cases-especially in nonischemic cardiomyopathy (NICM) and arrhythmogenic right ventricular cardiomyopathy-a critical epicardial substrate can be identified as an essential component of the reentry circuit so that for the ablation-based modification of the substrate in these cases an epicardial approach is necessary...
May 9, 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28472724/relevance-of-molecular-testing-in-patients-with-a-family-history-of-sudden-death
#13
Silke Kauferstein, Nadine Herz, Stefanie Scheiper, Stephanie Biel, Tina Jenewein, Malte Kunis, Damir Erkapic, Britt-Maria Beckmann, Thomas Neumann
Sudden cardiac death (SCD) is a major cause of death in industrial countries. Although SCD occurs mainly in adults, it may also affect young persons, where genetic cardiac disorders comprise at least half of these cases. This includes primary arrhythmogenic disorders such as long QT syndrome and inherited cardiomyopathies. However, in many cases, postmortem examinations provide no conclusive results explaining the cause of death. Since family members of the deceased may eventually have inherited the same disease, they are at risk of SCD...
April 13, 2017: Forensic Science International
https://www.readbyqxmd.com/read/28471438/electronic-health-record-phenotype-in-subjects-with-genetic-variants-associated-with-arrhythmogenic-right-ventricular-cardiomyopathy-a-study-of-30-716-subjects-with-exome-sequencing
#14
Christopher M Haggerty, Cynthia A James, Hugh Calkins, Crystal Tichnell, Joseph B Leader, Dustin N Hartzel, Christopher D Nevius, Sarah A Pendergrass, Thomas N Person, Marci Schwartz, Marylyn D Ritchie, David J Carey, David H Ledbetter, Marc S Williams, Frederick E Dewey, Alexander Lopez, John Penn, John D Overton, Jeffrey G Reid, Matthew Lebo, Heather Mason-Suares, Christina Austin-Tse, Heidi L Rehm, Brian P Delisle, Daniel J Makowski, Vishal C Mehra, Michael F Murray, Brandon K Fornwalt
PurposeArrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart disease. Clinical follow-up of incidental findings in ARVC-associated genes is recommended. We aimed to determine the prevalence of disease thus ascertained.MethodsIndividuals (n = 30,716) underwent exome sequencing. Variants in PKP2, DSG2, DSC2, DSP, JUP, TMEM43, or TGFβ3 that were database-listed as pathogenic or likely pathogenic were identified and evidence-reviewed. For subjects with putative loss-of-function (pLOF) variants or variants of uncertain significance (VUS), electronic health records (EHR) were reviewed for ARVC diagnosis, diagnostic criteria, and International Classification of Diseases (ICD-9) codes...
May 4, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28469947/successful-long-term-use-of-eltrombopag-in-a-patient-with-refractory-severe-thrombocytopenia-associated-with-chronic-lymphocytic-leukemia-that-allowed-oral-anticoagulant-treatment-for-severe-cardiomyopathy
#15
Juárez Salcedo Luis Miguel, Gil-Fernández Juan José
Autoimmune cytopenias (AICs) are frequently associated with chronic lymphocytic leukemia (CLL). The most common of these AICs is autoimmune hemolytic anemia (AIHA); the second most is immune thrombocytopenia (ITP). Here, we report on a patient with CLL-associated ITP, with thrombocytopenia refractory to corticosteroids and intravenous immunoglobulins, in which continuous oral treatment with Eltrombopag allowed initiation and maintenance of an oral anticoagulation treatment with Acenocoumarol that was indicated because of a severe arrhythmogenic cardiomyopathy...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28465114/heart-failure-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-what-are-the-risk-factors
#16
Yoshitaka Kimura, Takashi Noda, Taka-Aki Matsuyama, Yosuke Otsuka, Tsukasa Kamakura, Mitsuru Wada, Kohei Ishibashi, Yuko Inoue, Koji Miyamoto, Hideo Okamura, Satoshi Nagase, Takeshi Aiba, Shiro Kamakura, Teruo Noguchi, Toshihisa Anzai, Kazuhiro Satomi, Yuko Wada, Seiko Ohno, Minoru Horie, Wataru Shimizu, Satoshi Yasuda, Hiroaki Shimokawa, Kengo Kusano
BACKGROUND: We previously demonstrated that heart failure (HF) was one of the major causes of death in arrhythmogenic right ventricular cardiomyopathy (ARVC). The purpose of this study was to elucidate the clinical impact and risk factors of HF in patients with ARVC. METHODS AND RESULTS: We evaluated cardiac adverse outcomes including HF in 113 consecutive patients with ARVC (85 men, mean age: 44±15years). During a median follow-up of 10.0years (interquartile range: 5...
April 21, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28457738/acromegalic-cardiomyopathy-with-malignant-arrhythmogenic-pattern-successfully-treated-with-mechanical-circulatory-support-and-heart-transplantation
#17
Sara Doimo, Daniela Miani, Nicoletta Finato, Mauro Driussi, Gianfranco Sinagra, Ugolino Livi, Alessandro Proclemer
Cardiovascular involvement is common in acromegaly and can lead to development of acromegalic cardiomyopathy, characterized by concentric biventricular hypertrophy with a progressive impairment of diastolic and systolic function. The onset of heart failure and arrhythmias are related to poor prognosis. We report on a case of a 48-year-old man with acromegalic cardiomyopathy caused by pituitary adenoma. Despite the successful trans-sphenoidal resection of the tumour, the patient was rehospitalized for ventricular arrhythmic storms that led to cardiogenic shock, which required mechanical hemodynamic support with intra-aortic balloon pump, venoarterial extracorporeal membrane oxygenation, and urgent heart transplantation...
February 21, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28457241/development-of-tachyarrhythmias-late-after-the-fontan-procedure-the-role-of-ablative-therapy
#18
REVIEW
Natasja M S de Groot, Ad J J C Bogers
Patients with a Fontan circulation are at a high risk of developing a variety of cardiac dysrhythmias after cardiac surgery. These dysrhythmias are most often supraventricular tachyarrhythmias (SVT), but ventricular tachyarrhythmias (VT) may also occur. Mechanisms underlying SVT are variable, including both ectopic activity and reentry. Over time, successive SVT may be caused by different mechanisms. The acute success rate of ablative therapy of atrial tachyarrhythmias is considerably high yet during long-term follow-up 'recurrences' frequently occur...
June 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28454914/genetic-and-epigenetic-regulation-of-arrhythmogenic-cardiomyopathy
#19
REVIEW
Stefan Mazurek, Gene H Kim
Arrhythmogenic cardiomyopathy (AC) is most commonly characterized as a disease of the intercalated disc that promotes abnormal cardiac conduction. Previously, arrhythmogenic cardiomyopathy was frequently referred to as arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D); however, genotype-phenotype studies have defined a broader phenotypic spectrum; with the identification of left-dominant and biventricular subtypes. Molecular insight into AC has primarily focused on mutations in desmosomal proteins and the downstream signaling pathways; however, desmosomal gene mutations can only be identified in approximately fifty percent of patients with AC...
April 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28453732/corrigendum-to-multilevel-analyses-of-scn5a-mutations-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-suggest-non-canonical-mechanisms-for-disease-pathogenesis
#20
(no author information available yet)
No abstract text is available yet for this article.
May 1, 2017: Cardiovascular Research
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