A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern

ABSTRACT 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 pedia...

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Main Authors: Hei‐To Leung, Sit‐Yee Kwok, Fong‐Ying Shih, Kin‐Shing Lun, Tak‐Cheung Yung, Sabrina Tsao
Format: Article
Language:English
Published: Wiley 2025-01-01
Series:Annals of Noninvasive Electrocardiology
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Online Access:https://doi.org/10.1111/anec.70009
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author Hei‐To Leung
Sit‐Yee Kwok
Fong‐Ying Shih
Kin‐Shing Lun
Tak‐Cheung Yung
Sabrina Tsao
author_facet Hei‐To Leung
Sit‐Yee Kwok
Fong‐Ying Shih
Kin‐Shing Lun
Tak‐Cheung Yung
Sabrina Tsao
author_sort Hei‐To Leung
collection DOAJ
description ABSTRACT 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. The diagnosis of BrS was established with a type 1 ECG pattern detected spontaneously or induced by flecainide, excluding secondary causes. Results Eight probands of mean age 10 years old were identified. Male dominance was observed (6 boys vs. 2 girls). The mean follow‐up duration was 4.6 years (Median 3.5 years). Patients had type 1 ECG pattern either spontaneously (n = 4) or provoked by flecainide (n = 4). Fever was present in seven patients at the initial presentation, and two patients experienced aborted cardiac arrest and one had symptomatic ventricular tachycardia. All symptomatic patients received implantable cardioverter‐defibrillator placement. Five asymptomatic patients (62.5%) were diagnosed with BrS through ECG during febrile illness, and they remained asymptomatic following conservative management involving strict fever control and medication avoidance. Two patients with mixed phenotype (one with long QT syndrome and another with ectopic atrial tachycardia) required antiarrhythmics and one patient received transcatheter ablation for atrial tachycardia to achieve optimal arrhythmia control. Conclusion Fever plays a significant role in unmasking BrS in children. Asymptomatic children with BrS managed conservatively have a favorable prognosis. Difficult arrhythmia control was found in patients with mixed phenotype.
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spelling doaj-art-f6416561f5404bf3bc9f839c621798472025-01-28T05:38:41ZengWileyAnnals of Noninvasive Electrocardiology1082-720X1542-474X2025-01-01301n/an/a10.1111/anec.70009A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG PatternHei‐To Leung0Sit‐Yee Kwok1Fong‐Ying Shih2Kin‐Shing Lun3Tak‐Cheung Yung4Sabrina Tsao5Department of Paediatrics and Adolescent Medicine Hong Kong Children's Hospital Ngau Tau Kok Hong KongDepartment of Paediatrics and Adolescent Medicine Hong Kong Children's Hospital Ngau Tau Kok Hong KongClinical Genetics Service Unit Hong Kong Children's Hospital Ngau Tau Kok Hong KongDepartment of Paediatrics and Adolescent Medicine Hong Kong Children's Hospital Ngau Tau Kok Hong KongDepartment of Paediatrics and Adolescent Medicine Hong Kong Children's Hospital Ngau Tau Kok Hong KongDepartment of Paediatrics and Adolescent Medicine The University of Hong Kong Pok Fu Lam Hong KongABSTRACT 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. The diagnosis of BrS was established with a type 1 ECG pattern detected spontaneously or induced by flecainide, excluding secondary causes. Results Eight probands of mean age 10 years old were identified. Male dominance was observed (6 boys vs. 2 girls). The mean follow‐up duration was 4.6 years (Median 3.5 years). Patients had type 1 ECG pattern either spontaneously (n = 4) or provoked by flecainide (n = 4). Fever was present in seven patients at the initial presentation, and two patients experienced aborted cardiac arrest and one had symptomatic ventricular tachycardia. All symptomatic patients received implantable cardioverter‐defibrillator placement. Five asymptomatic patients (62.5%) were diagnosed with BrS through ECG during febrile illness, and they remained asymptomatic following conservative management involving strict fever control and medication avoidance. Two patients with mixed phenotype (one with long QT syndrome and another with ectopic atrial tachycardia) required antiarrhythmics and one patient received transcatheter ablation for atrial tachycardia to achieve optimal arrhythmia control. Conclusion Fever plays a significant role in unmasking BrS in children. Asymptomatic children with BrS managed conservatively have a favorable prognosis. Difficult arrhythmia control was found in patients with mixed phenotype.https://doi.org/10.1111/anec.70009coexisting arrhythmiafocal atrial tachycardialong QT syndromepediatric Brugada syndromesubcutaneous ICD
spellingShingle Hei‐To Leung
Sit‐Yee Kwok
Fong‐Ying Shih
Kin‐Shing Lun
Tak‐Cheung Yung
Sabrina Tsao
A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern
Annals of Noninvasive Electrocardiology
coexisting arrhythmia
focal atrial tachycardia
long QT syndrome
pediatric Brugada syndrome
subcutaneous ICD
title A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern
title_full A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern
title_fullStr A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern
title_full_unstemmed A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern
title_short A Tale of Two Conditions: Pediatric Brugada Syndrome Unveiled—Navigating the Challenges of Coexisting Arrhythmia and Fever‐Induced ECG Pattern
title_sort tale of two conditions pediatric brugada syndrome unveiled navigating the challenges of coexisting arrhythmia and fever induced ecg pattern
topic coexisting arrhythmia
focal atrial tachycardia
long QT syndrome
pediatric Brugada syndrome
subcutaneous ICD
url https://doi.org/10.1111/anec.70009
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