Pediatric Cardiology 101: Syncope, Chest Pain

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Pediatric Cardiology 101 Syncope, Chest Pain
Murmurs

• Karen Raines, MD
• Associate Professor of Pediatrics
• WFU School of Medicine
• And
• Whitewater Raft Guide
• New Gauley Rivers, West Virginia

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I have no conflicts of interest to disclose
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Objectives

• Keep your attention and teach ya some useful
  stuff
• Review syncope in the ped pt including the when
  to worry and when not to worry types and their
  evaluation
• Review chest pain in the ped pt ditto above
• Quick review of the common normal murmurs found
  in children
• Sneak in a few rafting videos!

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SYNCOPE
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Definitions

• Syncope transient loss of consciousness
  muscle tone most often due to inadequate cerebral
  perfusion
• Pre-syncope the feeling that one is about to
  pass out but no loss of consciousness
• Sudden death fall down, but dont get up

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How Common is It?

• Probably more common than reported
• Variable reports range from 12,000 ped ER visits
  to 47 of interviewed college students
• Best estimate is about 15 of children will
  have syncope
• Often a familial tendency towards syncope is
  discovered

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Why So Much Angst?Fancy word for worry

• Is syncope a harbinger of sudden death?
• Well, it depends on the circumstances of the
  syncopal episode
• Simple syncope is NOT associated with a higher
  incidence of sudden death

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HOWEVER . . .

• SYNCOPE DURING EXERTION MAY BE THE ONLY WARNING
  OF A LETHAL CONDITION

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Categories of Syncope

• Neurocardiogenic
• Cardiac
• Non-cardiac

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Neurocardiogenic Syncope

• Most common cause of syncope
• Multiple names
• Neurally-mediated syncope
• Vasovagal syncope
• Simple fainting
• Disturbed autonomic control of HR BP in
  response to emotion, pain or postural change
  (Huh?!) whatdidshesay?!

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The Physiology Part(But no biochemical flow
charts!!!)

• Surveillance of blood pressure performed by
  multiple receptors
• Baroreceptors
• Carotid artery bifurcation (carotid sinus)
• Aortic arch
• Mechanoreceptors
• Atria
• Pulmonary artery
• Left ventricle

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More Physiology !

• Afferent (input) signals from receptors are sent
  to medulla
• Parasympathetic part
• Nucleus ambiguus
• Dorsal motor nucleus of the vagus
• Sympathetic part
• Costal ventromedial ventrolateral medulla

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A Little More !

• Two pronged output results
• Sympathetic withdrawal
• Vasodilatation
• Decreased contractility
• Parasympathetic output
• Venodilation
• Decreased heart rate

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So What Makes Em Sink?

• Appears to be due to excessive input from the
  cardiac mechanoreceptors
• Stretch or distortion is detected, but its due
  to an empty stretching inward heart, not a full
  one!
• Which triggers one last physiology slide and
  diagram !!!

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What Reflex You May Ask?!?
Bezold-Jarisch
Reflex
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(No Transcript)
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Evaluation of the Sinker

• History
• Physical exam
• EKG

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History

• Probably the single most important part of the
  evaluation
• Careful, non-hurried non-leading questions
  detailing entire episode
• Description of circumstances, prodrome, the
  actual faint and after-effects/appearance from
  patient and/or observers

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Circumstances

• Where did it happen
• Time of day
• ?meals, ?intake
• Body position
• Preceding activity
• Any intercurrent illness

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Prodrome

• What was the first sensation?
• Time betw 1st warning the faint?
• Any visual changes?
• Any auditory changes?
• Any nausea, diaphoresis or pain?
• Any palpitations preceding the event?
• If so, temporal relationship to event

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The Faint

• Any injury?
• Do they remember the fall?
• How long unconscious?
• Any tonic activity (vs T-C)?
• Appearance during?
• Color, diaphoretic?
• Incontinence?

