a not-for profit/sale presentation for educational purposes only. Design heavily influenced and inspired by Jesse Desjardins. Thank you to Jesse Desjardins.

1. The
ashing
Cr
c Baby
Cardia
Looks Sick
@! This Kid
Or: Holy $#
Dan Park
MUSC
Pediatric
cy Medicine
Emergen

2. You only retain
10%
of a 1 hr lecture after
3 weeks
(Bligh 2000)
heard somewhere
high estimate
want to hit the most high yield practical information for you
if you take away 10% i’ll be pumped

3. Pediatric Cardiac Emergencies
Kawasaki disease
Arrhythmias
Congenital heart disease
Congestive Myocarditis
heart
Infective
failure Corrected
endocarditis
congenital heart
disease Hypertrophic
Heart
transplant
cardiomyopathy
broad topic
why i chose it: super interesting, scares the shit out of me
teaching it makes me understand it better

4. 60 combined years of pediatric ER experience
hypoplastic left heart
coarctation of aorta
post surgical
Unstable SVT
coarctation of aorta
coarctation of aorta
V fib
Unstable SVT
V fib
single ventricle
coarctation of aorta
asked most experienced ER attendings how many times they’ve taken care of a crashing
cardiac kid
this works out to about once every 5 years
that’s not nothing, but it happens rarely enough that we might start to forget stuff

5. when you
overestimate the
probability of
events associated
with memorable or
vivid occurrences
or underestimate
things you haven’t For the nerds:
seen in a while
It impacts your
PRETEST
PROBABILITY
ilit
ilab
Ava
ias
yB
common trick that your mind plays on you that can lead to misdiagnosis or error
its the tendency to judge probabilities based on how easily examples come to mind
you don’t see crashing cardiac kids often so your mind doesn’t even consider them as a
possibility when you see a sick kid

6. Most of the kids that come through the peds side are healthy and will do fine no matter what
you do
Your real job is to find the needle in the haystack. This is also called Ruling out the worst
case scenario.
The whole point of spending time in the peds ER is to see a shit ton of healthy kids so you
know what a sick one looks like when you see one

7. CASE
7 day old boy is brought to the ED by his frantic father. He
reports that his son was having difficulty feeding throughout
the day and then became limp in his arms just before
presentation. On exam, you note a cyanotic infant with the
following vital signs: T 37. C, HR 165, RR 65 (labored), and
BP 72/palp. The patient is poorly perfused and the pulse
oximetry reading is not detectable. The intervention most
likely to benefit this child is a:
a. Stat chest xray
b. cardiology consult
c. dopamine infusion
d. epinephrine infusion
e. prostaglandin infusion

8. ABCs + Dextrose
MANTRA
ABCs
A: airway issues are a special consideration in kids with heart disease and other potential midline defects as part
of a congenital syndrome
(like choanal atresia (nasal passage doesn’t develop fully; this is a life-threatening event because infants only
breathe through their nose) and Pierre Robin syndrome (their small jaws might make intubation difficult; 20% of
kids with pierre robin will have a congenital heart defect, Pearl 1982)
C: you want to get IV or IO access as soon as possible in a sick neonate.
D: always check a sugar in a sick looking kid

9. ABCs + Dextrose
MANTRA
ABCs
A: airway issues are a special consideration in kids with heart disease and other potential midline defects as part
of a congenital syndrome
(like choanal atresia (nasal passage doesn’t develop fully; this is a life-threatening event because infants only
breathe through their nose) and Pierre Robin syndrome (their small jaws might make intubation difficult; 20% of
kids with pierre robin will have a congenital heart defect, Pearl 1982)
C: you want to get IV or IO access as soon as possible in a sick neonate.
D: always check a sugar in a sick looking kid

10. 4
objectives
1. Become familiar with the common presentations
of CHD in the neonate
2. Broadly classify the type of cardiac lesion
(not the specific defect)
3. Establish a diagnostic game plan for yourself
if you see this in your ER
4. Know how to treat a kid with CHD in extremis

