Pediatric Cardiac

Emergencies
Gavin Greenfield
Peggy Thomsen

 4 year old female presents with fast

breathing, grunting, cough x 5 days
seen 2 days earlier and started on steroids
and bronchodilators
initial vitals: HR 150, BP 100/85, RR 36, T
37.5

 1 month old with irritability, poor feeding

(fatigues), failure to thrive, fast breathing
no fever or runny nose
physical exam: HR 160, RR 60 with
minimal respiratory distress, gallop
rhythm, rales

Infant Cardiac Disease Leading to

ER Presentation
Congenital

Acquired
Cardiomyopathy
Myocarditis (usually with CHF)
Dysrhythmias

Congestive Heart Failure

the physiologic state in which cardiac
output is unable to meet tissue metabolic
demands (Rosen)
CO = HR x SV
SV dependent upon preload, afterload,
contractility

CHF - Presentation
infants: irritable, poor feeding (early
fatigue), failure to thrive, respiratory
symptoms
always consider in patients with
respiratory symptoms
often misdiagnosed as respiratory illness /
infection

CHF - Etiology

Increased Preload
L to R shunts (VSD, PDA, AV fistula)
severe anemia

Increased Afterload
HTN
Congenital (aortic stenosis, coarctation of aorta)

Decreased Contractility

myocarditis, pericarditis with tamponade

cardiomyopathy (dilated or hypertrophic)
Kawasaki syndrome (early phase)
metabolic: electrolyte, hypothyroid
myocardial contusion
toxins: dig, calcium channel blockers, beta blockers

Dysrhythmia

CHF - Etiology
presents immediately at birth
anemia, acidosis, hypoxia, hypoglycemia,
hypocalcemia, sepsis

presents at 1 day (congenital)

PDA in premature infants

presents in first month (congenital)

HPLV, aortic stenosis, coarctation, VSD presents later

presents later (acquired)

myocarditis, cardiomyopathy (dilated or hypertrophic),
SVT, severe anemia, rheumatic fever

Myocarditis
leading cause of dilated cardiomyopathy and
one of the most common causes of CHF in
children
etiology: idiopathic, viral, bacterial, parasitic
hallmark is CHF
failure to respond to bronchodilators in wheezing
child
treatment includes inotropes, afterload
reduction, diuretics, antibiotics, antivirals

Pericarditis
sharp stabbing precordial pain
worse with supine and better leaning
forward
no sensory innervation of the pericardium
pain referred from diaphragmatic and pleural
irritation

Etiology
infectious

viral
bacterial
TB
fungal
parasitic

Connective tissue
RA
Rheumatic fever
SLE

Metabolic / Endocrine
uremia
hypothyroid

Hematology / Oncology
bleeding diathesis
malignancy

Trauma
Iatrogenic

Pericarditis
usually a benign course
virulent bacteria (H. flu, E. coli) can cause
constrictive pericarditis and subsequent
tamponade may need urgent
pericardiocentesis
uncomplicated pericarditis usually
responds to rest and anti-inflammatories

Chest Pain
4% of children will have a cardiac origin
remainder: MSK, pulmonic (asthma,
bronchitis, pneumonia), GI
Cardiac causes: myocarditis, pericarditis,
structural abnormalities such as congenital
heart disease or hypertrophic
cardiomyopathy

 14 year old male collapses at school while

in class
non-responsive for one minute
feels fine in the department
Approach?

Syncope
20-50% of adolescents experience at least
one episode of syncope
most cases benign

Pathophysiology
vascular
orthostatic, hypovolemia
neurally mediated
hypoxia: PE, CNS depression from OD, CO
cardiac

Cardiac Syncope
Dysrhythmias
tachy
brady

Outflow obstruction
Myocardial Dysfunction
cardiac syncope often precedes future
sudden cardiac death

Sudden Cardiac Death

includes those causes that directly relate
to cardiovascular dysfunction
one third of all sudden deaths

Sudden Cardiac Death

Etiology

myocarditis
cardiomyopathy (hypertrophic)
cyanotic and noncyanotic congenital heart disease
valvular heart disease
congenital complete heart block
WPW
long QT syndrome
Marfan syndrome
coronary artery disease
anomalous coronary arteries

Risk Factors for Serious Cause of

Syncope
history of cardiac disease in patient
FH of sudden death, cardiac disease, or
deafness
recurrent episodes
recumbent episode
exertional
prolonged loss of consciousness
associated chest pain or palpitations
medications that can alter cardiac conduction

What to look for in the Department:

EKG
Long QT syndrome
congenital or acquired
get paroxysmal v tach with torsades de pointes
congenital long QT associated with hypertrophic
cardiomyopathy
long QT defined as corrected QT longer than 0.44 s
T wave alternans sometimes present
can have normal ECG in the department
two clinical syndromes not associated with structural
heart disease: Romano-Ward and Jervell-LangeNielsen

