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Acute paediatrics
Near-miss SIDS due to Brugada syndrome
  1. J R Skinner1,
  2. S-K Chung3,
  3. D Montgomery4,
  4. C H McCulley3,
  5. J Crawford2,
  6. J French2,
  7. M I Rees2
  1. 1Department of Paediatric Cardiology, Starship Hospital, Grafton, Auckland, New Zealand
  2. 2Cardiac Inherited Disease Group, Auckland Hospital, Grafton, Auckland, New Zealand
  3. 3Department of Molecular Medicine, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand
  4. 4Department of Emergency Paediatrics, Kidz First Hospital, Middlemore, Auckland, New Zealand
  1. Correspondence to:
    Dr J R Skinner
    Department of Paediatric Cardiology, Starship Hospital, Grafton, Auckland, New Zealand; JSkinneradhb.govt.nz

Abstract

A 19 day old infant was successfully resuscitated from ventricular fibrillation. The 12 lead ECG was normal, with a normal QT interval, and remains so over three years follow up. DNA analysis revealed a missense mutation (R1193Q) in the SCN5A gene, previously linked with familial sudden unexpected nocturnal death syndrome, also known as Brugada syndrome.

  • ICD, implantable cardioverter defibrillator
  • LQTS, long QT syndrome
  • SIDS, sudden infant death syndrome
  • SUNDS, sudden unexpected nocturnal death syndrome
  • VF, ventricular fibrillation
  • Brugada syndrome
  • cardiac channelopathy
  • long QT syndrome
  • SIDS
  • SUNDS

https://doi.org/10.1136/adc.2004.058115

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    Footnotes

    • Competing interests: none declared