ARVD/C and CPVT are rare inheritable sudden cardiac death syndromes predominantly expressed in younger individuals. ARVD/C is characterized by a progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia while CPVT is characterized by exercise induced bidirectional/polymorphic ventricular tachycardia (VT) and a structurally normal heart. A mutation in different genes causes these syndromes but recently, mutation in a common gene RYR2 has been associated with both disorders and it has been suggested that CPVT and ARVD/C represent a phenotypic spectrum. We present a case unique in expressing both these phenotypes.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.jelectrocard.2015.06.005 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardial condition that mostly affects the right ventricle (RV). Atrial involvement is poorly understood and the evidence for atrial involvement remains limited. In this case report, we describe an 18-year-old woman who had ARVC with the atrial tachyarrhythmia and initially presented with palpitations.
View Article and Find Full Text PDFThe role of cardiac sympathetic denervation for ventricular arrhythmias: an updated systematic review and meta-analysis.
J Interv Card Electrophysiol
January 2025
Rooney Heart Institute, 311 9th St N #201, Naples, FL, 34102, USA.
Introduction: The role of the sympathetic nervous system in the initiation and continuation of ventricular tachyarrhythmias (VTA) is well established. However, whether CSD reduces implantable cardioverter-defibrillator (ICD) shocks and recurrent VTA is still uncertain.
Methods: A comprehensive literature search was performed at Medline and Embase until March 2023.
β2-Adrenergic Receptor Agonist Clenbuterol Protects Against Acute Ischemia/Reperfusion-Induced Arrhythmia by Regulation of Akt/eNOS/NO/Cx43 Signaling Pathway.
Pharmacol Res Perspect
February 2025
Department of Pharmacology, School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Ventricular arrhythmias induced by ischemia/reperfusion injury limits the therapeutic effect of early reperfusion therapy for acute myocardial infarction. This study investigated the protective effects of the β2-adrenergic receptor (β2-AR) agonist clenbuterol against ischemia/reperfusion-induced arrhythmias and the underlying mechanism. Anesthetized rats were subjected to 10-min left coronary artery occlusion and 10-min reperfusion in vivo.
View Article and Find Full Text PDFFunctional Substrate Mapping: A New Frontier in the Treatment of Ventricular Tachycardia in Structural Heart Disease.
Arrhythm Electrophysiol Rev
December 2024
Heart Rhythm Research Group, Division of Biomedical Sciences, Warwick Medical School, Clinical Sciences Research Laboratory Coventry, UK.
Functional substrate mapping has emerged as an essential tool for electrophysiologists, overcoming many limitations of conventional mapping techniques and demonstrating favourable long-term outcomes in clinical studies. However, a consensus on the definition of 'functional substrate' mapping remains elusive, hindering a structured approach to research in the field. In this review, we highlight the differences between 'functional mapping' techniques (which assess tissue response to the 'electrophysiological stress' using short coupled extrastimuli) and those highlighting regions of slow conduction during sinus rhythm.
View Article and Find Full Text PDFDefining variances in practice for use of electrophysiology studies in risk stratification of patients with repaired tetralogy of Fallot: a PACES survey.
Cardiol Young
January 2025
Department of Medical Education and Clinical Sciences, Elson S Floyd College of Medicine, Washington State University, Spokane, WA, USA.
Sudden cardiac death poses a significant risk in patients with surgically repaired tetralogy of Fallot. Despite extensive research, risk stratification practices vary. This study surveyed the Pediatric and Adult Congenital Electrophysiology Society to identify these differences.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!