Other dysrhythmias
WPW and other SVTs
AV block
usually acquired, rarely congenital

Sick sinus syndrome

Idiopathic Hypertrophic
Cardiomyopathy
aka IHSS
both a fixed and dynamic subvalvular obstruction
characterized by ventricular hypertrophy with
principle involvement of the ventricular septum
associated with long QT
autosomal dominant
often presents with exertional syncope
10 year mortality is 50% for children diagnosed
by age 14

Other structural cardiac

diseases
dilated cardiomyopathy
usually secondary to myocarditis
syncope and death secondary to ventricular dysrhythmias or
severe myocardial dysfunction

arrhythmogenic RV dysplasia
congenital cyanotic and non-cyanotic heart disease
valvular diseases
aortic stenosis

coronary artery anomalies

exertional syncope or sudden death
aberrant artery passes between aorta and pulmonary artery

 2 week old infant brought in by parents

with difficulty breathing
HR 180, BP 50/P, RR 80, T 37.5
history and physical
investigations
repeat vitals: HR 30, no BP, RR 12
definitive treatment:

 4 year old male presents with 2 weeks

history of cough, fast breathing, fatigue,
decreased exercise tolerance, puffy eyes
On exam: tachypneic, moderate
respiratory distress, O2 sats 92%, bilateral
crackles

 6 month male presents with failure to

thrive, fast breathing, blue lips
On exam tachypnea but no respiratory
distress, lips and extremities blue, oxygen
saturations 70%

Congenital Heart Disease

Fetal to Neonatal Circulation

Anatomic Classification; 4
groups
Right to Left Shunt

Tetralogy of Fallot
Transposition of
the Great Arteries
Tricuspid Atresia
Left to Right Shunt

ASD
VSD
PDA

Stenotic

Aortic valve stenosis

Pulmonic valve
stenosis
Aortic coarctation
Mixing

Truncus
Total Anomalous
Pulmonary Venous
Return
Hypoplastic left heart
syndrome

CHD Classified as Cyanotic vs.

Acyanotic
Cyanotic (R to L shunt and mixing lesions)
tetralogy of Fallot
transposition of great vessels
tricuspid atresia
total anomalous pulmonary venous return
truncus arteriosus
hypoplastic left heart syndrome

CHD Classified as Cyanotic vs.

Acyanotic
Acyanotic (L to R shunts, stenotic
lesions)
ASD
VSD
PDA
aortic valve stenosis
pulmonic valve stenosis
aortic coarctation

Cyanosis
Classified as central or peripheral
Central cyanosis (always abnormal)
mucous membranes, trunk, extremities
classified as cardiac (R to L shunt) or pulmonary

Peripheral cyanosis (acrocyanosis)

no involvement of mucous membranes

involves hands, feet, circumoral area
common in neonates from vasomotor instability
CHF, PVD, shock, cold extremities

Congenital Heart Disease

History
feeding difficulties
tachypnea
diaphoresis
syncope
cyanotic episodes
failure to thrive

Congenital Heart Disease

Physical Examination
colour: pink, blue, gray
vitals: tachypnea, tachycardia, BP
symptoms suggestive of infection
palpation and auscultation of precordium
chest auscultation
survey for organomegaly
pulses in all extremities

Cyanotic Congenital
Heart Disease
R to L shunts
mixing lesions

Tetralogy of Fallot the classic

cyanotic lesion
RV outflow
obstruction
RVH
VSD
overriding aorta
CXR reveals boot
shaped heart with
decreased pulmonary
blood flow

 2 month old female with known tetralogy

of Fallot brought in with 24 hour history of
vomiting and diarrhea
On exam: moderate dehydration
during IV attempts patient becomes
irritable and cyanotic

Treatment of Tet Spell

quiet, calm environment
knee-chest or squatting position
increases afterload thus decreasing R to L shunting

Oxygen
Morphine
to treat hyperpnea and decrease systemic catecholamines

Phenylephrine
increases afterload thereby decreasing R to L shunt

Manual external aortic compression below level of renal

arteries
Propranolol
to block beta receptors in infundibulum therefore lessening RV
outflow obstruction

Consider
consider small volume challenge (5-10
cc/kg) to increase preload and reduce
dynamic outflow obstruction
?NaHCO3 for correction of acidosis
may need general anesthesia if severe
and/or prolonged spell
interim prophylactic treatment with
propranolol while awaiting surgery

Acyanotic Congenital
Heart Disease
L to R shunts
stenotic lesions

VSD
Most common congenital lesion
Large VSDs may be silent and
become symptomatic in first few
weeks as pulmonary resistance
SOB and diaphoresis w feeds
Poor weight gain
Systolic murmur
CXR demonstrates CHF

 2 week old infant brought in by parents

with difficulty breathing
HR 180, BP 50/P, RR 80, T 37.5
history and physical
investigations
repeat vitals: HR 30, no BP, RR 12
definitive treatment:

