Welcome to the bed. We'll go ahead and give you
the story. This is all going to happen super
fast. Welcome to the emergency room. Welcome
back to the deep dive. Today, we are staring
down the beast. And I say that with a healthy
amount of, you know, respect, but let's be honest.
Pediatric cardiac nursing is the monster under
the bed for a lot of students. It really is.
Even experienced nurses transitioning to the
PICU find this stuff. daunting. It is massive.
It's physically intimidating because you're dealing
with these tiny humans. I mean, sometimes neonates
weighing less than a bag of sugar. Right. But
then intellectually, it's just so much to juggle.
You have plumbing issues, which is the actual
structure of the heart. You have electrical issues
and then you have the pharmacology. Oh, the pharmacology.
Which is a complete minefield because the safety
margins in pediatrics are razor thin. What works
for an adult can be a fatal dose for a neonate.
Exactly. I was just looking at the stack of sources.
as we have for today. And it's a stack. We've
got this huge comprehensive textbook chapter
on cardiovascular disorders, both congenital
and acquired. And then we have these incredibly
dense drug monographs for the real heavy hitters.
We're talking alprosadil, indomethacin, furosemide,
heparin. It's a lot of data to synthesize. It
is a lot, but our mission today is to cut right
through that noise. We are not just going to
read slides here. We are going to cross reference
the disease processes with that medication database
to build what we call the Pareto 80 -20 guide.
The Pareto principle. I love this. The idea that,
what, 20 % of the causes lead to 80 % of the
results. Precisely. So the goal is to give you,
the listener, the 20 % of the cardiac concepts
that are going to answer 80 % of your exam questions.
And maybe more important. More importantly, yeah,
prevent that. that failure to rescue scenario
at the bedside. That's the real goal. Is moving
from just memorizing lists of signs and symptoms
to actually understanding the patterns. Exactly.
If you understand the flow, the basic physics
of the blood, you understand the defect. If you
understand the defect, the medication suddenly
makes perfect sense. You don't have to memorize
that endomethacin closes the duct if you understand
why the duct is open and why that's a problem
in that specific kid. So we're orienting this
whole deep dive for the learner today. Whether
you're cramming for your finals, studying for
the NCLEX, or prepping for your very first shift
in the PICU, this is designed to keep you safe
and get you those keywords. Yep. Let's do it.
Let's dive in. So before we get into the alphabet
soup of defects, the ASDs, VSDs, TOFs, you've
pulled together what you're calling a master
80 -20 map. I have. These are the high yield
patterns that seem to apply across the board.
Yeah. Can you walk us through this map? I feel
like we can get this framework right. Everything
else just kind of falls into place. Right. Before
you even try to memorize a single defect, you
have to understand the rules of the road. And
pattern number one is simply the flow problem.
It's hemodynamics. OK. And the golden rule here
is simple physics. Blood follows the path of
least resistance. Always. It's like water flowing
downhill. It's just gravity. High pressure to
low pressure. It's never going to decide to swim
upstream unless something forces it to. Exactly.
So think about a normal, healthy heart. The left
side is the high pressure pumping system. And
why is that? It has to pump to the whole body.
The whole body. It has to push blood all the
way down to your toes, up to your brain, out
to your fingertips. It needs torque. It needs
serious muscle. The right side, on the other
hand, is a low pressure receiving system. It
only has to get the blood next door. Just next
door to the lungs. It's a very short trip. Low
resistance. So just structurally, the left ventricle
is physically thicker and stronger? Therefore,
the pressure on the left is naturally going to
be much higher than the pressure on the right.
Correct. So just burn that gradient into your
mind. Left is high, right is low. Now, what happens
if there's a hole connecting them, a septal defect,
for example? Physics takes over. Physics takes
over. Blood is going to flow from the high pressure
left side over to the low pressure right side.
We call this a left to right shunt. Which means
that nice oxygenated blood that was supposed
to go out to the body it's already been to the
lungs, it's pink, it's ready to go, is now sneaking
back into the right side. And going back to the
lungs for another lap. A totally unnecessary
trick. It's inefficient, it's recirculating,
and that right there gives you your first major
category of defects, increased pulmonary blood
flow. OK. These kids are pink because their blood
is oxygenated. In fact, it's too oxygenated in
the lungs. But their lungs are flooded. They
are wet. They have pulmonary congestion. They're
drowning in their own circulation. Pink and wet.
