Inherited arrhythmias in the pediatric population: an updated overview

MV Mariani, N Pierucci, F Fanisio, D Laviola, G Silvetti… - Medicina, 2024 - mdpi.com
Pediatric cardiomyopathies (CMs) and electrical diseases constitute a heterogeneous
spectrum of disorders distinguished by structural and electrical abnormalities in the heart …

RYR2-ryanodinopathies: from calcium overload to calcium deficiency

C Steinberg, TM Roston, C van der Werf, S Sanatani… - Europace, 2023 - academic.oup.com
The sarcoplasmatic reticulum (SR) cardiac ryanodine receptor/calcium release channel
RyR2 is an essential regulator of cardiac excitation–contraction coupling and intracellular …

An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic …

A Lamba, TM Roston, PJ Peltenburg, D Kallas… - Heart rhythm, 2024 - Elsevier
Background Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause
sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter …

Flecainide is associated with a lower incidence of arrhythmic events in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia

AT Bergeman, KVV Lieve, D Kallas, JM Bos… - Circulation, 2023 - Am Heart Assoc
BACKGROUND: In severely affected patients with catecholaminergic polymorphic
ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this …

Clinical genetics of inherited arrhythmogenic disease in the pediatric population

E Martínez-Barrios, S Cesar, J Cruzalegui… - Biomedicines, 2022 - mdpi.com
Sudden death is a rare event in the pediatric population but with a social shock due to its
presentation as the first symptom in previously healthy children. Comprehensive autopsy in …

Pediatric catecholaminergic polymorphic ventricular tachycardia: a translational perspective for the clinician-scientist

D Kallas, A Lamba, TM Roston, A Arslanova… - International journal of …, 2021 - mdpi.com
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and potentially
lethal inherited arrhythmia disease characterized by exercise or emotion-induced …

Gene therapy for Catecholaminergic polymorphic ventricular tachycardia

PR Pérez, RJ Hylind, TM Roston, VJ Bezzerides… - Heart, Lung and …, 2023 - Elsevier
Over the last three decades, the genetic basis of various inherited arrhythmia syndromes
has been elucidated, providing key insights into cardiomyocyte biology and various …

[HTML][HTML] Treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia: a single institutional experience

J Lee, BS Kwon, MK Song, SY Lee… - Korean Circulation …, 2024 - pmc.ncbi.nlm.nih.gov
Background and Objectives Catecholaminergic polymorphic ventricular tachycardia (CPVT)
is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVT …

Catecholaminergic Polymorphic Ventricular Tachycardia: A Review of Therapeutic Strategies

AT Bergeman, AAM Wilde… - Cardiac …, 2023 - cardiacep.theclinics.com
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare malignant inherited
arrhythmia syndrome, characterized by bidirectional or polymorphic ventricular arrhythmia …

Catecholaminergic Polymorphic Ventricular Tachycardia: Clinical Characteristics, Diagnostic Evaluation and Therapeutic Strategies

A Aggarwal, A Stolear, MM Alam, S Vardhan… - Journal of Clinical …, 2024 - mdpi.com
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe hereditary
arrhythmia syndrome predominantly affecting children and young adults. It manifests …