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Arrhythmogenic cardiomyopathy

Ida S Leren, Jørg Saberniak, Trine F Haland, Thor Edvardsen, Kristina H Haugaa
OBJECTIVES: The aim of this study was to investigate early markers of arrhythmic events (AEs) and improve risk stratification in early arrhythmogenic right ventricular cardiomyopathy (ARVC). BACKGROUND: AEs are frequent in patients with ARVC, but risk stratification in subjects with early ARVC is challenging. METHODS: Early ARVC disease was defined as possible or borderline ARVC diagnosis according to the ARVC Task Force Criteria 2010. We performed resting and signal averaged electrocardiogram (ECG)...
October 14, 2016: JACC. Cardiovascular Imaging
J Vila, R Pariaut, N S Moïse, E M Oxford, P R Fox, C A Reynolds, C Saelinger
OBJECTIVE: To investigate the expression and distribution of desmosomal and gap junction proteins of the intercalated disc in the atria of boxers with arrhythmogenic right ventricular cardiomyopathy (ARVC). ANIMALS: Nineteen control dogs and 13 boxers with histopathologically confirmed ARVC. METHODS: Right and left atrial samples were examined using immunofluorescence and Western blots. The intercalated disc proteins investigated included total and phosphorylated connexin43 (Cx43 and pCx43), connexin45, connexin40, plakoglobin, plakophilin-2, desmoplakin, and N-cadherin...
October 18, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
Seiko Ohno
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by degeneration of the right ventricle and ventricular tachycardia originating from the right ventricle. Additionally, the disease is an inherited cardiomyopathy that mainly follows the autosomal dominant pattern. More than 10 genes have been reported as causative genes for ARVC, and more than half of ARVC patients carry mutations in desmosome related genes. The desmosome is one of the structures involved in cell adhesion and its disruption leads to various diseases, including a skin disease called pemphigus...
October 2016: Journal of Arrhythmia
Christopher Semsarian, Jodie Ingles
Sudden cardiac death (SCD) is a rare but devastating complication of a number of underlying cardiovascular diseases. While coronary artery disease and acute myocardial infarction are the most common causes of SCD in older populations, inherited cardiac disorders comprise a substantial proportion of SCD cases aged less than 40 years. Inherited cardiac disorders include primary inherited arrhythmogenic disorders such as familial long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and inherited cardiomyopathies, most commonly hypertrophic cardiomyopathy (HCM)...
October 2016: Journal of Arrhythmia
Sahan P Semasinghe Bandaralage, Selvanayagam Nirthanan, Selvanayagam Niranjan
Despite proven effectiveness in treating tachyarrhythmias, sotalol is proarrhythmic and can cause torsades de pointes. Given the emergence of studies that show no benefit from rhythm control strategies in managing atrial fibrillation, as well as the introduction of nonpharmacological approaches to treating arrhythmias, we felt it necessary to ascertain if there was any role for sotalol given its side effects. Review of the literature regarding sotalol use in the prevention and treatment of supraventricular and ventricular tachyarrhythmias seems to show that more effective and safer agents and nonpharmacological alternatives are currently available...
October 3, 2016: American Journal of Therapeutics
Kristin McLeod, Samuel Wall, Ida Skrinde Leren, Jørg Saberniak, Kristina Hermann Haugaa
BACKGROUND: Altered right ventricular structure is an important feature of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), but is challenging to quantify objectively. The aim of this study was to go beyond ventricular volumes and diameters and to explore if the shape of the right and left ventricles could be assessed and related to clinical measures. We used quantifiable computational methods to automatically identify and analyse malformations in ARVC patients from Cardiovascular Magnetic Resonance (CMR) images...
October 14, 2016: Journal of Cardiovascular Magnetic Resonance
Livija Sušić, Vedrana Baraban, Josip Vincelj, Lana Maričić, Jasmina Ćatić, Robert Blažeković, Spomenka Manojlović
Detection of an intracardiac mass always represents a clinical challenge. We present a 61-year-old female patient with symptoms of New York Heart Association class III. Two-dimensional transthoracic echocardiography revealed a hypoechogenic mass in the cavity of the dilated right ventricle (RV). Cardiac MRI described a pathologic structure of the RV free wall with pedunculated tumor in its cavity. Three months later, on a repeated echocardiography, there were three individual masses. The patient underwent surgery and the pathohistologic report demonstrated thrombotic masses...
