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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.
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.
Abstract:
We report a 6-month-old Japanese infant with a malignant form of Brugada syndrome, who had frequent episodes of ventricular fibrillation (VF) and nonsustained polymorphic ventricular tachycardia (VT). To the best of our knowledge, this infant is the youngest patient reported to have Brugada syndrome. Continuous infusion of a beta-adrenergic agonist and intravenous injection of a parasympathetic antagonist suppressed the electrical storm of polymorphic VT and VF. Combined oral administration of a beta1-adrenergic agonist, a parasympathetic antagonist, and quinidine has successfully suppressed recurrences of VT or VF for 6 months, and the combination may have the potential to decrease the incidence of VT or VF as an adjunctive therapy with prophylactic placement of an implantable cardioverter defibrillator.