Introduction: Inducibility of ventricular arrhythmias at programmed electrical stimulation (PES) ranges between 50% and 80% of patients with Brugada syndrome. However, the variety of PES protocols and the lack of data relative to a control group or to ventricular arrhythmia reproducibility contribute to a still undefined interpretation of PES outcome in Brugada syndrome.
Methods And Results: Twenty-one patients with Brugada syndrome (18 men and 3 women; mean age 34 years; 9/21 symptomatic; 8/21 with SCN5A gene mutation) underwent a PES protocol from two right ventricular sites. The endpoint was PES protocol completion or induction of sustained or reproducible (>6 consecutive inductions) nonsustained (>6 beats) fast ventricular arrhythmia. In 17 of 21 patients with Brugada syndrome, PES was repeated 2 months later to test ventricular arrhythmia reproducibility. Twenty-five healthy patients (17 men; mean age 36 years) formed the control group. In patients with Brugada syndrome, ventricular arrhythmia inducibility rate at PES was high (18/21 patients [85%]) and increased with protocol aggressiveness, independent of clinical presentation. In control subjects, no ventricular arrhythmias were induced. Among patients with Brugada syndrome, 14 (82%) of 17 patients remained inducible at a second PES.
Conclusion: In our experience, ventricular arrhythmia inducibility in patients with Brugada syndrome, at variance with healthy controls, is high and does not correlate with clinical presentation. PES inducibility is deeply influenced by the protocol used. PES outcome is reproducible at a mid-term follow-up mainly if a categorical endpoint (inducible vs noninducible) is used. The need to assess the predictive value of specific PES protocols in targeted studies is widely emerging and is confirmed by our results.
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http://dx.doi.org/10.1046/j.1540-8167.2002.00880.x | 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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