Brugada syndrome is increasingly recognized as a cause of sudden cardiac death. Many of these patients do not get diagnosed due its dynamic and often hidden nature. We have come a long way in understanding the disease process, and its electrophysiology appears to be intimately linked with sodium channel mutations or disorders. The cardiac rhythm in these patients can deteriorate into fatal ventricular arrhythmias. This makes it important for the clinician to be aware of the conditions in which arrhythmogenicity of Brugada syndrome is revealed or even potentiated. We present such an instance where our patient's Brugada syndrome was unmasked by fever.
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http://dx.doi.org/10.1177/2324709615577414 | DOI Listing |
JACC Clin Electrophysiol
January 2025
National Cerebral and Cardiovascular Center, Suita, Japan.
JACC Clin Electrophysiol
January 2025
Fondazione Toscana "Gabriele Monasterio," Pisa, Italy; Scuola Superiore Sant'Anna, Pisa, Italy. Electronic address:
Eur Heart J Case Rep
January 2025
Echocardiography Department, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK.
Background: Superior caval vein obstruction is a rare complication of endocardial pacing lead implantation that can result in a right to left shunt.
Case Summary: A 3-year-old child with type 2 Brugada syndrome presented with mild cyanosis post-endocardial pacing implantation due to evolutionary right superior caval vein obstruction. This obstruction resulted in a right to left shunt across a previously unrecognized patent levo-atrial cardinal vein associated with partial anomalous pulmonary venous drainage.
Pacing Clin Electrophysiol
January 2025
Department of Cardiovascular Medicine, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Background: This study aimed to compare inappropriate shock (IAS) rates between subcutaneous implantable cardioverter-defibrillator (S-ICD) and transvenous ICD (TV-ICD) in Brugada syndrome (BrS) patients and identify risk factors for IAS in S-ICD use.
Methods: We enrolled consecutive patients with BrS who underwent ICD implantation between 2013 and 2023. Data on clinical characteristics, S-ICD screening test data, and IAS occurrence were retrospectively analyzed.
Pediatr Neurol
January 2025
Faculty of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain; Pediatrics Research Group, Institut de Recerca Sant Pau (IR-Sant Pau), Barcelona, Spain; Pediatric Neurology Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Background: Dravet syndrome (DS) is a severe developmental and epileptic encephalopathy associated with loss-of-function variants in the SCN1A gene. Although predominantly expressed in the central nervous system, SCN1A is also expressed in the heart, suggesting a potential link between neuronal and cardiac channelopathies. Additionally, DS carries a high risk of sudden unexpected death in epilepsy (SUDEP).
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