Brugada syndrome and reduced right ventricular outflow tract conduction reserve: a final common pathway?

ER Behr, Y Ben-Haim, MJ Ackerman… - European heart …, 2021 - academic.oup.com
Brugada syndrome (BrS) was first described as a primary electrical disorder predisposing to
the risk of sudden cardiac death and characterized by right precordial lead ST elevation …

SCN5A channelopathy: arrhythmia, cardiomyopathy, epilepsy and beyond

CA Remme - … Transactions of the Royal Society B, 2023 - royalsocietypublishing.org
Influx of sodium ions through voltage-gated sodium channels in cardiomyocytes is essential
for proper electrical conduction within the heart. Both acquired conditions associated with …

International evidence based reappraisal of genes associated with arrhythmogenic right ventricular cardiomyopathy using the clinical genome resource framework

CA James, JDH Jongbloed… - Circulation: Genomic …, 2021 - Am Heart Assoc
Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited
disease characterized by ventricular arrhythmias and progressive ventricular dysfunction …

Brugada syndrome genetics is associated with phenotype severity

G Ciconte, MM Monasky, V Santinelli… - European heart …, 2021 - academic.oup.com
Abstract Aims Brugada syndrome (BrS) is associated with an increased risk of sudden
cardiac death due to ventricular tachycardia/fibrillation (VT/VF) in young, otherwise healthy …

Subepicardial cardiomyopathy: a disease underlying J-wave syndromes and idiopathic ventricular fibrillation

C Miles, BJ Boukens, C Scrocco, AAM Wilde… - Circulation, 2023 - Am Heart Assoc
Brugada syndrome (BrS), early repolarization syndrome (ERS), and idiopathic ventricular
fibrillation (iVF) have long been considered primary electrical disorders associated with …

Arrhythmias as presentation of genetic cardiomyopathy

J Lukas Laws, MC Lancaster… - Circulation …, 2022 - Am Heart Assoc
There is increasing evidence regarding the prevalence of genetic cardiomyopathies, for
which arrhythmias may be the first presentation. Ventricular and atrial arrhythmias …

The genetics of Brugada syndrome

M Cerrone, S Costa, M Delmar - Annual review of genomics and …, 2022 - annualreviews.org
Brugada syndrome is a heritable channelopathy characterized by a peculiar
electrocardiogram (ECG) pattern and increased risk of cardiac arrhythmias and sudden …

Prognostic value of nonischemic ringlike left ventricular scar in patients with apparently idiopathic nonsustained ventricular arrhythmias

D Muser, G Nucifora, D Muser, G Nucifora, M Pieroni… - Circulation, 2021 - Am Heart Assoc
Background: Left ventricular (LV) scar on late gadolinium enhancement (LGE) cardiac
magnetic resonance has been correlated with life-threatening arrhythmic events in patients …

Subcellular diversity of Nav1. 5 in cardiomyocytes: distinct functions, mechanisms and targets

GA Marchal, CA Remme - The Journal of Physiology, 2023 - Wiley Online Library
In cardiomyocytes, the rapid depolarisation of the membrane potential is mediated by the α‐
subunit of the cardiac voltage‐gated Na+ channel (NaV1. 5), encoded by the gene SCN5A …

Loss of nuclear envelope integrity and increased oxidant production cause DNA damage in adult hearts deficient in PKP2: a molecular substrate of ARVC

M Pérez-Hernández, CJM van Opbergen, N Bagwan… - Circulation, 2022 - Am Heart Assoc
Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by
high propensity to life-threatening arrhythmias and progressive loss of heart muscle. More …