Related Experiment Video

Updated: Jan 3, 2026

Microelectrode Array Recording of Sinoatrial Node Firing Rate to Identify Intrinsic Cardiac Pacemaking Defects in Mice
09:20

Microelectrode Array Recording of Sinoatrial Node Firing Rate to Identify Intrinsic Cardiac Pacemaking Defects in Mice

Published on: July 5, 2021

3.4K

Clinical Profile and Long-Term Follow-Up of Children with Brugada Syndrome

Ibrahim El-Battrawy1,2, Gretje Roterberg3, Kim Schlentrich3

  • 1First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Theodor-Kutzer-Ufer 1-3, 68167, Mannheim, Germany. Ibrahim.el-battrawy@umm.de.

Pediatric Cardiology
|November 22, 2019
PubMed

Insights

Brugada syndrome (BrS) in children is rare but challenging to manage. Implantable cardioverter-defibrillator (ICD) therapy is effective for high-risk pediatric BrS patients, despite potential complications.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Brugada syndrome (BrS) is a rare genetic channelopathy linked to sudden cardiac death (SCD).
  • While adult BrS outcomes are documented, pediatric data remain limited.
  • Understanding pediatric BrS is crucial for timely diagnosis and management.

Purpose of the Study:

  • To analyze the clinical characteristics, treatment strategies, and long-term outcomes of pediatric Brugada syndrome.
  • To synthesize existing literature on childhood BrS for improved clinical guidance.

Main Methods:

  • Systematic literature review across major databases (PubMed, Web of Science, Cochrane Library, Cinahl).
  • Inclusion of 4 studies comprising 262 pediatric BrS patients.
  • Analysis of clinical presentation, ECG findings, electrophysiological studies, and treatment outcomes.

Main Results:

  • The mean patient age was 12.1 years; 53.8% were male.
  • 19.8% had spontaneous Type I BrS ECG, with 80.2% developing Type I after sodium channel blockers.
  • While 76% were asymptomatic, 17.9% experienced syncope; 1.5% presented with aborted SCD. ICDs were implanted in 56 children, with 16% experiencing appropriate shocks and 19.6% facing ICD-related complications.

Conclusions:

  • Pediatric Brugada syndrome presents unique diagnostic and management challenges.
  • Implantable cardioverter-defibrillator (ICD) therapy demonstrates efficacy in high-risk pediatric BrS patients.
  • Significant ICD-related complications necessitate careful consideration in pediatric BrS management.

Keywords:
BrugadaChildrenComplicationsPediatric

More Related Videos

Measuring Cardiac Autonomic Nervous System ANS Activity in Children09:45

Measuring Cardiac Autonomic Nervous System ANS Activity in Children

Published on: April 29, 2013

21.4K
Electrophysiological Analysis of human Pluripotent Stem Cell-derived Cardiomyocytes hPSC-CMs Using Multi-electrode Arrays MEAs11:13

Electrophysiological Analysis of human Pluripotent Stem Cell-derived Cardiomyocytes hPSC-CMs Using Multi-electrode Arrays MEAs

Published on: May 12, 2017

20.7K

Related Concept Videos

Acute Coronary Syndrome III: Diagnostic Studies01:30

Acute Coronary Syndrome III: Diagnostic Studies

Diagnosing acute coronary syndrome or ACS begins with a thorough patient history. Notable symptoms include central, crushing chest pain radiating to the left arm, neck, jaw, or back, along with shortness of breath, sweating (diaphoresis), nausea, vomiting, dizziness, and palpitations.It is crucial to note any history of cardiac illnesses and assess risk factors, including age, gender, smoking, hypertension, diabetes, hyperlipidemia, and a sedentary lifestyle.During physical examination, vital...
176
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
178
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
329
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
417
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
445
Dysrhythmias IV: Characteristics of Bradyarrhythmias01:18

Dysrhythmias IV: Characteristics of Bradyarrhythmias

Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...
425