TheCrashing

Cardiac Baby

Or: Holy $#@! This Kid Looks Sick

Dan ParkMUSC

Pediatric

Emergency Medicine

10%You only retain

of a 1 hr lecture after 3 weeks

(Bligh 2000)heard somewherehigh estimatewant to hit the most high yield practical information for you if you take away 10% i’ll be pumped

Congestive heart failure

Kawasaki disease

Myocarditis

Hypertrophic cardiomyopathy

Arrhythmias

Infective endocarditis

Heart transplant

Corrected congenital heart

disease

Congenital heart disease

Pediatric Cardiac Emergencies

broad topicwhy i chose it: super interesting, scares the shit out of meteaching it makes me understand it better

60 combined years of pediatric ER experience

coarctation of aorta

hypoplastic left heart post surgical

V fib

single ventricle

coarctation of aorta

coarctation of aorta

coarctation of aorta

Unstable SVT

Unstable SVT

V fibasked 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

Availability Bias

when you overestimate the probability of events associated with memorable or vivid occurrences

For the nerds:It impacts your PRETEST PROBABILITY

or underestimate things you haven’t seen in a while

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

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

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 xrayb. cardiology consultc. dopamine infusiond. epinephrine infusione. prostaglandin infusion

ABCs + Dextrose

MANTRA ABCsA: 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

ABCs + Dextrose

MANTRA ABCsA: 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

4objectives

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

Objective 1

LEARN THE COMMON PRESENTATIONS OF CONGENITAL HEART DISEASE

CHF

CYANOSIS

SHOCK

BALANCED

4 PRESENTATIONS IN PEDS CARDIAC PATIENTS

Left-sided lesion

Right-sided 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

Objective 2

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

Let’s review fetal circulation

FO DA

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.

STRUCTURALNONSTRUCTURAL

Cardiac emergencies come in two flavors: structural and nonstructural

non structural emergencies include arrhythmias (bradycardia and tachycardia) and disorders of myocardial function

STRUCTURAL

PRESSURE OVERLOAD (OBSTRUCTIVE)

VOLUME OVERLOAD

VOLUME OVERLOADVSD

ASD

Total anomalous pulmonary venous return

AV canal

Truncus arteriosus

PDA

Double outlet right ventricle

Ebstein anomaly

VOLUME OVERLOAD25% of CHD

L R shunt can lead to right sided

heart failure

Can’t finish a feed

Failure to thrive

Hepatomegaly

Pressure Overload (Obstructive)

1. CARDIOVASCULAR COLLAPSE

2. GRADUAL DYSFUNCTION

AKA DUCTAL DEPENDENT LESIONS

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

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

DUCTAL DEPENDENT LESIONS

LEFT SIDED (BLOOD CAN’T GET TO BODY)

RIGHT SIDED (BLOOD CAN’T GET TO LUNGS)

IT

DOESN’T

MATTEReach 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

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

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.

TRANSPOSITION

OF

THE

GREAT

VESSELSaorta 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.

TRICUSPIDATRESIA

tricuspid valve doesn’t form so blood won’t make it from the RA into the RVyou 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

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

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

Objective 3

How do you go about diagnosing this?

H&P

CXR/EKG

HYPEROXIA TEST

DIFFERENTIAL DX

VITALS

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:

Trauma

Heart

Endocrine

Metabolic

Inborn errors

Seizures

Formula

Intestinal catastrophes

Toxins

Sepsis

The crashing neonate is scaryhaving a go-to ddx might relieve some anxiety sepsis in a sick newborn until proven otherwise

HISTORICAL CLUES

Poor feeding

Irritability

Diaphoresis with feeds(stress test)

these suggest a heart issue

EXAM

Murmur+ concerning history= get EKG and CXR

Tachypnea

CHF: hepatomegaly, gallop

gallop= 3 or 4 heart sounds instead of 2

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.

tall R waves

inverted T waves

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 monthsIf you see left axis deviation in the first week of life you should suspect a CHD

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.

tall R waves

inverted T waves

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 monthsIf you see left axis deviation in the first week of life you should suspect a CHD

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 fallotshape 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

1. VSD

2. RV outflow tract narrowing

3. Overriding

aorta

4. RV hypertrophy

Tetralogy of Fallot

KNEE TO CHEST/

BOLUS

phenylephrine

MORPHINE

OXYGEN

SVR

PVR

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 fallotshape 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

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

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

VITALS

Pre and post ductal O2 saturations

4 extremity BPs

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

Hyperoxia TestAnswers the question: Is it a lung problem or a heart problem?

Textbook: 1. Get an ABG on room air2. Put patient on 100% O2 for 10 mins3. Get another ABGIf it’s a lung problem the PaO2 will be >150 mmHgIf 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.

Hyperoxia TestAnswers the question: Is it a lung problem or a heart problem?

Textbook: 1. Get an ABG on room air2. Put patient on 100% O2 for 10 mins3. Get another ABGIf it’s a lung problem the PaO2 will be >150 mmHgIf 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.

Hyperoxia TestAnswers the question: Is it a lung problem or a heart problem?

Textbook: 1. Get an ABG on room air2. Put patient on 100% O2 for 10 mins3. Get another ABGIf it’s a lung problem the PaO2 will be >150 mmHgIf 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.

Know what to do if this kid comes into your ER

Objective 4

TAKE YOUR OWN PULSE

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

BALANCE PULMONARY AND SYSTEMIC BLOOD FLOW

(Qp=Qs)

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

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

PROSTAGLANDINSPROSTAGLANDINS(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

0.03 to 0.1

mcg/kg/minDoesn’t require central accessIf you have extra support you’ll call cardiology and they will echo the kidProstaglandin drip continues until they go to the OR

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.

PRESSORS

RIGHT SIDED LESIONS

LEFT SIDED LESIONS

(blood shunted AWAY from pulmonary circulation)

(blood shunted TOWARD pulmonary circulation)

PROBLEM SOLUTION

Increase systemic circulationDecrease afterload

MILRINONE/DOBUTAMINE

Decrease systemic circulationIncrease SVR

EPI/NE/HIGH DOSE DOPA

Qp:Qs = 1:1your 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

Sodium Bicarb: “Basically Useless Therapy”

(Aschner. Pediatrics. 2008)

“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”

Usher 1967Simmons 1974Papile 1978

van Alfen-van der Velden 2006

Lipshultz 2003

Sirieix 1997Marangoni 1995

Wakabayashi 1994

Awada 2007

Evidence For Evidence Against

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

Sodium Bicarb: “Basically Useless Therapy”

(Aschner. Pediatrics. 2008)

“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”

Usher 1967Simmons 1974Papile 1978

van Alfen-van der Velden 2006

Lipshultz 2003

Sirieix 1997Marangoni 1995

Wakabayashi 1994

Awada 2007

Evidence For Evidence Against

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

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.

THE YOU SHOULD TAKE AWAY FROM THIS10%

AVAILABILITY BIAS

Get IV/IO access ASAP

HYPEROXIA TEST

PROSTAGLANDINS

0.03-0.1 mcg/kg/min

APNEA

THE MISFITS

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

THANK YOU

GOOD LUCK

TRY NOT TO SUCK OUT THERE

AND