Download Pediatric Cardiac Emergencies

Pediatric Cardiac
Emergencies
Graham Thompson
Francois Belanger
Feb 26th 2004
Objectives
Review transitional changes in pediatric cardiology
CHD – presentations in ED, blue vs pink, ED Tx
 Post-op issues presenting to the ED
 Pacers
 SVT in the PED
 Acquired Cardiac Disease


– Pericarditis
– KD
– ARF



MIs in the pediatric world
Cardiac Arrest
PALS Guidelines (attached)
Neonatal Circulation
Changes at Birth

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Decreased PVR from expansion of lung and high
PaO2
Increased SVR from stopping placental
circulation
Functional closure of PFO from high LA
pressures
Reversal of flow and then closure of PDA
Closure of ductus venosus
Slow change from R dominant to L dominant
circulation
(Malformations often cause changes to this
pattern (can be life saving i.e. PDA in CoA!!)
Normal Vitals for Children
Case #1
4 do boy with central cyanosis
 Term baby of 17 yo girl, minimal prenatal
care
 ROM 6 hrs, uncomplicated delivery
 Left hospital w/in 24 hrs
 Bottle feeding well until today, now only 1
oz/feed
 WOB increased over the day

Case (cont)



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
Brought into resusc room
Hr 165 RR 75 BP 65/28
LA
SpO2 79% R/A, cyanosed
100% supplied
– SpO2 – 84%
IV started, NS bolus 70 cc





A/E good, clear, mildmoderate WOB
S1 S2 (? Single) IV/VI
systolic murmur
Pulses good, liver 2cm
BCM
Font flat, soft, neuro
intact
Bld wk, ECG and CXR
pending
Congenital Heart Disease
0.5-0.8% of live births (not including PDA
or bicuspid aorta)
 Increased in syndromes

– T21 – endocardial cushion defects, ASD, VSD
– XO – CoA, bicuspid AV

Increased with maternal prenatal drugs
– Lithium – Ebstein anomaly
– VPA – CoA, HLHS, VSD, AS

40-50% dx in 1st wk, 50-60% by 1st
month
Frequency of major CHD
(% of total)

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VSD
ASD (sec)
PDA
CoA
TOF
PS
AS
d-TGA
HLHS
25–30
6–8
6–8
5–7
5–7
5–7
4–7
3–5
1–3
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

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HRHS
Truncus
TAPVR
Tricuspid atresia
Single ventricle
DORV
Others
1–3
1–2
1–2
1–2
1–2
1–2
5–10

* Excluding PDA in preterm
neonates, bicuspid AV,
physiologic PPS, and MVP
Presentations
Most complex CHD are picked up on routine
prenatal ultrasound (up to 75%)
 Complex CHD is often picked up prior to D/C
from the nursery with cyanosis or abnormal
heart sounds
 BUT…….Timing and symptoms relate to
physiologic changes of newborn and the lesion
(obstruction vs shunt) so they may show up in
the ED!!!
 Any newborn looking comfortably cyanotic is
probably cardiac!! – mixing lesion

0-2 wks
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Duct dependant lesions
Takes 2-14 days for duct closure
May present to the ED more often, because of
early D/C (duct hasn’t closed yet!)
Most common are Left outflow tract obstructions
(CoA, interrupted arch, AS, HLHS), TGA, TOF
In the words of Dr. Patton
– “Beware of the “septic”-like 2 wk old infant with poor
pulses: is it possibly CoA or critical AS?”
In



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
st
1
year
CHD that presents in failure (tachypnea, sweating
with feeds, feeds >30 min, FTT, pallor)
Most commonly related to L-to-R shunt (ie VSD)
At birth, R pressures are high, shunt is minimal so
defect may not present itself
Drop in R side pressures and increased L pressures
occurs over 6-8 wks causing shunt and symptoms
Murmurs are not heard in nursery because lack of
shunt
Late TOF presentations that are relatively protected
from failure by RVOT obstruction. May present
with tet spells.
Childhood

