A DIAGNOSIS TO BE EVOKED: The Brugada syndrome is a rare but serious inherited disease that causes sudden cardiac death by ventricular tachycardia and fibrillation, especially in younger men. Diagnostic problems are related to the possible absence of symptoms although the electrocardiogram (ECG) reveals the characteristics of a Brugada syndrome and to the variations in the ECG in the same patient over time. THREE ELECTROCARDIOLOGICAL ASPECTS: Type 1 corresponds to the historical description with ST segment elevation at point J of at least 3 mm from its summit and upward convex ST segment followed by a negative T wave. In Type 2, the extent of point I is of 2 mm, the ST segment has a saddle form and remains at least 1 mm above the isoelectric line, the T wave is positive or biphasic. In Type 3, the terminal section of the ST segment never exceeds 1 mm above the isoelectric line. In the case of a Type 1 ECG, a pharmacodynamic test is of no interest. REGARDING TREATMENT: The only treatment to have demonstrated its efficacy is the implantable automatic defibrillator, indicated in symptomatic patients.
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http://dx.doi.org/10.1016/s0755-4982(04)98753-2 | DOI Listing |
J Korean Med Sci
January 2025
Division of Cardiology, Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.
Background: The ionic mechanism underlying Brugada syndrome (BrS) arises from an imbalance in transient outward current flow between the epicardium and endocardium. Previous studies report that artemisinin, originally derived from a Chinese herb for antimalarial use, inhibits the Ito current in canines. In a prior study, we showed the antiarrhythmic effects of artemisinin in BrS wedge preparation models.
View Article and Find Full Text PDFJ Mol Cell Cardiol
January 2025
Department of Biochemistry, University of Cambridge, Tennis Court Road, Cambridge CB2 1QW, UK. Electronic address:
Introduction: Brugada Syndrome (BrS) is an inherited arrhythmia syndrome characterised by ST-segment elevation in the right precordial ECG leads and is associated with an increased risk of sudden cardiac death. We identify and characterise a novel SCN3B variant encoding the regulatory β3-subunit of the cardiac voltage-gated sodium channel, Na1.5.
View Article and Find Full Text PDFA A Pract
January 2025
Division of Anaesthesiology and Perioperative Medicine, Department of Pain Medicine, Singapore General Hospital, Singapore.
Cureus
December 2024
Pediatric Cardiology, Children's Hospital at Montefiore, Bronx, USA.
Brugada syndrome (BrS) is a genetic channelopathy that may predispose to ventricular arrhythmia. It is inherited as an autosomal dominant pattern with incomplete penetrance. Fever can unmask Brugada syndrome in children who have a genetic predisposition.
View Article and Find Full Text PDFAnn Noninvasive Electrocardiol
January 2025
Department of Paediatrics and Adolescent Medicine, The University of Hong Kong, Pok Fu Lam, Hong Kong.
Background: Brugada syndrome (BrS) is an inherited channelopathy characterized by right precordial ST-segment elevation. This study investigates the clinical and genetic characteristics of children with BrS in Hong Kong.
Methods: A retrospective review was conducted at the only tertiary pediatric cardiology center in Hong Kong from 2002 to 2022, including all pediatric BrS patients under 18 years old.
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