Dysautonomia and POTS
Presented by Dr. Roy Maynard
October 2, 2013
Objectives
• Understand basics related to the
autonomic nervous system
• Describe normal orthostatic response in
transitio...
POTS

http://thewishfulartist.blogspot.com/2012_08_01_archive.html

Accessed on 8/5/13

3 of 36
Definitions
• Dysautonomia:- malfunction of the
autonomic nervous system
• POTS:- Postural Orthostatic Tachycardia
Syndrom...
Autonomic Nervous System
• Two Components
– Sympathetic
• Fight or flight

– Parasympathetic
• Rest or digest

5 of 36
Autonomic Nervous System
• Anatomy
– Lower brainstem
• Sympathetic T1- L3
• Parasympathetic S2-S4, Cranial Nerves 3, 7, 9,...
Autonomic Nervous System

http://www.womenhealthsite.com/images/ans-05.jpg

Accessed on 8/5/13

7 of 36
Normal Orthostatic Response
• Standing transfers more than 17 ounces of
blood into the abdomen and legs
• Initial period o...
Normal Orthostatic Response
• Mechanisms to restore hemodynamics
– Arterial vasoconstriction
– Elastic recoil of venous bl...
Normal Orthostatic Response

http://www.nymc.edu/fhp/centers/syncope/normal.htm

Accessed on 8/5/13

10 of 36
Douglas Bader

http://en.wikipedia.org/wiki/Supermarine_Spitfire

http://en.wikipedia.org/wiki/Douglas_Bader

Accessed on ...
G-Forces and the Fighter Pilot

http://www.gforces.com/military.html

Accessed on 8/5/13

12 of 36
Types of Orthostatic
Hypotension
• Initial Orthostatic Hypotension
• Orthostatic Hypotension
– Nonneurogenic orthostatic h...
diagnosis
• Tilt table

14 of 36
Initial Orthostatic
Hypotension

PEDIATRIC Journal, Volume 131,
Number 5, May 2013 – Page 969

15 of 36
Types of Orthostatic
Hypotension
• Initial Orthostatic Hypotension
• Orthostatic Hypotension
– Non-neurogenic orthostatic ...
Orthostatic Hypotension
• Non-neurogenic orthostatic hypotension
– Common in young
– Cause – drugs, dehydration, hemorrhag...
Orthostatic Hypotension
• Neurogenic orthostatic hypotension
– Rare in young people, no increase in heart
rate
– Associate...
Types of Orthostatic
Hypotension
• Initial Orthostatic Hypotension
• Orthostatic Hypotension
– Non-neurogenic orthostatic ...
https://www.facebook.com/pages/Postural-Orthostatic-Tachycardia-Syndrome-POTS/150392785031462

Accessed on 8/5/13

20 of 3...
Postural Orthostatic Tachycardia
Syndrome
• Definition: chronic day-to-day symptoms of
orthostatic intolerance plus excess...
Postural Orthostatic Tachycardia
Syndrome
• Clinical Presentation
– Palpitations
– Fatigue
– Lightheadedness
– Exercise in...
POTS

http://www.nymc.edu/fhp/centers/syncope/normal.htm

Accessed on 8/5/13

23 of 36
Epidemiology
• Females > Males 3:1 to 5:1; > 500,000 in 1999
• Triggers: Onset often follows flulike illness, selflimited ...
POTS

http://www.nymc.edu/fhp/centers/syncope/pots.htm

Accessed on 8/5/13

25 of 36
POTS
• Neuropathic POTS
– Cause- loss of regional vasoconstrictive ability
– Blood pooling in lower extremities
– Heart ra...
Treatment
• Neuropathic POTS- defect is loss of
regional vasoconstrictive ability
– Physical counter-manuevers
– Salt and ...
POTS
• Hyperadrenergic POTS
– Increased circulating norepinephrine
– May have increased orthostatic blood
pressure
– Less ...
Treatment
• Hyperadrenergic POTS:- Defect adrenergic
potentiation
– Physical counter-manuevers
– Beta-blockers
– Angiotens...
OUTCOME for patients with
POTS
• Adolescent-onset: 80% recover by mid
20’s
• Adult-onset: large percentage recover
within ...
Types of Orthostatic
Hypotension
• Initial Orthostatic Hypotension
• Orthostatic Hypotension
– Non-neurogenic orthostatic ...
Postural Vasovagal Syncope
• Most common form of orthostatic
intolerance
• Onset age 15, 2/3 are female
• 40% of the popul...
Treatment
• Postural vasovagal syncope- defect ?
Loss of regional vasoconstrictive ability,
acute reversible baroreflex dy...
Summary
• Orthostatic Intolerance defined by
symptoms when upright that are relieved
by lying down
• Orthostatic hypotensi...
Summary
• POTS is a form of chronic orthostatic
intolerance results
• (1) from a circulatory system not
responding to auto...
The End
• Thanks for coming.
• Questions?

