A 35-year effective treatment of catecholaminergic polymorphic ventricular tachycardia with propafenone
Sign Up to like & getrecommendations! Published in 2019 at "HeartRhythm Case Reports"
DOI: 10.1016/j.hrcr.2018.04.003
Abstract: Key Teaching Points • Despite proven catecholaminergic polymorphic ventricular tachycardia (CPVT) with pathogen RyR2 mutation and recurrent syncope, patients could have a favorable long-term outcome over 35 years under treatment. • Propafenone could be effective… read more here.
Keywords: ventricular tachycardia; effective treatment; polymorphic ventricular; treatment ... See more keywords
Scared to death—A novel mutation in catecholaminergic polymorphic ventricular tachycardia
Sign Up to like & getrecommendations! Published in 2020 at "HeartRhythm Case Reports"
DOI: 10.1016/j.hrcr.2020.01.009
Abstract: The decision to implant an implantable cardioverter-defibrillator in patients with CPVT is challenging, as there are no randomized controlled Introduction Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare familial cardiac arrhythmia that results in bidirectional… read more here.
Keywords: death; ventricular tachycardia; polymorphic ventricular; catecholaminergic polymorphic ... See more keywords
Sudden death after inappropriate shocks of implantable cardioverter defibrillator in a catecholaminergic polymorphic ventricular tachycardia case with a novel RyR2 mutation.
Sign Up to like & getrecommendations! Published in 2021 at "Journal of electrocardiology"
DOI: 10.1016/j.jelectrocard.2021.09.015
Abstract: BACKGROUND Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic syndrome and a cause of exercise-related sudden death. CPVT has been reported to be caused by gain of function underlying a mutation of cardiac ryanodine… read more here.
Keywords: sudden death; ryr2 mutation; ryr2; catecholaminergic polymorphic ... See more keywords
Documenting the descent – remote monitoring and adult-onset Catecholaminergic Polymorphic Ventricular Tachycardia associated with ventricular fibrillation and bradycardia
Sign Up to like & getrecommendations! Published in 2021 at "Cardiology in the Young"
DOI: 10.1017/s1047951121002377
Abstract: Abstract This image highlights a 38-year-old female with ventricular fibrillation and spontaneous return to sinus rhythm found on an implantable loop recorder inserted for recurrent syncope. Ultimately, she was diagnosed with catecholaminergic polymorphic ventricular tachycardia,… read more here.
Keywords: ventricular tachycardia; polymorphic ventricular; catecholaminergic polymorphic; ventricular fibrillation ... See more keywords
Catecholaminergic Polymorphic Ventricular Tachycardia.
Sign Up to like & getrecommendations! Published in 2017 at "Pediatric emergency care"
DOI: 10.1097/01.pec.0000520710.58614.76
Abstract: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a condition characterized by an abnormal heart rhythm (arrhythmia). As the heart rate increases in response to physical activity or emotional stress, it can trigger an abnormally fast and… read more here.
Keywords: polymorphic ventricular; catecholaminergic polymorphic; ventricular tachycardia; tachycardia ... See more keywords
A gain of function ryanodine receptor 2 mutation (R1760W‐RyR2) in catecholaminergic polymorphic ventricular tachycardia
Sign Up to like & getrecommendations! Published in 2022 at "Clinical and Experimental Pharmacology and Physiology"
DOI: 10.1111/1440-1681.13722
Abstract: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome associated with Ca2+ leak predominantly caused by ryanodine receptor 2 (RyR2) mutations. We identified a R1760W‐RyR2 mutation located between the N‐terminal domain and the central… read more here.
Keywords: polymorphic ventricular; catecholaminergic polymorphic; r1760w ryr2; ryr2 ... See more keywords
Efficacy of RyR2 inhibitor EL20 in induced pluripotent stem cell‐derived cardiomyocytes from a patient with catecholaminergic polymorphic ventricular tachycardia
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Cellular and Molecular Medicine"
DOI: 10.1111/jcmm.16521
Abstract: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac arrhythmia syndrome that often leads to sudden cardiac death. The most common form of CPVT is caused by autosomal‐dominant variants in the cardiac ryanodine receptor type‐2… read more here.
Keywords: el20; ventricular tachycardia; polymorphic ventricular; ryr2 ... See more keywords
Nationwide experience of catecholaminergic polymorphic ventricular tachycardia caused by RyR2 mutations
Sign Up to like & getrecommendations! Published in 2017 at "Heart"
DOI: 10.1136/heartjnl-2016-310509
Abstract: Objective The aim of this study was to characterise disease penetrance, course of disease and use of antiarrhythmic medication and implantable cardioverter-defibrillator (ICD) therapy in a Danish nationwide cohort of patients with catecholaminergic polymorphic ventricular… read more here.
Keywords: nationwide experience; ventricular tachycardia; polymorphic ventricular; disease ... See more keywords
Antiarrhythmic Effects of Carvedilol and Flecainide in Cardiomyocytes Derived from Catecholaminergic Polymorphic Ventricular Tachycardia Patients
Sign Up to like & getrecommendations! Published in 2018 at "Stem Cells International"
DOI: 10.1155/2018/9109503
Abstract: Mutations in the cardiac ryanodine receptor (RYR2) are the leading cause for catecholaminergic polymorphic ventricular tachycardia (CPVT). In this study, we evaluated antiarrhythmic efficacy of carvedilol and flecainide in CPVT patient-specific induced pluripotent stem cell-derived… read more here.
Keywords: carvedilol flecainide; ventricular tachycardia; polymorphic ventricular; ipsc cms ... See more keywords
Novel Calmodulin Variant p.E46K Associated With Severe Catecholaminergic Polymorphic Ventricular Tachycardia Produces Robust Arrhythmogenicity in Human Induced Pluripotent Stem Cell–Derived Cardiomyocytes
Sign Up to like & getrecommendations! Published in 2023 at "Circulation: Arrhythmia and Electrophysiology"
DOI: 10.1161/circep.122.011387
Abstract: Background: CaM (calmodulin) is a ubiquitously expressed, multifunctional Ca2+ sensor protein that regulates numerous proteins. Recently, CaM missense variants have been identified in patients with malignant inherited arrhythmias, such as long QT syndrome and catecholaminergic… read more here.
Keywords: polymorphic ventricular; catecholaminergic polymorphic; ventricular tachycardia; cam ... See more keywords
Insights into the Pathogenesis of Catecholaminergic Polymorphic Ventricular Tachycardia from Engineered Human Heart Tissue.
Sign Up to like & getrecommendations! Published in 2019 at "Circulation"
DOI: 10.1161/circulationaha.119.039711
Abstract: BACKGROUND Modeling of human arrhythmias using induced pluripotent stem cell-derived cardiomyocytes has focused on single cell phenotypes. However, arrhythmias are the emergent properties of cells assembled into tissues, and the impact of inherited arrhythmia mutations… read more here.
Keywords: heart tissue; human heart; catecholaminergic polymorphic; tissue ... See more keywords