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Atrial Arrhythmia and Bradycardia as a Presentation of Congenital Long QT Syndrome.
Pediatr Cardiol
July 2024
Department of Pediatric Cardiology, University of Illinois, Chicago, USA.
We present a term newborn with atrial arrhythmia on the first day of life (DOL). An echocardiogram showed normal structure and normal function; laboratory testing showed normal electrolytes and thyroid function. After initiation of flecainide, the EKG on DOL 2 showed significant and increasing bradycardia with atrial arrhythmia and extremely prolonged QTc interval.
View Article and Find Full Text PDFAn inherited life-threatening arrhythmia model established by screening randomly mutagenized mice.
Proc Natl Acad Sci U S A
April 2024
International Institute for Integrative Sleep Medicine, University of Tsukuba, Tsukuba 305-8575, Japan.
Inherited arrhythmia syndromes (IASs) can cause life-threatening arrhythmias and are responsible for a significant proportion of sudden cardiac deaths (SCDs). Despite progress in the development of devices to prevent SCDs, the precise molecular mechanisms that induce detrimental arrhythmias remain to be fully investigated, and more effective therapies are desirable. In the present study, we screened a large-scale randomly mutagenized mouse library by electrocardiography to establish a disease model of IASs and consequently found one pedigree that exhibited spontaneous ventricular arrhythmias (VAs) followed by SCD within 1 y after birth.
View Article and Find Full Text PDFClass 1C Antiarrhythmics for Premature Ventricular Complex Suppression in Nonischemic Cardiomyopathy With Implantable Cardioverter-Defibrillators.
JACC Clin Electrophysiol
May 2024
Electrophysiology Section, Division of Cardiovascular Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA. Electronic address:
Background: Premature ventricular complexes (PVCs) are common and associated with worse outcomes in patients with heart failure. Class 1C antiarrhythmic drugs (AADs) effectively suppress PVCs, but guidelines currently restrict their use in structural heart disease.
Objectives: This study aimed to assess the safety and efficacy of class 1C AADs in patients with nonischemic cardiomyopathy (NICM) and implantable cardioverter-defibrillators (ICDs).
-Mutant Cardiomyocyte Model of Multifocal Atrial Tachycardia.
Circ Arrhythm Electrophysiol
April 2024
Mindich Child Health & Development Institute (N.A.R., N.P., S.N., A.G.A., M.R., B.D.G.), Icahn School of Medicine at Mount Sinai, New York, NY.
Background: Germline gain-of-function pathogenic variants cause Costello syndrome (CS). During early childhood, 50% of patients develop multifocal atrial tachycardia, a treatment-resistant tachyarrhythmia of unknown pathogenesis. This study investigated how overactive HRAS activity triggers arrhythmogenesis in atrial-like cardiomyocytes (ACMs) derived from human-induced pluripotent stem cells bearing CS-associated variants.
View Article and Find Full Text PDFBackground: We identified a novel variant, E171Q, in a neonate with very frequent ectopy and reduced ejection fraction which normalized after arrhythmia suppression by flecainide. This clinical picture is consistent with multifocal ectopic Purkinje-related premature contractions (MEPPC). Most previous reports of MEPPC have implicated variants such as R222Q that neutralize positive charges in the S4 voltage sensor helix of the channel protein Na1.
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