11. Objective
1
LEARN THE
COMMON
PRESENTATIONS
OF CONGENITAL
HEART DISEASE

12. CYANOSIS
SHOCK
CHF
BALANCED

13. 4 PRESENTATIONS IN PEDS CARDIAC PATIENTS
Leftsided
lesion
Rightsided
lesion
Busy slide but has a lot of good info in it so I kept it intact
Rapid onset of intense cyanosis strongly suggests a ductal dependent congenital heart
defect.
In left-sided lesions supplemental O2 should be used sparingly in these infants because high
O2 levels will make the ductus close quicker

14. Objective
2
Classify the type of
cardiac lesion. Not
the specific defect
(that’s what
cardiologists are
paid for)

15. Let’s review
fetal circulation
DA
FO
DV
The umbilical vein brings oxygenated blood from placenta through DUCTUS VENOSUS to the IVC.
Blood enters the RA where it goes through the FO to the LA, bypassing the RV and pulmonary circulation.
Deoxygenated blood from upper body comes from the SVC to the RA and down to the RV and out the pulmonary
artery.
Since the pulmonary system is vasoconstricted only 10% goes to the lungs.
The rest of the blood takes the path of least resistance into the DA, a connection to the descending aorta.
When the baby takes its first breath the increase in O2 results in a huge decrease in pulmonary vascular resistance
and blood starts flowing to the lungs.
Overs hours to days the ductus and foramen ovale closes.

16. STRUCTURAL
NONSTRUCTURAL
Cardiac emergencies come in two flavors: structural and nonstructural
non structural emergencies include arrhythmias (bradycardia and tachycardia) and disorders
of myocardial function

17. STR
UCT
UR
AL
VOLUME OVERLOAD
PRESSURE OVERLOAD
(OBSTRUCTIVE)

18. VOLUME OVERLOAD
VSD
Total anomalous pulmonary venous return
PDA
ASD
Truncus arteriosus
Double outlet right ventricle
AV canal
Ebstein
anomaly

19. VOLUME OVERLOAD
25% of CHD
L R shunt can
lead to right sided
heart failure
Can’t finish a feed
Failure to thrive
Hepatomegaly

20. Pressure Overload (Obstructive)
AKA DUCTAL DEPENDENT LESIONS
1. CARDIOVASCULAR COLLAPSE
2. GRADUAL DYSFUNCTION
will present in 2 ways
The first, the more dramatic form presents in newborns with cardiovascular collapse because
blood won’t be able to flow into the pulmonary or systemic circulation.
The second is seen with INCOMPLETE forms of obstructive lesions that will gradually cause
dysfunction

21. DUCTAL DEPENDENT LESIONS
LEFT SIDED (BLOOD CAN’T GET TO BODY)
RIGHT SIDED (BLOOD CAN’T GET TO LUNGS)
each defect could be an hour lecture
people do entire fellowships to learn this stuff
trivia at this point for us
same emergency treatment for all of them

22. DUCTAL DEPENDENT LESIONS
IT
LEFT SIDED (BLOOD CAN’T GET TO BODY)
DOESN’T
RIGHT SIDED (BLOOD CAN’T GET TO LUNGS)
MATTER
each defect could be an hour lecture
people do entire fellowships to learn this stuff
trivia at this point for us
same emergency treatment for all of them

23. The Classic Cyanotic Congenital Heart Lesions
Mnemonic time!
If you’re dying to come away with this knowing a few congenital lesions these are probably
the best ones to know for testing purposes.
These are Right-sided lesions and will present as a cyanotic baby

24. TRUNCUS
ARTERIOSUS
1 finger because aorta and pulm artery emerge as a single vessel allowing total mixing of
deoxygenated and oxygenated blood.
This is lesion will cause volume overload.
Kids are born cyanotic.

25. TRANSPOSITION
OF
THE
GREAT
VESSELS
aorta comes off the RV
pulmonary artery comes off the LV
you need some sort of mixing lesion to survive
its both a mixing and obstructive lesion: mixing until the ductus closes then they develop
severe cyanosis.