Restrictive L-to-R shunts
– ASD, Small VSD
Mild CoA, mild AS – may present with
fatigue or leg pain/wkness or HTN
 The very rare TOF

Presentation of CHD
Approach to CHD Dx

Cyanotic
– Increased Pulmonary flow
– Decreased pulmonary flow (RV outflow
obstruction)

Acyanotic
– Increased volume load (ASD, VDS, PDA)
– Increased pressure load (LV outflow
obstruction)
Approach to CHD
Hx in CHD

Cyanotic episodes
– Central vs peripheral

Feeding pattern
–
–
–
–
diaphoresis
Stopping frequently
Prolonged (>30 min)
FTT
Gestational age
 Maternal Hx of
medications,
infections
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Prenatal U/S
Genetic D/O
Previous child with
CHD
Exercise intolerance
Syncope
R/O sepsis stuff!!!
Px in CHD

VS
– Tachypnea
– Arrhythmias
– 4 limb BPs (SBP in legs
N > by 10, if not look
for LVOT obstruction)
– 4 limb SpO2
– T0C – hypermetabolic
Colour
 Diaphoresis
 Wt gain
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Heart sounds and
murmurs (don’t forget
to listen to the back –
CoA)
Distal pulses, R/F
delay
Precordium
Abdomen – HSM
Clubbing
Cyanotic Heart Disease
Central vs Peripheral
 Central

– 50 g/l of non-oxygenated Hgb
– Level of hypoxia varies according to hct, so
polycythemic kids can look cyanotic at higher
SpO2s

Peripheral
– Perfusion issues
Hyperoxic test

Technically
– Do gas
– Give 100% O2
– After 10 min, redo gas
– Mixing cardiac lesion won’t be able to get
PaO2 > 100-150

Practically
– SpO2 won’t get into 90% in mixing cardiac
lesion
Cyanotic Heart Disease

The 5 T’s
– Tetralogy Of Fallot (TOF)
– Truncus Arteriosus
– Tricuspid Lesions (atresia, Ebstein’s)
– Transposition of the Great Arteries (TGA)
– Total Anomalous Pulmonary Venous Return
(TAPVR)

+ PA/severe PS
TOF

Tetralogy
– RV outflow obstruction
– Overriding aorta
– VSD (usually non
restrictive)
– RVH
TOF clinical
5 - 10% of CHD
 Most common cyanotic CHD after infancy
 Cyanotic or acyanotic depending on degree of
RVOT obstruction

– Blue if ++ obstruction = R-L shunt (present earlier)
– Pink if minimal obstruction = L-R shunt
RVH (felt under xyphoid)
 Loud, probably single S2
 III-V/VI SEM at LSB

TOF - Dx
Boot shaped heart
 Decreased pulm.
Vasc.
 20% R sided arch
 RVH
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“Tet” Spells
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Most frequent in 1st 2 yrs (peak at
2-4 mo)
After startle, crying or upon
waking
Hyperpnea, restless, increasing
cyanosis, syncope
Sudden decrease in pulmonary
flow, increased R-L shunt
Prolonged spells can lead to LOC,
acidosis, szs
Tx
–
–
–
–
–
–
–
Calm child
Knee to chest or Squat position
O2
Morphine 0.1-0.2mg/kg SC
HCO3 if prolonged
ketamine
Phenylephrine to increase
systemic pressures
TGA
d-TGA is 2 systems in
parallel
 l-TGA is
physiologically
corrected (lung-LARV-aorta-body-RA-LVlung)

TAPVR
A – supracardiac
 B – cardiac
 C – cardiac
 D - infracardiac

Truncus
Tricuspid atresia
No RA outflow
 Requires ASD (natural or balloon) to have
R-L shunt.
 Requires PDA or VSD to allow L-R shunt
 RV usually really small, so Fontan works
well
 Usually Dx at birth, but may be later

