Monomorphic ventricular tachycardia due to Brugada syndrome successfully treated by hydroquinidine therapy in a 3-year-old child

J Cardiovasc Electrophysiol. 2006 Jan;17(1):97-100. doi: 10.1111/j.1540-8167.2005.00329.x.

Abstract

Mutations in the SCN5A gene can cause Brugada syndrome, a genetically inherited form of idiopathic ventricular fibrillation. We describe the case of a 3-year-old child with a structurally normal heart presenting with monomorphic ventricular tachycardia. Her electrocardiogram suggested a Brugada syndrome and the diagnosis was confirmed by the identification of a Brugada syndrome in her mother and in two other family members. Genetic study led to the identification of a c.2516T-->C SCN5A mutation. The child was treated with quinidine therapy without recurrence of arrhythmic events for a time period of 16 months.

Publication types

  • Case Reports

MeSH terms

  • Anti-Arrhythmia Agents / therapeutic use*
  • Bundle-Branch Block / complications*
  • Bundle-Branch Block / genetics
  • Bundle-Branch Block / physiopathology
  • Child, Preschool
  • DNA / genetics
  • DNA Mutational Analysis
  • Electrocardiography
  • Female
  • Follow-Up Studies
  • Heart Rate
  • Humans
  • Muscle Proteins / genetics
  • Mutation
  • NAV1.5 Voltage-Gated Sodium Channel
  • Quinidine / analogs & derivatives*
  • Quinidine / therapeutic use
  • Sodium Channels / genetics
  • Syncope / complications*
  • Syncope / genetics
  • Syncope / physiopathology
  • Syndrome
  • Tachycardia, Ventricular / drug therapy*
  • Tachycardia, Ventricular / etiology
  • Tachycardia, Ventricular / genetics

Substances

  • Anti-Arrhythmia Agents
  • Muscle Proteins
  • NAV1.5 Voltage-Gated Sodium Channel
  • SCN5A protein, human
  • Sodium Channels
  • hydroquinidine
  • DNA
  • Quinidine