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Infant case with a malignant form of Brugada syndrome

H Suzuki1, K Torigoe, O Numata

  • 1Department of Pediatrics, Nagaoka Red Cross Hospital, Nagaoka, Japan.

Journal of Cardiovascular Electrophysiology
|November 18, 2000
PubMed

Insights

This study details the youngest infant diagnosed with Brugada syndrome, experiencing ventricular fibrillation (VF) and polymorphic ventricular tachycardia (VT). Treatment with beta-adrenergic agonists, parasympathetic antagonists, and quinidine successfully managed the condition.

Area of Science:

  • Pediatric Cardiology
  • Electrophysiology
  • Genetic Cardiology

Background:

  • Brugada syndrome is a rare genetic disorder affecting heart rhythm.
  • Malignant forms can manifest early in infancy, posing significant risk.

Observation:

  • A 6-month-old Japanese infant presented with frequent ventricular fibrillation (VF) and nonsustained polymorphic ventricular tachycardia (VT).
  • This represents the youngest reported case of Brugada syndrome.

Findings:

  • Continuous infusion of a beta-adrenergic agonist and a parasympathetic antagonist temporarily suppressed the electrical storm.
  • Combined oral therapy with a beta1-adrenergic agonist, parasympathetic antagonist, and quinidine achieved 6-month suppression of VT/VF recurrences.

Implications:

  • This therapeutic combination shows potential for managing malignant Brugada syndrome in infants.
  • Adjunctive therapy with an implantable cardioverter-defibrillator may further reduce VT/VF incidence.

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