26. TRICUSPID
ATRESIA
tricuspid valve doesn’t form so blood won’t make it from the RA into the RV
you need either a mixing lesion to get from from the right to left side
then you need a PDA to get blood to the lungs

27. TETRALOGY
OF
FALLOT
we’ll get to this one in a few slides but you put up four fingers because there are four
features involved

28. TOTAL
ANOMALOUS
PULMONARY
VENOUS
RETURN
5 fingers because there are 5 words in the name
pulmonary veins don’t return to the LA so no oxygenated blood pumped out of aorta unless
you have a mixing lesion

29. Objective
3
How do
you go
about
diagnosing
this?

30. H&P
VITALS
HYPEROXIA TEST
CXR/EKG
DIFFERENTIAL DX
We don’t see this all the time so we need a very concrete way of dealing with this
The work up for CHD includes:

31. Trauma
Seizures
Heart
Formula
Endocrine
Intestinal
Metabolic
Inborn errors
The crashing neonate is scary
having a go-to ddx might relieve some anxiety
sepsis in a sick newborn until proven otherwise
catastrophes
Toxins
Sepsis

32. HISTORICAL CLUES
Poor feeding
Irritability
Diaphoresis with feeds
(stress test)
these suggest a heart issue

33. EXAM
Murmur+ concerning history=
get EKG and CXR
Tachypnea
CHF: hepatomegaly, gallop
gallop= 3 or 4 heart sounds instead of 2

34. inverted T waves
tall R waves
Infant ECG with right ventricular hypertrophy. Signs of right
ventricular hypertrophy include larger than normal amplitudes
of R and inverted T waves in right precordial leads.
Axis: normal newborns have a right axis deviation, normal QRS is RIGHT and ANTERIOR (+135
to +180)
R waves will be higher in the right/septal precordial leads (V1 and V2)
T waves are typically inverted in all the precordial leads
Axis will slowly evolve into the normal leftward axis over the next few months
If you see left axis deviation in the first week of life you should suspect a CHD

35. inverted T waves
tall R waves
Infant ECG with right ventricular hypertrophy. Signs of right
ventricular hypertrophy include larger than normal amplitudes
of R and inverted T waves in right precordial leads.
Axis: normal newborns have a right axis deviation, normal QRS is RIGHT and ANTERIOR (+135
to +180)
R waves will be higher in the right/septal precordial leads (V1 and V2)
T waves are typically inverted in all the precordial leads
Axis will slowly evolve into the normal leftward axis over the next few months
If you see left axis deviation in the first week of life you should suspect a CHD

36. You won’t dx CHD on CXR but it might make you more suspicious
In kids, cardiomegaly is usually defined as a cardiac silhouette >60% of the AP diameter
Remember, the thymus can make reading infant xrays difficult.
So get a lateral to look for true cardiomegaly.
Boot shaped heart chest xray in an infant with tetralogy of fallot
shape results from right ventricular hypertrophy due to right ventricular outflow tract
obstruction
Tetralogy of Fallot accounts for 10% of the cases of congenital heart disease.
It is the most common cyanotic heart defect
tet spell is a cyanotic episode due to worsening of the R to L shunt

37. Tetralogy of Fallot
KNEE
TO CHEST/
BOLUS
1. VSD
SVR
2. RV outflow
tract narrowing
phenylephrine
3.
Overriding
aorta
MORPHINE
PVR
4. RV
hypertrophy
OXYGEN
You won’t dx CHD on CXR but it might make you more suspicious
In kids, cardiomegaly is usually defined as a cardiac silhouette >60% of the AP diameter
Remember, the thymus can make reading infant xrays difficult.
So get a lateral to look for true cardiomegaly.
Boot shaped heart chest xray in an infant with tetralogy of fallot
shape results from right ventricular hypertrophy due to right ventricular outflow tract
obstruction
Tetralogy of Fallot accounts for 10% of the cases of congenital heart disease.
It is the most common cyanotic heart defect
tet spell is a cyanotic episode due to worsening of the R to L shunt

38. Another classic xray finding in CHD is known as the Egg on a String
Narrowing of superior mediastinum (anterior position of aorta) and globular shape of heart in
neonates with transposition of great arteries