Differential Dx of Infant Shock

infection (septic shock/ meningitis)
bacterial: GBS, E. coli, S. aureus
virus: enteroviruses, H. simplex

metabolic: amino/organic acidopathies,

urea cycle defect
hypoxic shock: eg. RSV, C.N.S.
depression
heart disease: congenital or acquired

LV Outflow
Obstruction

LV Outflow Obstruction
Aortic coarctation
Hypoplastic left heart syndrome
Aortic stenosis (presents later)
Rosen: any neonate in shock that does not
respond to fluids or pressors has LV outflow
obstruction until proven otherwise
complete obstruction incompatible with life
unless there is shunting

Coarctation of the aorta

Most often distal to L subclavian
Can be diagnosed anytime
Neonates present as acutely ill,
gray shocky (from DA closure)
Systolic murmur at the back
Hepatomegaly
Diminished femoral pulses
BP difference b/t arms and legs
CXR demonstrates CHF
Treatment of CHF
Prostaglandin E1

Duct Dependant Lesions

Duct needed to perfuse lungs or periphery
Lungs
Tetralogy of Fallot, transposition of great
arteries, tricuspid or pulmonary atresia
a patent ductus arteriosus results in preserved
pulmonary blood flow

Periphery
Aortic coarctation (severe) and Hypoplastic
left heart

Treatment of acute decline in

patients with ductal dependant
lesions

Open the closed duct

Prostaglandin E1 0.1 ug/kg/min infusion
reduce dosage as perfusion and colour
return
Rosen: any infant in the first week of life
with decreased perfusion, hypotension, or
acidosis should be considered a candidate
for PGE1 administration

What do you need to know about

PGE ?
it functions by dilating vascular smooth
muscle, both systemically and in the
pulmonary vascular bed
its use in CHD pts is to maintain patency
of the PDA, whether to maintain PBF or to
maintain systemic blood flow past a

Classification Review
pink child in respiratory distress suggests
acyanotic chd (L to R shunt, coarct, aortic
stenosis)
blue cyanotic child in little respiratory
distress suggests R to L shunt or mixing
lesions
gray, shocky baby suggests outflow tract
obstruction

Bradyarrhythmias
Etiology
hypoxia, acidosis, hypoglycemia
excess vagal stimulation (ex. intubation)

Treatment
Epinephrine
Atropine if known vagally mediated or heart
block

Congenital Bradyarrhythmias
complete AV block
autoimmune injury to fetal conduction system
secondary to maternal autoimmune disease
atropine, isoproteronol, epinephrine may be
tried temporarily prior to pacing

Tachyarrhythmias
Supraventricular Tachycardia
re-entrant with accessory pathway (AV nodal
or WPW)
re-entrant without accessory pathway (reentry occurs within sinus node or within
atrium)
ectopic

nonspecific presentations in infants

Murmurs
Areas
aortic: R 2nd intercostal space
pulmonic: L 2nd intercostal space
mitral: apex
tricuspid and VSD: L lower sternal border

Pathologic
diastolic, holosystolic, late systolic, continuous

Innocent Heart Murmurs

History
normal growth and development, normal exercise
tolerance
no history of cyanosis

Physical Examination

Grade II or less, localized

varies with position (decreased with upright posture)
normal precordium
normal pulses

Lab
normal EKG, normal CXR

3 innocent murmurs
Stills
short ejection systolic murmur
musical or vibratory quality
heard best between apex and left sternal border

physiologic pulmonary flow murmur

harsh, located at pulmonic area

peripheral arterial stenosis

low-intensity systolic ejection murmur best heard in
axilla and back

 8 year old male presents with fever,

arthralgias
mother mentions that he had a sore throat
3 weeks ago for a few days with
spontaneous resolution
a throat swab was done and positive for
GAS but patient better so did not take the
prescribed antibiotics

Acute Rheumatic Fever

school aged children
associated with certain strains of Group A betahemolytic streptococcal infections
the streptococcal organism stimulated antibody
production to host tissues
CT of heart, joints, CNS, subcutaneous tissues, skin

carditis is an endomyocarditis with valvulitis

involving mitral and aortic valves
2 to 6 weeks post streptococcal pharyngitis

Jones Criteria
Major
carditis
new or changing
murmur
cardiomegaly, CHF
pericarditis

migratory polyarthritis
chorea
erythema marginatum
subcutaneous nodules

Minor
fever
arthralgia
history of previous
ARF
elevated ESR, CRP
prolonged PR on EKG
Rising titer of
antistreptococcal
antibodies

ER Treatment
management of complicating features of
carditis (CHF)
significant carditis or CHF managed with
glucocorticoids

high-dose ASA 75-100 mg/kg/day

pencillin
long term management of rheumatic heart
disease

Pediatric EKGs General Principles

RV Dominance at birth; gradually changes
to LV dominance
axis up to +180 in normal newborn
T waves negative in right precordial leads
until adolescence (except they are upright
in first week of life)