October 18, 2016: Journal of Clinical Ultrasound: JCU
Akm Monwarul Islam, Md Toufiqur Rahman, Abu Hana Chowdhury
Naxos disease is a rare autosomal recessive form of arrhythmogenic right ventricular cardiomyopathy (ARVC) with woolly hair and palmoplantar keratoderma. The cardiomyopathy presents by adolescence with syncope, ventricular tachycardia (VT) of left bundle branch block (LBBB) morphology, and/or ventricular fibrillation. The diagnosis and management of ARVC are at present in evolution; the recently published modified Task Force Criteria for diagnosis and International Task Force consensus statement for treatment of ARVC will hopefully bring about uniformity in recognition and management of Naxos disease as well...
October 2016: Cardiovascular Diagnosis and Therapy
Raimundo Barbosa-Barros, Andrés Ricardo Pérez-Riera, Luiz Carlos de Abreu, Ricardo Paulo de Sousa-Rocha, Danielli Oliveira da Costa Lino, Adrian Baranchuk, Li Zhang
Left ventricular involvement in the advanced stage of arrhythmogenic cardiomyopathy (AC) is an evolution of this desmosomopathy. Mutation in intercalated discs explains the phenotypic variability. Abnormal trafficking of the intercalated discs at the site of end-to-end contacts between cardiomyocytes and the canonical Wnt/β-catenin and Hippo signaling pathways have been implicated. In AC, T-wave inversion in lateral leads is the hallmark of left ventricular (LV) involvement. We herein report a case of isolated LV AC in which the initial ECG has typical features: fragmented QRS (fQRS), Twave inversion (TWI) in lateral leads, and ventricular tachycardia from inferobasal LV wall...
September 8, 2016: Journal of Electrocardiology
Antonio Berruezo, Juan Acosta, Juan Fernández-Armenta, Alonso Pedrote, Alberto Barrera, Eduardo Arana-Rueda, Andrés Ignacio Bodegas, Ignasi Anguera, Luis Tercedor, Diego Penela, David Andreu, Rosario Jesus Perea, Susana Prat-González, Lluis Mont
BACKGROUND: First-line endoepicardial ventricular tachycardia (VT) ablation has been proposed for patients with arrhythmogenic cardiomyopathy (AC). This study reports procedural safety, outcomes, and predictors of recurrence. METHODS AND RESULTS: Forty-one consecutive patients [12 with left ventricle (LV) involvement, 7 left-dominant] underwent first-line endoepicardial VT substrate ablation. Standard bipolar and unipolar thresholds were used to define low-voltage areas (LVA)...
October 12, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Štěpán Havránek, Tomáš Paleček, Petr Kuchynka, Ivana Vítková
Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a rare condition characterised by progressive fibrofatty replacement of the myocardium of the left ventricle in combination with arrhythmias of left ventricular origin. ALVC has been linked to autosomal dominant mutations of genes encoding desmosomal proteins, similarly to the classic arrhythmogenic right ventricular cardiomyopathy with which it also shares pathological and prognostic features. It seems that isolated left or right ventricular abnormalities represent two extremes of the spectrum of clinical manifestations of a single disease: arrhythmogenic cardiomyopathy...
2016: Vnitr̆ní Lékar̆ství
Chaoyu Sun, Lei Tong, Wenran Zhao, Yan Wang, Yuan Meng, Lexun Lin, Bingchen Liu, Yujia Zhai, Zhaohua Zhong, Xueqi Li
Coxsackievirus B3 (CVB3) is a common causative agent in the development of inflammatory cardiomyopathy. However, whether the expression of peripheral blood microRNAs (miRNAs) is altered in this process is unknown. The present study investigated changes to miRNA expression in the peripheral blood of CVB3-infected mice. Utilizing miRNA microarray technology, differential miRNA expression was examined between normal and CVB3-infected mice. The present results suggest that specific miRNAs were differentially expressed in the peripheral blood of mice infected with CVB3, varying with infection duration...
October 2016: Experimental and Therapeutic Medicine
M Qasem, V Utomi, K George, J Somauroo, A Zaidi, L Forsythe, S Bhattacharrya, G Lloyd, B Rana, L Ring, S Robinson, R Senior, N Sheikh, M Sitali, J Sandoval, R Steeds, M Stout, J Willis, D Oxborough
INTRODUCTION: Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is an inherited pathology that can increase the risk of sudden death. Current Task Force Criteria for echocardiographic diagnosis do not include new, regional assessment tools which may be relevant in a phenotypically diverse disease. We adopted a systematic review and meta-analysis approach to highlight echocardiographic indices that differentiated ARVC patients and healthy controls. METHODS: Data was extracted and analysed from prospective trials that employed a case-control design meeting strict inclusion and exclusion as well as a-priori quality criteria...
