A successfully novel ICD implantation and medical treatment in a child with LQT syndrome and self-limiting ventricular fibrillation

Int J Cardiol. 2007 Jun 12;118(3):e108-12. doi: 10.1016/j.ijcard.2007.01.066. Epub 2007 Apr 3.


Beta-blocker is the first line drug therapy for congenital long QT syndrome. However, in some children this drug is ineffective. In a non-responder patient, Shimizu et al. used Mexiletine to suppress the ventricular arrhythmias, obtaining a good result. In the high risk patient, the ICD is necessary. However the implantation of a device in small children can have technical problems. We report a case of a child affected by long QT syndrome with recurrent episodes of syncope due to self-limiting torsade de point/ventricular fibrillation, successfully treated by an association of mexiletin and propanolol, and in whom an ICD was implanted with a new subcutaneous approach.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Anti-Arrhythmia Agents / therapeutic use*
  • Child, Preschool
  • Combined Modality Therapy
  • Defibrillators, Implantable*
  • Electrocardiography
  • Electrocardiography, Ambulatory
  • Humans
  • Long QT Syndrome / complications*
  • Long QT Syndrome / congenital*
  • Male
  • Prognosis
  • Propranolol / therapeutic use
  • Risk Assessment
  • Severity of Illness Index
  • Treatment Outcome
  • Ventricular Fibrillation / diagnosis
  • Ventricular Fibrillation / etiology
  • Ventricular Fibrillation / therapy*


  • Anti-Arrhythmia Agents
  • Propranolol