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

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.

• Authors: Vincent Probst, Stephane Evain, Veronique Gournay, Allouis Marie, Jean-Jacques Schott, Pierre Boisseau, Herve LE Marec
• Journal: Journal of cardiovascular electrophysiology (J Cardiovasc Electrophysiol) Vol. 17 Issue 1 Pg. 97-100 (Jan 2006) ISSN: 1045-3873 [Print] United States
• PMID: 16426410 (Publication Type: Case Reports, Journal Article)

Chemical References

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

Topics

• Anti-Arrhythmia Agents (therapeutic use)
• Bundle-Branch Block (complications, genetics, 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, therapeutic use)
• Sodium Channels (genetics)
• Syncope (complications, genetics, physiopathology)
• Syndrome
• Tachycardia, Ventricular (drug therapy, etiology, genetics)