Ebstein’s Anomaly
Downward displacement
of an abnormal TV
 Atrialization of RV

Evaluation of Cyanotic Infant
ABC
 IV access
 Physical exam
 Labs, hyperoxic test
 CXR
 Consider meds
 Call cardio
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PGE1
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Relaxation of ductal smooth muscle
Infusion of 0.05-0.2 ug/kg/min
– 80 cc NS in 500ug vial run at BW = 0.1ug/kg/min (or
36cc in 225ug vial)

Side effects
–
–
–
–
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Jitteriness
Fever
Hypotension
Apnea
Relative contraindication
– TAPVR – may worsen systemic perfusion
CXR in CHD
Egg on a string – TGA
 Snowman – TAPVR
 Boot – TOF
 Super huge heart – Ebstein’s
 Backward E – CoA – notched ribs by
collaterals

Acyanotic Heart Disease
RVOT obstruction presents with shock
 CoA
 VSD
 ASD

Surgical Procedures
Post-op issues in the ED

Shunts
– Can obstruct, especially passive flow (glenn)
 Think of this child presents with URTI or gastro causing
dehydration
 BT shunts are active flow, and when obstructing have
increased cyanosis and changed shunt murmur
 Glenn shunts are passive flow and present with SVC
syndrome and decreased murmur
– Can flood lungs
 If R-L shunt is too high, then may present similar to
failure – tachypnea, crackles, wet chest
Post-op issues in the ED
MI – especially in atrial or arterial switches
for TGA
 Arrhythmias

– AV block for TGA, VSDs, ASDs
– WPW in Ebstein’s anomaly
– Atrial flutter
Post-op issues in the ED
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Post pericardotomy syndrome
– Sustained febrile period 1 wk – 2 months (mean 3-4
wks) after sx
– Thought to be related to autoimmune response
– Pericarditis symptoms, CXR and ECG
– Tamponade may occur
– Usually self limiting in 2-3 wks
– Tx
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Bed rest
NSAIDs
Steroids
Pericardiocentesis if needed.
CHD presentations to ED
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
5 yr retrospective study at UCLA ED
Only 8 new CHD presentations
–
–
–
–
–
–
–
Age ranged from 1 wk to 5 months
ASD – 3
VSD – 1
AS – 1
ALCAPA – 1
CoA – 1
TAPVR – 1
– Savitsky E J.Emerg Med 2003, 24(3):239
CHD in the ED

Really great references on CHD
– Woods WA et al Emerg Med Reports 2003
24(6) – CHD in the ED
– Woolridge DP et al Ped Emerg Med Reports
2002 7(7) – CHD in the PED Pt 1
– Woolridge DP et al Ped Emerg Med Reports
2002 7(8) – CHD in the PED pt 2
Case
3 mo boy to ED with grey spells
 Has happened 3 times in past 2 days
 Hasn’t taken more than 1 oz for past 3
feeds
 Intermittent WOB, no URTI sympt.
 Had been seen last month for decreased
feeding, difficulty breathing, stuffy nose
and pallor– dx – bronchiolitis

Case (cont)
Looked dusky at triage
 Taken directly to resusc. room
 O2 and monitors, IV started immediately
 Pinked up with O2, SpO2 = 99%


ECG
SVT
Post Adenosine
Child had echo showing Ebstein’s Anomaly
Non-pharmacologic Tx for SVT
Interrupts about 25% of SVT
 Cooperative children

– Blow against straw
– Crouch down

Uncooperative or Small Children and Infants
–
–
–
–
Push legs into chest
Carotid sinus massage
Ocular pressure
Diving reflex
Carotid massage for SVT
Randomized X-over trial of carotid
massage vs valsalva maneuver
 148 episodes
 No difference in efficacy
 Total of 25% responded to one of the
therapies
Lim SH et al Ann Emerg Med 1998 31(1):30


