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37 Successful insertion of an implantable cardioverter defibrillator in a 9 month old with out of hospital ventricular tachycardia who was subsequently diagnosed with brugada syndrome type 1: a case report
  1. L Rittey,
  2. D Hares,
  3. M Blackburn
  1. Department of Paediatric Cardiology, Leeds General Infirmary


Purpose Brugada syndrome is a well described cause of arrhythmias and can cause sudden death. It generally presents later in life or in late childhood following screening. The presentation of Brugada in a 9 month old infant as ventricular tachycardia is extremely rare.

The gold standard for treating Brugada is an implantable cardioverter defibrillator (ICD). This can be technically extremely challenging in small children.

Methods An ICD was inserted in the cardiac catheter lab jointly with the electrophysiology team and a congenital cardiac surgeon. An endocardial single coil lead was inserted into the apex of her right ventricle via her left subclavian vein. This was then tunnelled subcutaneously across her chest to a single chamber defibrillator generator (Boston Inogen Mini) in the left upper quadrant of her abdomen in a sub-muscular position.

Results The initial test shock, with induced ventricular fibrillation, at 16J was unsuccessful, however the second test at 26J was successful in cardioverting to sinus rhythm. Her device was therefore set up to provide a first shock if she has ventricular arrhythmias with a heart rate over 230 bpm at 26J and subsequent shocks at 35J if required. She was seen in clinic 2 months later and had no further episodes of arrhythmia and no inappropriate shocks.

Conclusion This case report shows that it is possible to successfully insert an ICD in a small child. For this child that could be lifesaving. To the best of our knowledge the positioning of this ICD is unique.

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