OK, so that's side A of the map. What is the
flip side? The reverse scenario. Yeah. What if
there is an obstruction, a blockage, a kink in
the hose that prevents blood from getting to
the lungs in the first place? Like a stenotic
valve. A stenotic pulmonic valve, for example.
Or if the pressure on the right side somehow
becomes effectively higher than the left for
some reason, blood will shunt right to left.
It completely skips the lungs. And if it skips
the lungs, it never picks up oxygen, and it stays
deoxygenated. It's blue blood. Exactly. That
blue, unoxygenated blood goes straight from the
right side through a defect to the left side
and out to the body. These are your decreased
pulmonary blood flow defects. And these are the
blue babies. These are the blue babies. They
are cyanotic. And this is almost always a medical
emergency. Wow. Okay, so just that first distinction.
Pink wet lungs versus blue dry lungs. That's
the first major fork in the road. That simplifies
things immensely. It's the master sorting hat
for congenital defects. What's pattern number
two on the map? Pattern number two is what I
call the ductal dependent safety net. This is
so critical for safety, and it's a concept that
trips people up constantly on exams. We all have
a ductus arteriosus as a fetus. It's a little
vessel, a tunnel, that connects the pulmonary
artery to the aorta. Its job is to bypass the
lungs because the fetus isn't using them. It's
supposed to close shortly after birth. We'll
get into that whole fetal transition in a minute,
but the core concept here is that for some of
these babies, that little duct is the only thing
keeping them alive. Exactly. If you have a severe
obstruction, like a completely blocked valve,
that duct might be the only way blood gets where
it needs to go. It becomes a temporary life -saving
bridge. So the safety item, the thing you can't
miss... Is knowing when we need to keep it open.
using prostaglandins and when we need to close
it using something like endomethacin. You mix
those two up. You close a duct on a baby who
needs it open. You kill the patient. It's that
stark. That is a sobering thought. But that's
why we learn the mechanisms. It's not just memorization.
Okay, pattern three. Pattern three is acquired
inflammation. So now we're shifting gears. These
are hearts that were built correctly. The plumbing
was fine at birth. No holes, no valve issues.
Everything was in the right place. But then something
attacks the heart. Exactly. Inflammation attacks
the system. Here we're talking about things like
Kawasaki disease, rheumatic fever, infective
endocarditis. So for these, the exam key isn't
about shunts and pressures. It's about recognizing
the triggers and the unique symptoms. Yes. It's
pure pattern recognition. A history of strep
throat two weeks ago for rheumatic fever, the
classic strawberry tongue for Kawasaki. And those
unique signs like Osler nodes for endocarditis,
these are classic textbook symptoms that you
actually see in real life, and exam writers love
them. OK, that makes sense. And finally, pattern
four on our map. Pattern four is the final destination.
It's the consequence. If you don't manage the
defects, if you don't manage the inflammation,
you often end up at the same place. Heart failure.
But in pediatrics, heart failure looks really
different than in adults, right? So different.
It's not just swollen ankles and shortness of
breath. In an infant, it's growth failure. It's
major feeding issues. It's that classic sign
of sweating all they're eating because it's such
hard work. OK, this map is fantastic. So flow
problem. ductal dependence, acquired inflammation,
and heart failure. Let's use that to unpack the
plumbing a bit more. The sources start with the
fetal to newborn transition. They do. And I feel
like you really can't understand what's wrong
if you don't know how it started. This is the
essential context. Absolutely. In utero, the
fetus is living in a fluid environment. The lungs
are basically these little fluid -filled bags.
They aren't doing any gas exchange. The placenta
is the lung. The placenta is the lung, the kidney,
the gut. It does everything. So the lungs and
utero are collapsed, high pressure, high resistance
zones. The fetal heart is brilliantly designed
to bypass them entirely because sending a lot
of blood there is just a waste of energy. And
it does that using two key shortcuts, the trap
doors. Shortcut number one is the Foreman Oval.
