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Brugada syndrome in children

Jane E Crosson1, Melanie Nies

  • 1Pediatric Cardiology, Bloomberg Children's Center, Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, MD 21287, USA.

Expert Review of Cardiovascular Therapy
|January 14, 2015
PubMed

Insights

Brugada syndrome, an inherited heart rhythm disorder, rarely affects children but can cause sudden death. Increased awareness and genetic testing may lead to more pediatric diagnoses.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Brugada syndrome is a genetic arrhythmia linked to ST elevation and sudden cardiac death.
  • Sudden cardiac death typically occurs around age 40, with pediatric cases being uncommon.
  • Early diagnosis and intervention are crucial for managing inherited cardiac conditions.

Purpose of the Study:

  • To highlight the rarity of Brugada syndrome in pediatric populations.
  • To emphasize the potential impact of genetic testing and increased awareness on pediatric diagnoses.
  • To underscore the importance of recognizing Brugada syndrome in children.

Main Methods:

  • Review of existing literature on Brugada syndrome.
  • Analysis of reported pediatric cases and their outcomes.
  • Discussion of diagnostic advancements, including genetic testing.

Main Results:

  • Brugada syndrome is predominantly diagnosed in adults, with pediatric cases being infrequent.
  • Characteristic ECG findings include ST elevation in right precordial leads.
  • Genetic mutations are a key factor in the inheritance of Brugada syndrome.

Conclusions:

  • Pediatric Brugada syndrome, though rare, requires heightened clinical suspicion.
  • Advances in genetic testing are expected to increase the identification of Brugada syndrome in children.
  • Prompt diagnosis and management are vital to prevent sudden cardiac death in affected children.

Keywords:
Brugada syndromeECGSCN5AST elevationchildrendefibrillatorgenetic syndromequinidinesudden deathventricular tachycardia

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