39. Snowman sign- total anomalous pulmonary venous return
Again, it doesn’t matter what the specific defect is. As long as you can determine this xray
doesn’t look right you’ve done your job

40. VITALS
4 extremity BPs
Pre and post ductal O2
saturations
two unique vital signs to check in a kid with suspected CHD
preductal sat will be from the right wrist or finger
post ductal from the lower extremities
in coarctation of the aorta you’ll have low lower extremity BPs and lower post ductal sats

41. Hyperoxia Test
Answers the question: Is it a lung
problem or a heart problem?
Textbook:
1. Get an ABG on room air
2. Put patient on 100% O2 for 10
mins
3. Get another ABG
If it’s a lung problem the PaO2 will be
>150 mmHg
If it’s a cardiac problem it will be
<100
Quick and dirty:
Put kid on 100% O2
and see if their sats
increase
If the kid’s O2 sat doesn’t respond you’re looking at a CHD and then you’re aiming for an O2
sat between 70-80%
You don’t need a cardiologist or any other help to do this.

42. Hyperoxia Test
Answers the question: Is it a lung
problem or a heart problem?
Textbook:
1. Get an ABG on room air
2. Put patient on 100% O2 for 10
mins
3. Get another ABG
If it’s a lung problem the PaO2 will be
>150 mmHg
If it’s a cardiac problem it will be
<100
Quick and dirty:
Put kid on 100% O2
and see if their sats
increase
If the kid’s O2 sat doesn’t respond you’re looking at a CHD and then you’re aiming for an O2
sat between 70-80%
You don’t need a cardiologist or any other help to do this.

43. Hyperoxia Test
Answers the question: Is it a lung
problem or a heart problem?
Textbook:
1. Get an ABG on room air
2. Put patient on 100% O2 for 10
mins
3. Get another ABG
If it’s a lung problem the PaO2 will be
>150 mmHg
If it’s a cardiac problem it will be
<100
Quick and dirty:
Put kid on 100% O2
and see if their sats
increase
If the kid’s O2 sat doesn’t respond you’re looking at a CHD and then you’re aiming for an O2
sat between 70-80%
You don’t need a cardiologist or any other help to do this.

44. Objective
4
Know what
to do if
this kid
comes into
your ER

45. TAKE YOUR
OWN PULSE

46. CLASSIFICATION OF LESION
TEMPO OF PRESENTATION
SEVERITY OF CLINICAL CONDITION
aggressiveness of your therapy will be determined by:
the type of lesion (whether its left sided or right sided)
how fast they decompensated
and how bad they look

47. BALANCE PULMONARY AND
SYSTEMIC BLOOD FLOW
(Qp=Qs)

48. BE CAREFUL WITH OXYGEN
IF O2 IS NOT HELPING SHUT IT OFF
SUSPECTED CHD: AIM FOR O2 75-85%
THAT’S A GAS OF 7.4/40/40
remember, oxygen is a potent pulmonary vasodilator,
it might make a left sided lesion worse making shock worse
also a PDA will close with more O2

49. FOR SUSPECTED CHD (failed hyperoxia test)
10 cc/kg NS BOLUS IS A GOOD PLACE TO START
for any super sick looking neonate you will rarely hurt them with O2 and a 20cc/kg NS bolus.
If your suspicion for a congenital heart defect is high then you might want to be a little more
careful with your oxygen and fluid bolus.
it’s easier to add fluids than take them away

50. PROSTAGLANDINS
PROSTAGLANDINS
(PGE1)
this is all you really need to remember from this talk
its a strong vasodilator that has a direct effect on smooth muscle of ductus arteriosus
you should consider this when any infant presents with severe hypoxemia or shock in the first
few weeks of life
decision to start a prostaglandin drip should be made clinically and not on a definite
diagnosis

51. 0.03 to
0.1
mcg/kg/min
Doesn’t require central access
If you have extra support you’ll call cardiology and they will echo the kid
Prostaglandin drip continues until they go to the OR

52. APNEA 12%
FEVER 10%
Elective intubation might be considered before transporting a kid requiring a prostaglandin
infusion
it’s probably a good idea to have your airway equipment by the bedside if you’re about to
start infusing
Fever is less of an issue because most of these sick looking kids will get a full septic work up
anyways.