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After the Faint

• Whats the first thing they remember?
• Any feelings of nausea, headache, chest
  sensations, confusion, fatigue
• ?Get up too fast?
• Skin color and ?diaphoretic
• How long until totally back to normal

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Typical Sinker Story

• Pre-teen or teen, sitting or standing
• Prodrome of hot feeling, nausea, racing heart,
  sweaty
• Hearing often dims
• Vision often grays out or tunnel vision
• Falls to floor without serious injury
• May have tonic activity

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Typical Sinker Story

• Awakens nearly immediately
• Described as pale, clammy, pasty gray,
  diaphoretic
• Often feels nauseated and fatigued for a while
  may not be back to normal for many hours
• Recurs if helped up too quickly

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History - Other Aspects

• Voiding history at school
• Prior medical history including cardiac history
  (duh)
• Previous syncopal episodes
• Medications
• Illicit drugs, behaviors (privately, of course)
• Family history

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Family History

• Sudden unexpected death
• HCM, LQTS, arrhythmias, etc
• Syncope
• Seizures
• Migraines

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Physical Exam

• Complete with special attention to
• Cardiac exam
• Neurologic exam

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Cardiac Exam

• Palpate
• Displaced PMI - lift/heave
• RV lift/heave
• Thrills
• Palpable S2
• Murmurs of outflow obstruction
• Single S2 indicative of pulm HTN

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EKG

• A must (in my opinion others)
• WPW syndrome
• Short PR with wide QRS (BBB)
• Syncope can be from a-fib with rapid ventric
  response
• Long QT Syndrome(s)
• Conduction defects
• Complete block, familial cond defects, etc
• Ventricular hypertrophy

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Further Evaluation or Not?

• If history typical, physical and EKG both normal,
  then further testing /or referral not necessary
• Explanation
• Reassurance
• No risk of SD, benign temporary
• Start simple therapy

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Lay Explanation

• Basic explanation of reflexes
• Immaturity /or exaggerated reflexes in
  pre-teens, teens
• Involves brain, heart blood vessels
• Stress benign nature
• Stress no risk of sudden death
• Stress self-limited time course

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NMS Rx - 1st Step

• Goal is to increase circulating volume
• Increased fluid intake
• Mention potential diuretic effect of caffeine
• Use more salt or salt tablets
• Stress importance of paying attention to the
  warning signs
• Get your head down

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NMS Rx - 2nd Step

• Mineralocorticoid fludrocortisone (Florinef)
• Dose range 0.1 mg qd or bid (max 0.4 mg/d)
• Must still maintain good fluid intake
• Warn about tight shoes!
• Takes few days to week for full effect
• Essentially, no side-effects
• Usual treatment course 6-12 mo

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NMS Rx - 3rd Step

• Multiple other agents available
• Beta-blockers
• Alpha-agonists
• Serotonin agonists

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Cardiac Causes

• Obstruction to flow
• Myocardial dysfunction
• Arrhythmias

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Cardiac Causes

• Obstruction to flow
• Aortic or pulmonary stenosis
• Hypertrophic cardiomyopathy
• Primary pulmonary hypertension
• Eisenmengers syndrome

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Cardiac Causes

• Myocardial dysfunction - primary
• Dilated cardiomyopathy
• Neuromuscular disorders
• Duchenne, Becker, Myotonic, etc
• Myocardial dysfunction - secondary
• Inflammatory - myocarditis, Kawasaki
• Ischemia - coronary abnormalities
• Aneurysm/obstr, ALCA, aberrant coronary

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Cardiac Causes

• Arrhythmias (nl heart vs CHD pt)
• Tachycardia
• VT or SVT (espec WPW)
• Channel-opathies
• Long QT, Brugada, probably more
• Conduction abnormalities
• Sinus node dysfunction
• AV block
• Arrhythmogenic RV dysplasia

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Non-Cardiac Causes

• Seizure disorder
• Often have aura, T-C activity and period of
  post-ictal confusion and lethargy
• More likely to be confused with simple syncope if
  complex partial type
• Convulsions occur before LOC
• Usually warm flushed after (not pallid or
  diaphoretic)

