The Role of the Autonomic Nervous System in SIDS and SADS Shubhayan - - PowerPoint PPT Presentation

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The Role of the Autonomic Nervous System in SIDS and SADS Shubhayan - - PowerPoint PPT Presentation

Mar 10, 2024 480 likes •842 views

9 th Annual International SADS Foundation Conference San Diego The Role of the Autonomic Nervous System in SIDS and SADS Shubhayan Sanatani, MD, FRCPC, CCDS, FHRS Head, Division of Cardiology, BC Childrens Hospital Associate Professor,

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The Role of the Autonomic Nervous System in SIDS and SADS

Shubhayan Sanatani, MD, FRCPC, CCDS, FHRS

Head, Division of Cardiology, BC Children’s Hospital Associate Professor, Department of Pediatrics, University of British Columbia

Frances Perry, BSc Candidate

Children’s Heart Centre, BC Children’s Hospital

9th Annual International SADS Foundation Conference – San Diego

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SLIDE 2

Voluntary Attention Frontal Lobe

  • Frontal and orbital frontal areas,
  • Medial frontal lobe,
  • Dorsalateral frontal area, and
  • Premotor cortex

Involuntary Attention

  • Reticular formation and
  • Medulla oblongata
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Sympathetic Parasympathetic

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Shen Circ Res 2014

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Does the ANS play a role in SIDS?

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Sudden Infant Death Syndrome in History

Russell-Jones Arch Dis Child 1985

1st description of SIDS comes from the Old Testament “Judgment of Solomon”

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Sudden Infant Death Syndrome Timeline

1BC

Russell-Jones 1985

1291 Germany 1733 1188

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Sudden Infant Death Syndrome Timeline

Jacobs Radiology 1999; Russell-Jones 1985

1800’s 1924-47 US 1965 1889

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Sudden Infant Death Syndrome Today

Moon Lancet 2007

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SLIDE 10

Phillips J Physiol 2006

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SLIDE 11

TRIGGER

  • EXERCISE
  • EMOTIONAL STRESS
  • SLEEP
  • MEDICATION

BIOLOGICAL

SUBSTRATE

  • ABNORMAL REPOLARIZATION
  • REENTRY CIRCUITS
  • DISORDERED CALCIUM

HANDLING

THE THIRD FACTOR

? AUTONOMIC N.S. ? ELECTROLYTES ? PERFUSION ? ENVIRONMENT

?

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Sudden Infant Death Syndrome Incidence

Wilders ISRN Cardiology 2012

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Weese-Mayer Pediatr Res2004

Genes involved in early development of the autonomic nervous system

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SIDS and ANS polymorphisms

Schwartz NEJM 1998

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SIDS and ANS polymorphisms

Harper Pediatr Res 1978

SS = SIDS infants CT = controls

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“Smart Sock Hopes to Prevent Sudden Infant Death Syndrome”

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Can we learn from the common faint?

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Investigations

  • Normal sinus ECG
  • Normal 24hr Holter
  • Normal heart sounds, no murmurs
  • Normal exercise test

….Diagnosis?

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Incidence of syncope across a lifespan

Wieling Heart 2004

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Tachycardia Bradycardia Normal

Dysautonomia arrhythmogenesis

Goldstein Ann Intern Med 2002

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When to worry about syncope

  • Occurs during exertion without time to stop

activity

  • Syncope without typical prodromal symptoms
  • Occurs when sitting or lying down
  • Syncope without a clear/reproducible trigger
  • FHx of sudden cardiac arrest/death, or an

inheritable arrhythmia

  • Abnormal ECG
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SLIDE 23

Syncope in SADS

  • 10 yo girl with syncope and seizures while swimming
  • Prior to fainting: presyncope, dyspnea
  • Regained consciousness within 10s without

resuscitation

  • Remarkable family history:
  • Father with episodes of syncope since 10 yo
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SLIDE 24
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Swimming and LQTS

Albertella Arch Dis Child 2011

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ANS Modulation

Hou Heart Rhythm 2016

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SLIDE 28

ANS Modulation

Streeter Med Hypotheses 2012

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SLIDE 29

Left cardiac sympathetic denervation

Collura Heart Rhythm 2009

Indicated:

  • As second line of therapy

for CPVT, LQTS

  • Failure of B-blocker

therapy

  • Occurrence of

appropriate ICD shocks

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SLIDE 30

Left cardiac sympathetic denervation

Collura 2009

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ANS and Other Arrhythmias

Shen 2014

LQTS J wave Syndrome

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SLIDE 32

ANS and Other Arrhythmias

Shen 2014

Brugada Syndrome

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Closing thoughts

  • ANS plays a major role in arrhythmogenesis and sudden death
  • Imbalance of the sympathetic and parasympathetic
  • Polymorphisms in genes for ANS development in SIDS
  • Dysautonomia has a profound affect on HR
  • Modulation of the ANS can be used to treat arrhythmias
  • LCSD
  • Vagal nerve stimulation, carotid sinus stimulation
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SLIDE 34

Going forward…

  • Further study into relations of the ANS and inherited

arrhythmias is needed

  • Determine if ANS polymorphisms can reliably contribute to

risk stratification

  • Assess ANS modulation as a potential first line therapy for

inherited arrhythmias