September 29, 2016: Echo Research and Practice
Julius Bogomolovas, Jennifer R Fleming, Brian R Anderson, Rhys Williams, Stephan Lange, Bernd Simon, Muzamil M Khan, Rüdiger Rudolf, Barbara Franke, Belinda Bullard, Daniel J Rigden, Henk Granzier, Siegfried Labeit, Olga Mayans
Missense single-nucleotide polymorphisms (mSNPs) in titin are emerging as a main causative factor of heart failure. However, distinguishing between benign and disease-causing mSNPs is a substantial challenge. Here, we research the question of whether a single mSNP in a generic domain of titin can affect heart function as a whole and, if so, how. For this, we studied the mSNP T2850I, seemingly linked to arrhythmogenic right ventricular cardiomyopathy (ARVC). We used structural biology, computational simulations and transgenic muscle in vivo methods to track the effect of the mutation from the molecular to the organismal level...
September 2016: Open Biology
Christian Steinberg, Zachary W M Laksman, Andrew D Krahn
Sudden cardiac death (SCD) is still among the leading causes of death in women and men, accounting for over 50% of all fatal cardiovascular events in the United States. Two arrhythmia mechanisms of SCD can be distinguished as follows: shockable rhythms (ventricular fibrillation and pulseless ventricular tachycardia) and non-shockable rhythms including asystole or pulseless electrical activity. The overall prognosis of cardiac arrest due to shockable rhythms is significantly better. While the majority of SCDs is attributed to coronary artery disease or other structural heart disease, no obvious cause can be identified in 5% of all events, and those events are labeled as sudden unexplained deaths (SUD)...
November 2016: Trends in Cardiovascular Medicine
Masayuki Koyama, Toshiyuki Yano, Keisuke Kikuchi, Daigo Nagahara, Hatsue Ishibashi-Ueda, Tetsuji Miura
No abstract text is available yet for this article.
September 20, 2016: European Heart Journal
Christian Steinberg, Gareth J Padfield, Jean Champagne, Shubhayan Sanatani, Paul Angaran, Jason G Andrade, Jason D Roberts, Jeffrey S Healey, Vijay S Chauhan, David H Birnie, Mikyla Janzen, Brenda Gerull, George J Klein, Richard Leather, Christopher S Simpson, Colette Seifer, Mario Talajic, Martin Gardner, Andrew D Krahn
BACKGROUND: Unexplained cardiac arrest (UCA) may be explained by inherited arrhythmia syndromes. The Cardiac Arrest Survivors With Preserved Ejection Fraction Registry prospectively assessed first-degree relatives of UCA or sudden unexplained death victims to screen for cardiac abnormalities. METHODS AND RESULTS: Around 398 first-degree family members (186 UCA, 212 sudden unexplained death victims' relatives; mean age, 44±17 years) underwent extensive cardiac workup, including ECG, signal averaged ECG, exercise testing, cardiac imaging, Holter-monitoring, and selective provocative drug testing with epinephrine or procainamide...
September 2016: Circulation. Arrhythmia and Electrophysiology
Francesco Danilo Tiziano, Vincenzo Palmieri, Maurizio Genuardi, Paolo Zeppilli
Although relatively rare, inherited primitive cardiac disorders (IPCDs) in athletes have a deep social impact since they often present as sudden cardiac death (SCD) of young and otherwise healthy persons. The diagnosis of these conditions is likely underestimated due to the lack of shared clinical criteria and to the existence of several borderline clinical pictures. We will focus on the clinical and molecular diagnosis of the most common IPCDs, namely hypertrophic cardiomyopathies, long QT syndrome, arrhythmogenic right ventricular cardiomyopathy, and left ventricular non-compaction...
2016: Frontiers in Cardiovascular Medicine
Deniz Akdis, Corinna Brunckhorst, Firat Duru, Ardan M Saguner
This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes...
August 2016: Arrhythmia & Electrophysiology Review
Zhikui Wang, Zhaoxia Wang, Zhongqi Zhou, Yueqin Ren
BACKGROUND: This study sought to investigate crucial genes correlated with diabetic nephropathy (DN), and their potential functions, which might contribute to a better understanding of DN pathogenesis. METHODS: The microarray dataset GSE1009 was downloaded from Gene Expression Omnibus, including 3 diabetic glomeruli samples and 3 healthy glomeruli samples. The differentially expressed genes (DEGs) were identified by LIMMA package. Their potential functions were then analyzed by the GO and KEGG pathway enrichment analyses using the DAVID database...
2016: BMC Nephrology
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