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Non-Cardiac Causes

• Breath holding spells
• Ages 6m-18m typical up to 6 yrs old
• Precipitated by pain, anger, etc
• Cry gt long expiration gt apnea gt LOC
• Pallid form often with asystole
• Often have tonic posturing
• Cyanotic form is blue (duh!)
• Resolves with LOC and breathing
• Benign resolves by age 5-6 yo

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Non-Cardiac Causes

• Migraine (basilar artery migraine)
• Typical presentation is headache aura, then LOC
  and awake w/ severe occipital HA
• Some dont have headache
• More in adolescent girls

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Non-Cardiac Causes

• Drugs
• Espec illicit ones
• Huffing volatile agents
• Metabolic
• Hypoglycemia VERY RARE (maybe never!) cause
  unless diabetic

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Non-Cardiac Causes

• Hyperventilation
• Usually assoc w/ anxiety, c/o dyspnea, chest
  discomfort, dizzy paresthesias
• More common in adolescents
• Hypocapnia gt decr cerebral blood flow
• Hysteria
• Always audience present graceful swoon
• LOC for long period

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Non-Cardiac Causes - Situational Syncope

• Cough syncope
• More often in pt w/ lung disease
• High thoracic press gt decr return
• Swallow (deglutition) syncope
• Rare in peds more in adults
• Esoph receptors gt CN IX gt vagal output

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Non-Cardiac Causes - Situational Syncope

• Micturition syncope
• Usually male before, during or after voiding
  (often fatigue, alcohol hx)
• Unknown causes
• Proposed due to combo of postural hypotension,
  visceral stretch receptors, vagal stim, etc
• Benign usually an isolated episode

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Non-Cardiac Causes - Situational Syncope

• Hair-grooming / neck stretch syncope
• Nearly always in adolescents
• Possible mechanisms include trigeminal nerve
  stim, carotid sinus hypersensitivity,
  vertebrobasilar circulation changes
• Benign

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When To Worry

• Circumstance
• Syncope during exertion
• Known cardiac patient
• Malignant family history
• Prodrome
• Angina
• True palpitations

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When To Worry

• The Faint
• True sudden death (big duh!)
• Prolonged LOC (/-)
• Serious bodily injury during fall (/-)
• After event
• Tachy-arrhythmia present

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When To Worry

• Abnormal physical exam
• Abnormal EKG

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Syncope During Exertion

• Careful history about details
• IF IT WAS A TRUE OUT AND OUT FACE-PLANT
  (dirtintheteeth) WHILE EXERTING THEN . . . . .
• Must exclude 3 potentially lethal conditions in
  ostensibly normal person

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Must Exclude

• Anomalous left coronary artery
• Aberrant coronary artery
• Hypertrophic cardiomyopathy

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Anomalous Left Coronary Artery

• Left coronary arises from pulm art
• Problem is not blue blood perfusing the
  myocardium
• Problem is run-off of blood bypassing the
  myocardial capillary bed and dumping into the low
  resistance pulm circulation
• Age mode of presentation depend on collateral
  connections, flow, etc

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Aberrant Coronary Artery

• Coronaries arise from aorta, but a major branch
  courses BETWEEN the Ao and PA
• Most common variant is LAD off the right
• Exercise gt high cardiac output gt Ao PA dilated
  gt compresses coronary gt oops !

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Hypertrophic Cardiomyopathy

• Usually, physical exam and/or EKG are abnormal
• May not have positive family history
• Can be very subtle

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CHEST PAIN
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Chest Pain

• Extremely common complaint, but I dont have any
  numbers
• Typically in the pre-teen, young teen age
• Probably all kids have some occasional pain, but
  not all of them complain
• Often it has been going on for a while but not
  mentioned to parent

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My Opinion

• The media campaign to save adults from heart
  attacks has led to a paranoid generation of lay
  people and the medico-legal society we live in
  has created the problem of over-reaction (the
  CYA approach to medicine). Additionally the
  sensationalism of rare sudden deaths in athletes
  heightens the parental anxiety.

