The current prognostic stratification of asymptomatic patients with Brugada syndrome is suboptimal. The so-called 'Brugada burden' concept is certainly emerging: the more extensive are the electrocardiographic alterations of the syndrome in space (peripheral as well as precordial derivations) and in time (persistence in the follow-up of electrocardiographic alterations), the greater the probability of arrhythmic events. Numerous clinical and electrocardiographic markers have been considered risk factors, but none of them alone is able to guide the choice of whether or not to implant a defibrillator, the only therapy so far proved effective in preventing SD (sudden death) in these patients. The prognostic value of the electrophysiology study also gradually decreased over time. Therapeutic decisions must therefore be taken, at the moment, considering a large number of variables, possibly included in risk scores to be validated prospectively and in large series. Magnetic resonance and the study of electro-anatomical alterations of the right ventricular outflow tract will most likely improve our prognostic stratification capacity in the future.
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http://dx.doi.org/10.1093/eurheartjsupp/suac088 | 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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