title: Cavotricuspid isthmus ablation and subcutaneous monitoring device implantation in a 2-year-old baby with 2 SCN5A mutations, sinus node dysfunction, atrial flutter recurrences, and drug induced long-QT syndrome: a tricky case of pediatric overlap syndrome?
abstract: We describe the case of 2-year-old baby with compound heterozygosity for paternal and maternal alleles mutation of α-subunit of the cardiac sodium channel (SCN5A), sinus node dysfunction, atrial flutter recurrences, and drug induced long-QT syndrome. In this setting, we chose at first to perform linear ablation of cavotricuspid isthmus resulting in a bidirectional isthmus block. As a second step, we decided to implant a miniaturized loop recorder that, with a minimally invasive procedure, permits us to follow the development of the disease in order to define the future strategy. After 8 months follow-up, automatic daily loop-recorder transmissions disclose the complete absence of any arrhythmia along with asymptomatic ventricular pauses due to sinus node dysfunction. Echocardiography shows normal findings, in particular no left ventricular dysfunction.
authors.affiliation: Cardiac Electrophysiology and Pacing Unit, Cardiovascular Department, Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: De Filippo
authors.forename: Paola
authors.initials: P
authors.lastname: Ferrari
authors.forename: Maria
authors.initials: M
authors.lastname: Iascone
authors.forename: Marco
authors.initials: M
authors.lastname: Racheli
authors.forename: Michele
authors.initials: M
authors.lastname: Senni
mesh_terms: D001282:Atrial Flutter / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D017115:Catheter Ablation / Q000379:methods; D002675:Child, Preschool; D015716:Electrocardiography, Ambulatory / Q000379:methods*; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D009154:Mutation / Q000235:genetics; D062554:NAV1.5 Voltage-Gated Sodium Channel / Q000235:genetics; D012804:Sick Sinus Syndrome / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D040521:Subcutaneous Tissue; D014261:Tricuspid Valve / Q000601:surgery
chemical_list: D062554:NAV1.5 Voltage-Gated Sodium Channel; C568320:SCN5A protein, human
keywords: SCN5A; atrial flutter; catheter ablation; children; implantable loop recorders