title: 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.affiliation: Department of Cardiology and Unité INSERM U533, Institut du thorax, CHU de Nantes, Nantes, France. vincent.probst@chu-nantes.fr
authors.forename: Vincent
authors.initials: V
authors.lastname: Probst
authors.forename: Stephane
authors.initials: S
authors.lastname: Evain
authors.forename: Veronique
authors.initials: V
authors.lastname: Gournay
authors.forename: Allouis
authors.initials: A
authors.lastname: Marie
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Schott
authors.forename: Pierre
authors.initials: P
authors.lastname: Boisseau
authors.forename: Herve
authors.initials: H
authors.lastname: LE Marec
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D002037:Bundle-Branch Block / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D002675:Child, Preschool; D004247:DNA / Q000235:genetics; D004252:DNA Mutational Analysis; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006339:Heart Rate; D006801:Humans; D009124:Muscle Proteins / Q000235:genetics; D009154:Mutation; D062554:NAV1.5 Voltage-Gated Sodium Channel; D011802:Quinidine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D015222:Sodium Channels / Q000235:genetics; D013575:Syncope / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D013577:Syndrome; D017180:Tachycardia, Ventricular / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics
chemical_list: D000889:Anti-Arrhythmia Agents; D009124:Muscle Proteins; D062554:NAV1.5 Voltage-Gated Sodium Channel; C568320:SCN5A protein, human; D015222:Sodium Channels; C014486:hydroquinidine; D004247:DNA; D011802:Quinidine