Present Status of Brugada Syndrome: JACC State-of-the-Art Review

J Brugada, O Campuzano, E Arbelo… - Journal of the American …, 2018 - jacc.org
The Brugada syndrome is an inherited disorder associated with risk of ventricular fibrillation
and sudden cardiac death in a structurally normal heart. Diagnosis is based on a …

Clinical Spectrum of SCN5A Mutations: Long QT Syndrome, Brugada Syndrome, and Cardiomyopathy

AAM Wilde, AS Amin - JACC: Clinical Electrophysiology, 2018 - jacc.org
SCN5A gene encodes the pore-forming ion-conducting α-subunit of the cardiac sodium
channel (Nav1. 5), which is responsible for the initiation and propagation of action potentials …

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 …

Fir (e) ing the rhythm: inflammatory cytokines and cardiac arrhythmias

PE Lazzerini, A Abbate, M Boutjdir… - Basic to Translational …, 2023 - jacc.org
Inflammatory activation is increasingly recognized as a nonconventional risk factor for
arrhythmias, and experimental studies provided robust evidence that this association is …

Proteostasis in cardiac health and disease

RH Henning, BJJM Brundel - Nature Reviews Cardiology, 2017 - nature.com
The incidence and prevalence of cardiac diseases, which are the main cause of death
worldwide, are likely to increase because of population ageing. Prevailing theories about …

Emerging arrhythmic risk of autoimmune and inflammatory cardiac channelopathies

PE Lazzerini, PL Capecchi, N El‐Sherif… - Journal of the …, 2018 - Am Heart Assoc
Cardiac arrhythmias are associated with high morbidity and mortality. 1 Specifically,
malignant arrhythmias are a recognized leading cause of sudden cardiac death (SCD) in the …

The definition of the Brugada syndrome

J Sieira, P Brugada - European heart journal, 2017 - academic.oup.com
Brugada syndrome (BS) is an inherited disease characterized by a coved-type ST-segment
elevation in the right precordial leads and increased risk of sudden cardiac death (SCD), in …

Patient-specific iPSC-derived cardiomyocytes reveal variable phenotypic severity of Brugada syndrome

Y Sun, J Su, X Wang, J Wang, F Guo, H Qiu, H Fan… - …, 2023 - thelancet.com
Summary Background Brugada syndrome (BrS) is a cardiac channelopathy that can result in
sudden cardiac death (SCD). SCN5A is the most frequent gene linked to BrS, but the …

A deep learning–enabled electrocardiogram model for the identification of a rare inherited arrhythmia: Brugada syndrome

CM Liu, CL Liu, KW Hu, VS Tseng, SL Chang… - Canadian Journal of …, 2022 - Elsevier
Background Brugada syndrome is a major cause of sudden cardiac death in young people
and has distinctive electrocardiographic (ECG) features. We aimed to develop a deep …

Dysfunctional Nav1. 5 channels due to SCN5A mutations

D Han, H Tan, C Sun, G Li - Experimental Biology and …, 2018 - journals.sagepub.com
The voltage-gated sodium channel 1.5 (Nav1. 5), encoded by the SCN5A gene, is
responsible for the rising phase of the action potential of cardiomyocytes. The sodium …