2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Developed by the task force for the …

K Zeppenfeld, J Tfelt-Hansen, M De Riva… - European heart …, 2022 - academic.oup.com
4004 ESC Guidelines label use of medication should be limited to situations where it is in
the patient's interest to do so, with regard to the quality, safety, and efficacy of care, and only …

Brugada syndrome

AD Krahn, ER Behr, R Hamilton, V Probst… - Clinical …, 2022 - jacc.org
Brugada syndrome (BrS) is an “inherited” condition characterized by predisposition to
syncope and cardiac arrest, predominantly during sleep. The prevalence is∼ 1: 2,000, and …

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 …

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 …

Genetic analysis of right heart structure and function in 40,000 people

JP Pirruccello, P Di Achille, V Nauffal, M Nekoui… - Nature …, 2022 - nature.com
Congenital heart diseases often involve maldevelopment of the evolutionarily recent right
heart chamber. To gain insight into right heart structure and function, we fine-tuned deep …

The complement C3-complement factor D-C3a receptor signalling axis regulates cardiac remodelling in right ventricular failure

S Ito, H Hashimoto, H Yamakawa, D Kusumoto… - Nature …, 2022 - nature.com
Failure of the right ventricle plays a critical role in any type of heart failure. However, the
mechanism remains unclear, and there is no specific therapy. Here, we show that the right …

Brugada syndrome: update and future perspectives

EMJ Marsman, PG Postema, CA Remme - Heart, 2022 - heart.bmj.com
Brugada syndrome (BrS) is an inherited cardiac disorder, characterised by a typical ECG
pattern and an increased risk of arrhythmias and sudden cardiac death (SCD). BrS is a …

Use, misuse, and pitfalls of the drug challenge test in the diagnosis of the Brugada syndrome

AAM Wilde, AS Amin, H Morita… - European Heart …, 2023 - academic.oup.com
The diagnosis of Brugada syndrome (BrS) requires the presence of a coved (Type 1) ST
segment elevation in the right precordial leads of the electrocardiogram (ECG). The dynamic …

Distinct Features of Probands With Early Repolarization and Brugada Syndromes Carrying SCN5A Pathogenic Variants

ZH Zhang, H Barajas-Martínez, H Xia, B Li… - Journal of the American …, 2021 - jacc.org
Background Two major forms of inherited J-wave syndrome (JWS) are recognized: early
repolarization syndrome (ERS) and Brugada syndrome (BrS). Objectives This study sought …

Biventricular myocardial fibrosis and sudden death in patients with Brugada syndrome

C Miles, A Asimaki, IC Ster, M Papadakis, B Gray… - Journal of the American …, 2021 - jacc.org
Background Electrophysiological, imaging, and pathological studies have reported the
presence of subtle structural abnormalities in hearts from patients with Brugada syndrome …