Carotid massage is not recommended in
young patients
(but no reason given!)
Paed Drugs 2000 2(3):171
SVT and the Diving Reflex

increased parasympathetic tone to the heart causing
bradycardia in association with systemic vasoconstriction
–
–
–
–
ice cube application to the lip and bottom nose
immersion of the head into a basin of ice and water for 4-6 s
application of a cold, wet facial cloth to the face
application of an ice bag to the face

“The diving reflex is much more effective than the ‘usual’
vagal maneuvers such carotid sinus massage, gag reflex
or rectal stimulation”

avoid inducing apnea and aspiration; and avoid
prolonged application of ‘cold’ to the face.
Moak JP Prog Ped Card 2000 11(1):25
The Diving Reflex and SVT

The use of the diving reflex to terminate a case
of paroxysmal supraventricular tachycardia
(PST) is described in a 2-week-old infant who
presented in severe congestive heart failure with
supraventricular tachycardia at a rate of 300.
The infant's face was placed in a basin of ice
water at 5 degrees C. for 5 seconds with
manual occlusion of the infant's nostrils to
prevent aspiration. The PST converted to a
sinus rhythm of 120 within 3 seconds of facial
immersion.
Hamilton J Am Heart J 1979 97(3):371
Ice for SVT
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Has been reported to abort up to 90% of SVT in infants
Supposedly initiates the “diving reflex”
1st report 1980
–
–
–
–
Filled plastic bag with equal volume of water and crushed ice
Covered entire face “to preauricular area”
Conversion occurred within 15 seconds (n=10)
“We can only speculate on the emotional trauma of the ice bag
technique”

Do you have to cover the whole face? Do you have to
cause apnea? – still controversial, no good data
Probably shouldn’t cover mouth and nose

Complications?

Craig JE et al J Pediatr 1998 133:727
Medications for SVT

Adenosine
–
–
–
–
Transient Av node blockade
0.1 mg/kg to max of 6 mg
Repeat at 0.2 mg/kg to max of 12 mg
Don’t get your knickers in a knot when the monitor shows a flat
line
– Needs proximal IV with rapid NaCl bolus ‘cause ½ life is a mere
15 seconds

Verapamil
– CONTRAINDICATED in children < 1yo because of several studies
demonstrated increased risk of hypotension, malignant
arrhythmias and electomechanical dissociation
– 0.1 mg/kg max dose 5mg over 15 minutes
Atkins, DL Clin Ped Emerg Med 20012(2):107
North American Society of Pacing and
Electrophysiology Generic Pacemaker Code
I
Chamber(s
) Paced
II
Chamber(s)
Sensed
O, None
O, None
A, Atrium
III
Response to
Sensing
IV
Programmability,
Rate Modulation
V
Antiarrhythmi
a Function
O, None
O, None
O, None
A, Atrium
T, Triggered
P, Simple
programmable
P, Pacing
(antitachyarrhyt
hmia)
V, Ventricle
V, Ventricle
I, Inhibited
M, Multiprogrammable
S, shock
D, Dual
(A + V)
D, Dual
(A + V)
D, Dual
(T + I)
C, Communicating
R, Rate Modulating
D, Dual (P + S)
Different Types of Pacing
DDDR Pacing
Pacemaker Complications

Most common include
– infection at site
– Electromagnetic interference
 Cell phones – unlikely
 Antitheft devices – unlikely
 MRI, cautery etc.
Case
14 yo girl gradual onset of chest pain x 2d
 Now acutely SOB, increased pain
 Worse when lying down
 Recent viral URTI symptoms
 Previously well

Pericarditis
Pericarditis
Normal pericardial fluid volume = 10-15cc
 Inflammatory response causes increased
fluid accumulation (can be > 1L)
 Symptoms – precordial pain improved with
sitting, cough, dyspnea, vomiting, fever
 Px – friction rub (variable), muffled heart
sounds, tachy, venous distention, pulsus
paradoxus

