irrelevant disclosures: too many to list…. · tachycardia syndrome cardioinhibitory syncope* both...

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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

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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