irrelevant disclosures: too many to list…. · tachycardia syndrome cardioinhibitory syncope* both...
TRANSCRIPT
Diagnosis and Management of Dysautonomia in the
Pediatric Population David M Bush, MD, PhD
Pediatric Cardiology/Electrophysiology Pediatric Cardiology Associates of San Antonio
Adjunct Associate Professor of Pediatrics University of Texas Health Science Center San Antonio
Disclosures
Relevant Disclosures: None. Irrelevant Disclosures: Too Many to List….
“What is wanted is not the will to believe, but
the will to find out, which is the exact
opposite.”
Bertrand Russell 1872-1970
http://www.goodreads.com/author/show/17854.Bertrand_Russell
On Discovery… Considerations
• Dysautonomia is both acknowledged and controversial
• Dysautonomia has been poorly studied
• Dysautonomia is one of the more common conditions you will confront in the late child and adolescent age group
My Goals for Giving This Talk
• Pass on personal knowledge/anecdotes
• Stimulate interest in research in the area
• Convince you I am not totally crazy
… Only Partially
Where We Are Going
• Explore the Normal Physiology of the Autonomic Nervous System & Possible Pathophysiology
• Describe Age-Specific Autonomic Conditions – Clinical Presentation – Diagnostic Criteria/Evaluation – Treatment Options
• Prognostic Thoughts
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Autonomic Nervous Physiology
• A part of the “primitive” central/peripheral nervous system
• Responsible for regulating subconscious homeostatic processes within the body and making them response to both internal and external stimuli
Autonomic Nervous
Physiology
www.merkmanuals.com
… Primary Effects
Autonomic Nervous Physiology
Secondary Effects
Cerebral Autoregulation Digestive Control
Thermal Autoregulation
Tertiary Effects Fatigue/Sleep
Decreased Sense of Well Being
www.uic.edu
Autonomic Nervous Physiology
• Gestational age correlates with HR variability – Longin E, et al 2006 – DeRogalski Landrot I, et al 2007
• HR variability correlates with sleep parameters and gestational age – Clairambault J, et al 1992
Evidence in Favor of Developmental Maturation
Longin E, et al. J Perinat Medicine 2006;34(4):303. DeRogalski Landrot I, et al. Auton Neurosci 2007; 136(1-2):105 Clairambault J, et al. Ear Human Develop 1992;28(2):169
Autonomic Nervous Physiology
• Maturation is a process of increased parasympathetic control – Eyre EL, et al 2014
• Disorders in other organ systems (such as OSA) can affect sleep and HR variability through enhanced sympathetic activity – Walter LM, et al 2015
Eyre EL, et al. Auton Neurosci 2014; 186:8 Walter LM, et al. Sleep Breath 2015; Oct 2 (epub)
Framework for Autonomic (Cardiovascular) Disorders
Persistent Dysautonomia Episodic Dysautonomia
Blood Pressure Orthostatic Hypotension Vasovagal Syncope
Heart Rate Postural Orthostatic Tachycardia Syndrome
Cardioinhibitory Syncope*
Both Mixed Mixed
* Known as “Pallid Infantile Syncope” in the newborn
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Episodic Dysautonomia
www. youtube.com
Pallid Infantile Findings
Indian Pacing Electrophysiol. J. 2009;9(5):283
Seizures and Syncope Infantile Correlates
www. Youtube.com (Tobi Scott)
Pallid versus Breatholding Syncope
Pallid Syncope Cyanotic Syncope
Age of Onset 2 -12 mo 6-48 mo
Appearance White Blue
Precipitated by Crying Occasional Frequent
Tonic–Clonic Activity Frequent Common
Mechanism Asystole Hypoxia/Bradycardia
Treatment Pacing ? Reflux Treatment ? Stimulants
Reassurance ? Iron ? Stimulants
Prognosis Resolution by 6 yrs age Resolution by 4-6 yrs age
Toddler/School-Age Presentations
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Toddler/School-Age Presentations
• Episodic Dysautonomia – Syncope/Convulsions – Pre-syncope (Pallor, Inattention) – Activity Avoidance – School Performance Issues
• Persistent Dysautonomia (?)