Etiology of Pericarditis

CONGENITAL ANOMALIES

INFECTIOUS

– Absence (partial, complete)
– Cysts
– Mulibrey nanism (muscle, liver,
brain, eye) with congenital
pericardial thickening and
constriction

– Viral (coxsackievirus B, EBV,
influenza, adenovirus)
– Bacterial (Streptococcus,
Pneumococcus, Staphylococcus,
Meningococcus, Mycoplasma,
tularemia)
– Immune complex (Meningococcus,
Haemophilus influenzae)
– Tuberculosis
– Fungal (histoplasmosis,
actinomycosis)
– Parasitic (toxoplasmosis,
echinococcosis)
CONNECTIVE TISSUE DISEASES
–
–
–
–
–
METABOLIC-ENDOCRINE
– Uremia
– Hypothyroidism
– Chylopericardium

HEMATOLOGY-ONCOLOGY
– Bleeding diathesis
– Malignancy (primary, metastatic)
– Radiotherapy-induced

OTHER
–
–
–
–
–
–

Rheumatoid arthritis
Rheumatic fever
Systemic lupus erythematosus
Systemic sclerosis
Sarcoidosis
Trauma
Iatrogenic (catheter related)
Postpericardiotomy
Aortic dissection
Idiopathic
Familial Mediterranean fever
Pericarditis - Tx

To tap or not to tap?
–
–
–
–
No good data
In typical viral pericarditis, probably don’t have to
If any doubt of bacterial causes, just do it
Other reasons to tap
 Significant symptoms
 Possible malignancy
 Abnormal diastolic fnctn on echo

NSAIDs, steroids. Again, no good evidence.
Wise words from Dr. Patton
ARF

Major Criteria
–
–
–
–
–
Carditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules

Minor Criteria
– Clinical
 Fever
 Arthralgia
– Investigations
 Increased ESR or CRP
 Prolonged PR interval
Plus Evidence of GAS infection (swab, ASOT, antiDNase B)
Dx = 2 major criteria, 1 major + 2 minor
Case
4 mo boy transferred to pediatrics for
significant HSM and fever for 7 days
 Very fussy, not tolerating feeds
 Erythematous blanching rash on day 3
now gone
 WBC - 50’s consistently, no blasts seen
hgb 99 -105 plts low 400’s
 Cultures negative
 Mild resp distress, likely b/c HSM (CXR N)

Case (cont)
Repeat SWU negative
 U/S abdo – significant HSM
 hem/onc investigations started


At 11 days – plt – 1340, rpt at 1360

Echo – 3 giant coronary aneurisms
Dx of KD
Supporting Evidence for KD








Sterile pyuria – 70%
Arthritis – 40%
GI symptoms (hydrops of GB and diarrhea) –
25%
Aseptic meningitis
Anterior uveitis
Irritability
Elevated ESR
Thrombocytosis at 1 wk from start of symptoms
Incomplete KD
At least 10% of children will CAAs never
meet diagnostic criteria
 Review of 127 pts Tx for KD

– 36% did not meet criteria, but this grp had
higher incidence of CAAs than those who did
meet criteria
Infants < 1 y have up to 45% incomplete
KD
 Up to 30% of atypical KD < 1yo had CAAs

Incomplete KD in Infants
Review of KD Dx in
children < 6 mo
 Of 33 cases reported,
28% had atypical
disease compared to
7-10% in > 1yo.