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The Benign Story

• Typical age is pre- to early adolescence
• SHARP, RANDOM stabbing mid-sternal chest pain
• Worse with breathing
• May have recently started a new activity or had
  an injury
• Sometimes mention a tender area on chest
• Often a family history of heart problems

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History Tid-bits

• Establish that pain occurs sometimes at rest
• Confirm aggravating factors movement, deep
  breaths, etc
• Query about new activity (new bike, new exercise
  equipment, etc) or injury
• Ask about attempted home treatments (antacid,
  analgesics how given)

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Physical Exam Tid-bits

• Obviously perform careful cardiac exam looking
  for cardiac enlargement, murmurs, single S2, etc
• Assess for tender areas
• Pressure on sternum
• Side rib squeeze

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Then Teach Em a Cool New Word !
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Costochondritis
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Explanation

• I review basic thoracic cage anatomy
• Breakdown word costochondritis into its separate
  parts (handout helpful)
• Stress benign nature of real pain (not
  imaginary!)
• Ibuprofen bid for several weeks
• Campfire analogy

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To Test or Not to Test

• Is a test absolutely necessary?
• EKG
• Painless, safe and relatively inexpensive
• Not absolutely needed
• Sometimes the parent just needs a test to help
  convince them!
• Chest x-ray
• Depends on story more pleuritic sx
• Radiation

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The Non-Benign Story

• Pressure, vise-grip substernal discomfort brought
  on only by activity (generally a predictable
  level)
• Relieved by rest
• Often associated with diaphoresis, nausea,
  dizziness
• OR
• Syncope associated with activity/pain

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True Angina

• Obviously this is a horse of a different color
• Requires major work-up
• Differential includes
• Anomalous or aberrant coronary
• Myocardial bridging
• Hypertrophic cardiomyopathy
• Coronary stenosis congenital vs acquired

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Innocent
Murmurs
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Innocent Murmurs

• Quick review of the common murmurs
• Unifying theme is that they change with position
• High output state will accentuate the murmurs
• Most common in the pre-school age child (except
  PPS in the newborn)

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Normal Murmurs

• Stills murmur
• Innocent pulmonary flow murmur
• Brachiocephalic murmur
• Branch pulmonary stenosis (PPS)
• Venous hum

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Stills Murmur

• Named for Frederick Still early 1900s
• Vibratory, twangy, pretty systolic murmur best
  heard at LLSB
• Loudest supine, soft/disappears upright
• Grade 1 3/6
• Typically in pre-school/school age children
• Infant version
• Thought to represent vibration of the great
  vessels /or LVOT

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Innocent Pulmonary Flow Murmur

• Systolic ejection murmur over the pulmonary area
• Grade 1-3/6
• May be heard in the back
• Increases with supine position, decreases upright
• Increased by high-output states
• No associated click

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Brachiocephalic Murmur

• aka innocent cervical bruit, supraclavicular
  murmur/bruit
• Grade 1-3/6 scratchy, harsh short SEM heard
  loudest above the right clavicle softer or
  absent below
• No preceding click
• May change with shoulder hyperextension
• Can be heard over left clavicle too
• Represents turbulent flow to the head

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Branch Pulmonary Stenosis

• aka Peripheral Pulmonary Stenosis (PPS)
• Grade 1-3/6 SEM heard at base with radiation to
  axillae and back
• No preceding click
• Normal in infants lt6 - 9 months age
• Usually not heard in the immediate newborn period
• Represents turbulence at the pulmonary bifurcation

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Venous Hum

• Grade 1-6/6 (!) continuous very low-pitched
  murmur heard best under the clavicles
• Loudest upright (gravity accentuates flow)
• Loudest during diastole
• Disappears with jugular vein pressure or supine
  position
• Represents turbulence in the great veins from
  cerebral blood flow

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Mammary Souffle

• Rare in pediatrics
• Grade 1-3/6 continuous high-pitched murmur along
  the sternum due to enlarged internal mammary
  arteries
• Found only in lactating females

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Conclusions

• Syncope can generally be fully evaluated by a
  very careful history physical (/- EKG)
• True syncope during exertion is a major red flag
• Chest pain is common and rarely cardiac
• True angina is rare, but obviously requires full
  evaluation
• Innocent murmurs are common