Inherited cardiac arrhythmias

PJ Schwartz, MJ Ackerman, C Antzelevitch… - Nature reviews Disease …, 2020 - nature.com
The main inherited cardiac arrhythmias are long QT syndrome, short QT syndrome,
catecholaminergic polymorphic ventricular tachycardia and Brugada syndrome. These rare …

[HTML][HTML] Cardiac transmembrane ion channels and action potentials: cellular physiology and arrhythmogenic behavior

V András, J Tomek, N Nagy, L Virág… - Physiological …, 2021 - journals.physiology.org
Cardiac arrhythmias are among the leading causes of mortality. They often arise from
alterations in the electrophysiological properties of cardiac cells and their underlying ionic …

A comparative review on heart ion channels, action potentials and electrocardiogram in rodents and human: extrapolation of experimental insights to clinic

S Joukar - Laboratory Animal Research, 2021 - Springer
Electrocardiogram (ECG) is a non-invasive valuable diagnostic tool that is used in clinics for
investigation and monitoring of heart electrical rhythm/conduction, ischemia/injury of heart …

Animal models to study cardiac arrhythmias

DJ Blackwell, J Schmeckpeper… - Circulation research, 2022 - Am Heart Assoc
Cardiac arrhythmias are a significant cause of morbidity and mortality worldwide, accounting
for 10% to 15% of all deaths. Although most arrhythmias are due to acquired heart disease …

Pro-arrhythmic effects of gain-of-function potassium channel mutations in the short QT syndrome

JC Hancox, CY Du, A Butler… - … of the Royal …, 2023 - royalsocietypublishing.org
The congenital short QT syndrome (SQTS) is a rare condition characterized by abbreviated
rate-corrected QT (QTc) intervals on the electrocardiogram and by increased susceptibility to …

ESC working group on cardiac cellular electrophysiology position paper: relevance, opportunities, and limitations of experimental models for cardiac electrophysiology …

KE Odening, AM Gomez, D Dobrev, L Fabritz… - EP …, 2021 - academic.oup.com
Cardiac arrhythmias are a major cause of death and disability. A large number of
experimental cell and animal models have been developed to study arrhythmogenic …

[HTML][HTML] Utilizing human induced pluripotent stem cells to study atrial arrhythmias in the short QT syndrome

A Shiti, G Arbil, N Shaheen, I Huber, N Setter… - Journal of Molecular and …, 2023 - Elsevier
Background Among the monogenic inherited causes of atrial fibrillation is the short QT
syndrome (SQTS), a rare channelopathy causing atrial and ventricular arrhythmias. One of …

Sex-related differences in cardiac channelopathies: implications for clinical practice

B Asatryan, L Yee, Y Ben-Haim, S Dobner… - Circulation, 2021 - Am Heart Assoc
Sex-related differences in prevalence, clinical presentation, and outcome of cardiac
channelopathies are increasingly recognized, despite their autosomal transmission and …

A detailed analysis of single‐channel Nav1.5 recordings does not reveal any cooperative gating

Z Selimi, JS Rougier, H Abriel… - The Journal of …, 2023 - Wiley Online Library
Cardiac voltage‐gated sodium (Na+) channels (Nav1. 5) are crucial for myocardial electrical
excitation. Recent studies based on single‐channel recordings have suggested that Na+ …

Therapeutic efficacy of mexiletine for long QT syndrome type 2: evidence from human induced pluripotent stem cell–derived cardiomyocytes, transgenic rabbits, and …

L Crotti, R Neves, F Dagradi, G Musu, F Giannetti… - Circulation, 2024 - ahajournals.org
BACKGROUND: Despite major advances in the clinical management of long QT syndrome,
some patients are not fully protected by beta-blocker therapy. Mexiletine is a well-known …