Genizi et al Clin Pediatr 2003 42:263
%
fever
97
rash
82
conjunctivitis
61
Extremities
61
MM
57
nodes
30
KD-Initial Therapy
Lang et al Best Pract Res Clin Rheum 2002 16(3):427
IVIG in KD

Dosing schemes
– 400 mg/kg x 4 days
– 1 g/kg x 1
– 2 g/kg x1
Highest reduction of CAAs and quickest
relief of fever in 2g/kg dose
 May be repeated fever not responsive in
24-48 hrs

Tx and Timing of Presentation
Children Tx with IVIG within 10 days of
symptoms have reduced rates of giant
CAAs (<2%) compared with those Tx at
15 days (6.1%)
 Benefit after 10 days has not been
adequately studied.
 Current suggestion include IVIG if still
evidence of active inflammation (ie fever)

Case
Risk factors for MI in Kids
MI Etiology in Neonates

Congenital heart disease
–
–
–
–
–







Anomalies of the origin of the coronary arteries
Anomalies producing left ventricular hypertrophy
Pulmonary atresia with intact ventricular septum
Transposition of the great arteries
Truncus arteriosus
Endocardial fibroelastosis
Mediocalcinosis of the coronary arteries
Poor coronary perfusion
Hypoxia
Sepsis
Disseminated intravascular coagulation
Embolism
MI Etiology in Children





Kawasaki disease
Idiopathic or inherited
cardiomyopathy
Myocarditis (Viral, Rheumatic)
Collagen vascular disease
Substance abuse

–
–
–
–
–
Homocysteinuria
Familial hypercholesterolemia
Progeria
Pseudoxanthoma elasticum
Mucopolysaccharidoses, Fabry's,
Alkaptonuria, Hurler's syndrome,
Pompe's Disease
– Hyperbetalipoproteinemia, familial
combined hyperlipidemia, and
hypoalphalipoprotenemia
– Cocaine
– Glue sniffing





Chest trauma
Iatrogenic
Post-cardiac transplant
Post bypass hypoperfusion
Other complications of
congenital heart disease
surgery, especially damage to
coronary arteries.
Genetic/metabolic disorders

Other systemic disorders
–
–
–
–
Nephrotic syndrome
Sepsis
Occult malignancy
Idiopathic or premature coronary
artery disease without
predisposing genetic disease
Symptoms of MIs in Kids
– Nausea
– Vomiting
– Irritability
– Pallor
– Diaphoresis
– Poor feeds
– Syncope
– Weakness
– WOB/SOB
– Anxiety
– Pain
Pediatric MIs on ECG

Wide Q waves (>35 milliseconds)
– Particularly I, aVL, V5, V6, but any lead other than
aVR

ST segment changes of >2 mm
– Elevation in any lead, especially in the presence of
reciprocal changes.
– ST depression in V1 -V3 .

Ventricular arrhythmias
– Calculated QTc of >0.48.


Based on autopsy proven MI
? Prospective use?
(Towbin JA Am J Card 1992 70:1545)
Reich JD et al AJ Emerg Med 1998 16(3):296
Troponin Levels in Kids
< 7 days
 8-30 days
 31-120 days
 > 120 days

<0.35
<0.2
<0.1
<0.03
Tx for MI in Children
Very small literature bank, most on KD kids
 Only case reports of streptokinase or urokinase
 Theoretical risk of using streptokinase as it is
contraindicated in pts with recent GAS infection
(and what kid doesn’t meet that criteria?!)
 tPA has been used

– Case study from 2000 in 7yo boy post-KD
– Resolution of symptoms, improved LV function and no
thrombus seen on cath at 1 month (echo + @
presentation)
Krendel et al Ann Emerg Med 2000 35(5): 502
Tx for MI in Children
Angioplasty pretty difficult in children <5
 IIa/IIIb inhibitors are not approved in
children (and may have a higher risk of
bleeding?)

Cardiac Arrests in Kids
20 yrs of cardiac arrest attended by EMS
 Of 5505 arrests, 2% were in children (017 y.o.)
 Overall survival = 5%

Engdahl J et al Resuscitation 2003 58:131
Cardiac Arrests in Kids
Majority of CA
occur in 0-1 yo
 Mostly related
to SIDS and
respiratory
causes

Cardiac Arrests in Kids