36 of 36
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Dysautonomia and pots

  1. 1. Dysautonomia and POTS Presented by Dr. Roy Maynard October 2, 2013
  2. 2. Objectives • Understand basics related to the autonomic nervous system • Describe normal orthostatic response in transitioning from supine to upright position • Identify symptoms associated with POTS • Name the most common form of orthostatic intolerance 2 of 36
  3. 3. POTS http://thewishfulartist.blogspot.com/2012_08_01_archive.html Accessed on 8/5/13 3 of 36
  4. 4. Definitions • Dysautonomia:- malfunction of the autonomic nervous system • POTS:- Postural Orthostatic Tachycardia Syndrome 4 of 36
  5. 5. Autonomic Nervous System • Two Components – Sympathetic • Fight or flight – Parasympathetic • Rest or digest 5 of 36
  6. 6. Autonomic Nervous System • Anatomy – Lower brainstem • Sympathetic T1- L3 • Parasympathetic S2-S4, Cranial Nerves 3, 7, 9, 10 • Function – – – – Subconscious level Visceral functions Heart rate, respiration, blood pressure Digestion, micturation, perspiration, salivation, pupillary dilatation – Dilatation and constriction of blood vessels (arteries and veins) 6 of 36
  7. 7. Autonomic Nervous System http://www.womenhealthsite.com/images/ans-05.jpg Accessed on 8/5/13 7 of 36
  8. 8. Normal Orthostatic Response • Standing transfers more than 17 ounces of blood into the abdomen and legs • Initial period of instability (initial orthostatic hypotension) ensues and blood pressure may decrease by 30% for 10 to 20 seconds after standing • Reflex tachycardia happens • Blood pressure restored within 30 to 60 seconds 8 of 36
  9. 9. Normal Orthostatic Response • Mechanisms to restore hemodynamics – Arterial vasoconstriction – Elastic recoil of venous blood in dependent veins – Active venoconstriction in splanchnic veins 9 of 36
  10. 10. Normal Orthostatic Response http://www.nymc.edu/fhp/centers/syncope/normal.htm Accessed on 8/5/13 10 of 36
  11. 11. Douglas Bader http://en.wikipedia.org/wiki/Supermarine_Spitfire http://en.wikipedia.org/wiki/Douglas_Bader Accessed on 8/5/13 Accessed on 8/5/13 11 of 36
  12. 12. G-Forces and the Fighter Pilot http://www.gforces.com/military.html Accessed on 8/5/13 12 of 36
  13. 13. Types of Orthostatic Hypotension • Initial Orthostatic Hypotension • Orthostatic Hypotension – Nonneurogenic orthostatic hypotension – Neurogenic orthostatic hypotension • POTS – Neuropathic POTS – Hyperadrenergic POTS • Vasovagal Syncope (VVS) 13 of 36
  14. 14. diagnosis • Tilt table 14 of 36
  15. 15. Initial Orthostatic Hypotension PEDIATRIC Journal, Volume 131, Number 5, May 2013 – Page 969 15 of 36
  16. 16. Types of Orthostatic Hypotension • Initial Orthostatic Hypotension • Orthostatic Hypotension – Non-neurogenic orthostatic hypotension – Neurogenic orthostatic hypotension • POTS – Neuropathic POTS – Hyperadrenergic POTS • Postural Vasovagal syncope 16 of 36
  17. 17. Orthostatic Hypotension • Non-neurogenic orthostatic hypotension – Common in young – Cause – drugs, dehydration, hemorrhage – Mechanism- incomplete autonomic nervous system compensation for stressors – Treatment- stop medication, prevent dehydration 17 of 36
  18. 18. Orthostatic Hypotension • Neurogenic orthostatic hypotension – Rare in young people, no increase in heart rate – Associated with diabetes, amyloidosis, Parkinson’s disease – Cause- autonomic vasoconstrictor failure – Treatment- physical counter-maneuvers, • Medications- IV saline, corticosteroids, midodrine, droxidopa 18 of 36
  19. 19. Types of Orthostatic Hypotension • Initial Orthostatic Hypotension • Orthostatic Hypotension – Non-neurogenic orthostatic hypotension – Neurogenic orthostatic hypotension • POTS – Neuropathic POTS – Hyperadrenergic POTS • Postural Vasovagal syncope 19 of 36
