Key result
Initial symptoms in pediatric suspected Brugada syndrome are linked to ~11-fold higher major arrhythmic risk.
Why the study?
Diagnosis and treatment of children and adolescents with suspected Brugada syndrome (BrS) is challenging, leaving the applicability of current diagnostic criteria and clinical outcomes uncertain.
Cohort (n=76)
Yes
In pediatric patients with suspected Brugada syndrome, major arrhythmic events occurred in 5.3% of the cohort, with symptoms at first consultation serving as a strong independent risk factor.
Answers are grounded in this paper and cite their sources.
May flag higher-risk pediatric Brugada suspects for closer monitoring; hypothesis-generating and needs prospective validation before guiding decisions.
Introduction Diagnosis and treatment of children and adolescents with suspected Brugada syndrome (BrS) is challenging. Aim of the study was to evaluate the applicability of current diagnostic criteria for pediatric patients with suspected BrS in the German multicenter study COGIA and to assess clinical outcome. Methods Data analysis of 76 pediatric patients from 12 German tertiary care centers. Primary outcome was defined by the occurrence of a major arrhythmic event (MAE), secondary outcome by symptoms, pharmacotherapy and implantation of devices. MAE was defined as sudden cardiac death (SCD), aborted cardiac arrest (ACA) and appropriate implantable cardioverter‐defibrillator (ICD) therapy. Results Criteria for BrS diagnosis were fulfilled in 39/76 pediatric patients (51.3%) according to ESC Guidelines, including 34 patients (44.7%) according to Shanghai Score. Genetic testing identified a (likely) pathogenic SCN5A variant in 31/66 tested children (47.0%) (14/30 with clinical diagnosis (46.7%) and 17/36 without clinical diagnosis (47.2%)). Twenty‐four patients (31.6%) showed a spontaneous type 1 ECG pattern, in 6 patients (7.9%) it was induced by fever. Ajmaline challenge was positive in 19/25 patients (76.0%). Syncope occurred in 15 patients (19.7%) and an ICD was implanted in 7 pediatric patients (9.2%). Four patients (5.3%) experienced a MAE (ACA), all of them fulfilling diagnostic criteria. “Symptoms at first consultation” was identified as an independent risk factor for MAE in the whole cohort (HR = 11.3, p = 0.04). Conclusion Establishing the diagnosis of BrS remains challenging in the pediatric cohort. MAE occurred in 5.3% of patients with suspected BrS. Symptoms at first consultation increased risk for MAE. Clinical Trial Registration: The study is registered at the German Clinical Trial Register (DRKS) of the Federal Institute for Drugs and Medical Devices (BfArM), DRKS‐ID DRKS00028138.
No takes yet. Share an insight, caveat, or question.
Lippert et al. (2026) conducted a cohort in Suspected Brugada syndrome (n=76). Symptoms at first consultation was evaluated on Major arrhythmic event (MAE) defined as sudden cardiac death, aborted cardiac arrest, and appropriate ICD therapy (HR 11.3, p=0.04). In pediatric patients with suspected Brugada syndrome, presenting with symptoms at first consultation significantly increased the risk of major arrhythmic events (HR 11.3; p=0.04).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: