PAC Conference – Pflaumer on Sudden Death in the young

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We have teamed up with APLS to share the videos from their Paediatric Acute Care Conferences. These videos have never been open access before, so if you weren’t able to attend the conferences, then now’s your chance to catch up.

The PAC Conference is run each year by APLS and consists of presentations on a range of topics relevant to paediatric acute and critical care.

Andreas Pflaumer is a Consultant Cardiologist and Electrophysiologist at the Royal Children’s Hospital Melbourne, a senior lecturer at the University of Melbourne and an Honorary Fellow at the MCRI. Before 2009 he was working as a Senior Physician at the Department for Paediatric Cardiology and Congenital Heart Disease in the German Heart Centre, Munich. Since 2004 his work has focused on paediatric electrophysiology (integration of 3D anatomic information into mapping systems), sudden death in the young and cardiac-resuscitation therapy. He is the co-founder of EURIPIDES and a member of AEPC, ESC, PACES, HRS and AHA.

  • Sudden cardiac death (SCD) <10 years old is more prevalent than accidents
  • About 5-10 per 100,000 children per year for SUDI/SIDS, 90% of these are infants <1 year old
  • SUDI accounts for about 40-80 sudden unexpected deaths in Australia each year (excluding SIDS)
  • There are no accurate statistics around aborted sudden death
  • Of note, HOCM is not as prevalent in Australia as first thought
  • Arrhythmias cannot be assessed in postmortem and, at best, are assumed
  • SCD is due to electrical causes, muscular cardiomyopathies, or structural heat diseases
  • SCD is relevant to the family of a deceased child, as there is a high risk of heritability
  • “The only difference between syncope and sudden death is that in syncope you wake up.” – GL Engel Ann Int Med 1978
  • Family history should include unwitnessed drownings (or similar events) in the young
  • Prof Pflamuer gives a really nice explanation of the delta wave representing the anatomic lesion in Wolf-Parkinson-White syndrome
  • Remember that the QTc segment is a range – there is likely some overlap between the LQTS and normal range, around 400-480ms
  • LQTS1 is associated with exercise syncope/arrest, LQTS2 is associated with startle-arrest pattern, LQTS3 is associated with arrest in sleep
  • A stress ECG for LQTS1 show increasing prolongation of the QT segment

The presentation also covers other causes of sudden cardiac death and aborted sudden death in children, including:

  • Short QT syndrome, which is compared and contrasted with both normal and long QT syndromes
  • Bidirectional ventricular tachycardia – also known as catecholamine provoked VT – a calcium channel disease
  • Brugada syndrome and the importance of observing a febrile child with Brugada syndrome due to the increased risk of ventricular tachycardia
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About 

A Paediatric Trainee based in Queensland, Australia, Henry is passionate about Adolescent Medicine & General Paediatrics, with a strong interest in Medical Education & Clinical Teaching. An admitted nerd & ironman with a penchant for Rubik's Cubes & 'Dad jokes'.

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