53. PRESSORS
PROBLEM
LEFT SIDED LESIONS
(blood shunted TOWARD
pulmonary circulation)
RIGHT SIDED LESIONS
(blood shunted AWAY from
pulmonary circulation)
SOLUTION
Increase systemic circulation
Decrease afterload
MILRINONE/DOBUTAMINE
Decrease systemic circulation
Increase SVR
EPI/NE/HIGH DOSE DOPA
Qp:Qs = 1:1
your main job is to stabilize these kids and send them to an icu
but if you’re stuck by yourself in a small ER and have to manage these kids without help you
might have to start some pressors

54. Sodium Bicarb: “Basically Useless Therapy”
“Insufficient data to recommend routine use of bicarb in
resuscitation of the newly born. In fact, the hyperosmolarity
and CO2 generating properties may be detrimental to
myocardial or cerebral function”
Evidence For
Evidence Against
Usher 1967
Simmons 1974
Papile 1978
Wakabayashi 1994
Marangoni 1995
Sirieix 1997
van Alfen-van der Velden 2006
Lipshultz 2003
Awada 2007
(Aschner. Pediatrics. 2008)
The use of bicarb in crashing cardiac kids is controversial.
Everyone does it but there’s really no evidence to show that it works.
It probably does more harm than good.
But if you’re in a code situation and you’ve tried everything and the kid still looks terrible no
one will fault you for throwing in some bicarb.
The point is that if you can’t ventilate the kid well then bicarb prob won’t help

55. Sodium Bicarb: “Basically Useless Therapy”
“Insufficient data to recommend routine use of bicarb in
resuscitation of the newly born. In fact, the hyperosmolarity
and CO2 generating properties may be detrimental to
myocardial or cerebral function”
Evidence For
Evidence Against
Usher 1967
Simmons 1974
Papile 1978
Wakabayashi 1994
Marangoni 1995
Sirieix 1997
van Alfen-van der Velden 2006
Lipshultz 2003
Awada 2007
(Aschner. Pediatrics. 2008)
The use of bicarb in crashing cardiac kids is controversial.
Everyone does it but there’s really no evidence to show that it works.
It probably does more harm than good.
But if you’re in a code situation and you’ve tried everything and the kid still looks terrible no
one will fault you for throwing in some bicarb.
The point is that if you can’t ventilate the kid well then bicarb prob won’t help

56. QUICK COMMENT ON KIDS BETWEEN STAGES
OF CHD REPAIR
Some kids are completely supported by a single
surgical intracardiac shunt
These shunts might clot
Can’t open a PDA anymore
Bolus with fluids, pressors, call cards,
Needs to go to OR
For the really bad lesions like hypoplastic left heart the repair process is made in stages as
the kid grows.
Partially corrected kids might present to you crashing.

57. THE 10% YOU SHOULD TAKE AWAY FROM THIS
AVAILABILITY BIAS
Get IV/IO access ASAP
THE MISFITS
HYPEROXIA TEST
PROSTAGLANDINS
0.03-0.1 mcg/kg/min
APNEA
These conditions are rare but not THAT rare. Have a healthy respect for them and look for the
needle in the haystack if a doesn’t look well
Know how to put an I/O in a kid
THE MISFITS mnemonic will give you the differential for a crashing baby: trauma, heart,
endocrine, metabolic, inborn errors, seizures, formula issues, intestinal catastrophes, toxins,
sepsis
Put the kid on 100% oxygen and see if their sats go up at all. If not, aim for sats between
75-85% with as minimal O2 as possible
Have a low threshold to start prostaglandins on super sick looking newborns that don’t
respond to oxygen
But be prepared to intubate them if they go apneic

58. THANK YOU
GOOD LUCK
AND
TRY NOT TO SUCK OUT THERE