Brugada syndrome is an inherited disorder characterized by a channelopathy of cardiac sodium, potassium, and calcium channel. The pathophysiology of this disorder is not completely elucidated yet, however, most of the reported cases are caused by a pathogenic alteration in the SCN5A gene, leading to the malfunction of cardiac sodium channels. Several stressors are well known to unmask this pathology including fever and electrolytes imbalance. Three ECG patterns are frequently described in the literature, type 1, type 2, and type 3. However, only the type 1 pattern is considered diagnostic of Brugada syndrome in the appropriate clinical context. Therapeutic strategies can range from conservative medical management with antiarrhythmic medications to Automatic Implantable Cardioverter Defibrillator (AICD) placement. Prompt recognition is of utmost importance since this pathology can rapidly evolve into life-threatening arrhythmias and sudden cardiac death. Here we present a case of a 22-year-old male who presented after a syncopal episode and was found to have Brugada syndrome in the setting of Influenza A infection.
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http://dx.doi.org/10.55729/2000-9666.1187 | DOI Listing |
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).
View Article and Find Full Text PDFBackground: Placement of right precordial leads in higher intercostal spaces (EEP-ECG) improves the detection of Brugada Syndrome (BrS). Given the potential difficulty of lead placement and the transient nature of BrS ECG patterns, we developed a model to predict EEP-ECG from a standard 12‑lead ECG.
Objective: To create and validate a model that derives EEP-ECG leads from a standard 12‑lead ECG.
Biomedicines
December 2024
Clinical and Rehabilitation Cardiology Division, San Filippo Neri Hospital, 00135 Rome, Italy.
Brugada syndrome (BrS) is an inherited arrhythmogenic disorder characterized by distinct electrocardiographic patterns and an increased risk of sudden cardiac death due to ventricular arrhythmias. Effective management of BrS is essential, particularly for high-risk patients with recurrent arrhythmias. While implantable cardioverter-defibrillator (ICD) is effective in terminating life-threatening arrhythmias, it does not prevent arrhythmia onset and can lead to complications such as inappropriate shocks.
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