Pediatric/Adolescent Triggers
• Postural changes • Noxious stimuli – Internal – External
• Post-exertion
Episodic Dysautonomia
• Prolonged stasis • Stress/Excitement • Panic/Hypocarbia • Micturition/Defecati
on
Adolescent Presentations
“Infectious Form” “Developmental Form”
Onset Characteristic Explosive Gradual
Age of Onset 12-21 years 4-16 years
Initial Symptoms Fatigue, Pre-Syncope Non-specific complaints “Stomach Aches”
Persistent Dysautonomia
Associated Disorders/Syndromes
• Connective Tissue Disorders – Ehler-Danlos syndrome – Loey-Dietz syndrome – Marfan syndrome
• Mitochondrial Disorders – Riley-Day syndrome
• Medications – High-Dose Beta-Blockade
• Co-Morbid Disease – Diabetes Mellitus
www.marfmom.com
Diagnostic Approach to Dysautonomia
• Historical Considerations – The cornerstone of the evaluation – Review of all prescribed and alternative
medications – Head-to-toe Review of Systems
Diagnostic Approach to Dysautonomia
System Symptoms
Neurologic Headaches, Fatigue, Sleep disturbance, Loss of consciousness, Pain
Ophthalmologic Vision changes, “bags”
Cardiovascular Chest pain, palpitations, near syncope, syncope
Gastrointestinal Nausea, emesis, constipation, diarrhea, abdominal pain
Dermatologic Venous stasis, Thermal dysregulation
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Physical Examination
wordpress.com
Diagnostic Approach to Dysautonomia
• History & Physical Examination – Electrocardiogram – Selected Bloodwork (CBC, Electrolytes, TFTs) – Echocardiogram – Tilt Table Testing – Ambulatory Monitoring
Examples of Significant Findings
Personal Library
Tilt Table Testing
Personal Library, Used with Permission
Examples of Tilt Table Findings
Personal Library
Examples of Tilt Table Findings
Personal Library
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Examples of Tilt Table Findings
Personal Library
Examples of Tilt Table Findings
Treatment Options
• Stimulus Avoidance
• Physical Conditioning
• “Hyperhydration”
• Pharmacologic Treatment
• Device Therapy
• Psychological Counseling
Treatment Approaches Physical Conditioning
www.potsrecovery.com
Treatment Approaches Physical Conditioning
Fu, et al. JACC 2010;55(25):2858
Treatment Approaches Physical Conditioning
Fu, et al. JACC 2010;55(25):2858
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Treatment Approaches
• “Dehydrated” versus “Underhydrated”
• Primacy of salt – Minimum goal 3-6 g
• Water that follows – 8-10 8 ounce glasses of non-caffeinated
beverages
“Hyperhydration”
wordpress.com
Treatment Approaches
Pharmacological Options
Treatment Approaches Psychological Counseling
Mayo Clinic Publication, 2014
Treatment Approaches Psychological Counseling
Prognostic Thoughts
Final Thoughts
• Any pathophysiologic condition that is difficult to “objectify” is difficult to treat
• Being a “cheerleader” is also more difficult than providing a discrete therapy
• The line between “trying to help” and “doing harm” can be very grey…
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX
Where We’ve Been
• Explore the Normal Physiology of the Autonomic Nervous System & Possible Pathophysiology
• Describe Age-Specific Autonomic Conditions – Clinical Presentation – Diagnostic Criteria/Evaluation – Treatment Options
• Prognostic Thoughts
“The New England Journal of Medicine
reports that 9 out of 10 doctors agree that 1 out
of 10 doctors is an idiot..”
Jay Leno
http://www.funny-jokes-quotes-sayings.com
The 10%ers…
Pediatric Grand Rounds 09 Oct 2015
University of Texas Health Science Center at San Antonio, TX