  20. 20. https://www.facebook.com/pages/Postural-Orthostatic-Tachycardia-Syndrome-POTS/150392785031462 Accessed on 8/5/13 20 of 36
  21. 21. Postural Orthostatic Tachycardia Syndrome • Definition: chronic day-to-day symptoms of orthostatic intolerance plus excessive increase in heart rate when upright – HR>30 from baseline or >120 after 10 minutes during tilt test in adults – HR>40 from baseline in children and teens – Blood pressure usually not low • Cause: alterations in autonomic nervous system 21 of 36
  22. 22. Postural Orthostatic Tachycardia Syndrome • Clinical Presentation – Palpitations – Fatigue – Lightheadedness – Exercise intolerance – Nausea – Headache – Syncope/near syncope 22 of 36
  23. 23. POTS http://www.nymc.edu/fhp/centers/syncope/normal.htm Accessed on 8/5/13 23 of 36
  24. 24. Epidemiology • Females > Males 3:1 to 5:1; > 500,000 in 1999 • Triggers: Onset often follows flulike illness, selflimited autoimmune disease, surgery or injury, pregnancy, rapid growth spurt • May be associated with joint hypermobility syndromes • Young women may be underweight; need to differentiate from eating disorders • Hot weather or hot showers worsens symptoms • Cognitive function may be affected 24 of 36
  25. 25. POTS http://www.nymc.edu/fhp/centers/syncope/pots.htm Accessed on 8/5/13 25 of 36
  26. 26. POTS • Neuropathic POTS – Cause- loss of regional vasoconstrictive ability – Blood pooling in lower extremities – Heart rate increases to compensate for decreased circulating blood volume now pooling in lower extremities 26 of 36
  27. 27. Treatment • Neuropathic POTS- defect is loss of regional vasoconstrictive ability – Physical counter-manuevers – Salt and water loading – Midodrine – Mestinon – Exercise – Rapid water ingestion 27 of 36
  28. 28. POTS • Hyperadrenergic POTS – Increased circulating norepinephrine – May have increased orthostatic blood pressure – Less common than neuropathic form – Symptoms attributed- anxiety, tremor, cold sweaty extremities 28 of 36
  29. 29. Treatment • Hyperadrenergic POTS:- Defect adrenergic potentiation – Physical counter-manuevers – Beta-blockers – Angiotension receptor blocker – Fludrocortisone 29 of 36
  30. 30. OUTCOME for patients with POTS • Adolescent-onset: 80% recover by mid 20’s • Adult-onset: large percentage recover within 2-5 years • Some patients will suffer from long term disability associated with POTS 30 of 36
  31. 31. Types of Orthostatic Hypotension • Initial Orthostatic Hypotension • Orthostatic Hypotension – Non-neurogenic orthostatic hypotension – Neurogenic orthostatic hypotension • POTS – Neuropathic POTS – Hyperadrenergic POTS • Postural Vasovagal syncope 31 of 36
  32. 32. Postural Vasovagal Syncope • Most common form of orthostatic intolerance • Onset age 15, 2/3 are female • 40% of the population has had at least one episode in their lifetime 32 of 36
  33. 33. Treatment • Postural vasovagal syncope- defect ? Loss of regional vasoconstrictive ability, acute reversible baroreflex dysfunction – Physical counter manuevers – Salt and water – Acute water ingestion – Midodrine – Rapid water ingestion 33 of 36
  34. 34. Summary • Orthostatic Intolerance defined by symptoms when upright that are relieved by lying down • Orthostatic hypotension is a form of orthostatic intolerance • Initial orthostatic hypotension is a common form of orthostatic intolerance in the young • Postural vasovagal syncope is the most common form of orthostatic intolerance 34 of 36
  35. 35. Summary • POTS is a form of chronic orthostatic intolerance results • (1) from a circulatory system not responding to autonomic inputs properly • (2) hyperadrenergic state • (3) chronic bed rest 35 of 36
  36. 36. The End • Thanks for coming. • Questions? 36 of 36
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