This is a literal door, a flap between the right
atrium and the left atrium. So blood comes back
from the body into the right side, hits that
door, and flows straight across to the left side,
completely bypassing the right ventricle and
the lungs. Smart. And shortcut number two is
the ductus arteriosus, which we just mentioned.
This is that tunnel connecting the pulmonary
artery directly to the aorta. So even the small
amount of blood that does get into the pulmonary
artery gets shunted through this tunnel into
the aorta and then out to the body, again, bypassing
the lungs. So in a perfect world, the baby is
born. takes that first big screaming breath,
the lungs expand like a popped balloon. And the
pressure inside the lungs just plummets. It goes
from a high resistance system to a super low
resistance system. Blood suddenly rushes into
the lungs. And that cascade of events slams those
fetal doors shut. It does. The rush of blood
returning from the newly opened lungs raises
the pressure in the left atrium, which physically
pushes that form an oval door shut. At the same
time, oxygen levels skyrocket in the blood, and
the mother's prostaglandins are cut off, which
signals the ductus arteriosus muscle to constrict
and close down, usually within a day or two.
But when they don't close, or when the heart
structure is so abnormal that it forces them
to stay functional, that's the entire basis of
congenital heart disease. It's the whole ball
game. Okay, let's finally get into the defects
themselves. Yeah. We'll start with our first
category from the map, the pink puffers. the
increased pulmonary blood flow group. What are
the main players here? So this is your atrial
septal defect ASD, your ventricular septal defect
VSD, the patent ductus arteriosus PDA. and a
more complex one called the atrioventricular
AV canal. And the unifying theme for all of them
is that left to right shunt we talked about,
high pressure to low pressure. Exactly. Let's
take the ventricular septal defect, VSD. It's
the most common congenital heart defect there
is. You simply have a hole in the wall, the septum,
between the ventricles. So when the big, strong
left ventricle squeezes to pump blood out to
the body, because of that hole, a jet of high
pressure oxygenated blood shoots back across
into the right ventricle. does the right ventricle
send all its blood? Straight to the lungs. So
now the poor lungs are getting the normal amount
of blood they're supposed to get from the right
side. PLUS, this extra high pressure jet of blood
from the left side. The entire pulmonary vascular
system gets flooded and overloaded. So clinically,
what does the nurse see at the bedside? What
are the need -to -know findings for a VSD? You
see signs of heart failure because the heart
is working double time and it's inefficient.
You'll see tachypnea, the breathing fast, sometimes
60, 70, 80 breaths a minute, just trying to compensate
for all that fluid. And a fast heart rate. Tachycardia,
absolutely. But a huge one for exams and for
real life clinical practice is frequent respiratory
infections. Because wet lungs are just a perfect
breeding ground for bacteria. It's a swamp. It's
a swamp, exactly. Fluid stasis leads to pneumonia
and bronchitis. So if you have a baby that's
coming back to the clinic over and over with
recurrent pneumonia, somebody needs to put a
stethoscope on their chest and listen to their
heart. And what are we going to hear? With a
VSD, you're going to hear a loud, harsh, often
holosostolic murmur best heard at the left sternal
border. It's harsh because of that huge pressure
gradient we talked about. High pressure forcing
blood through a small hole makes a lot of noise.
OK, that makes sense. Now let's talk about the
patent ductus arteriosus, PDA. This is that fetal
tunnel that just never got the memo to close.
Right. Remember, in the fetus, this shunts blood
away from the lungs, from the pulmonary artery
to the aorta. But after birth, the pressures
in the body flip. The aorta is now the high pressure
hose. The pulmonary artery is low pressure. So
the blood flow. reverses direction through the
duct. Exactly. Now, blood shunts backwards from
the high -pressure aorta into the low -pressure
pulmonary artery. So once again, you're flooding
the lungs with extra blood. And this has very
specific murmur, right? This is a key word I
see in every single review book. It is. It's
the classic exam descriptor, a continuous machinery
-like murmur. It sounds like a washing machine
or a humming engine because blood is flowing
through it during both systole and diastole.
And another key assessment finding is wide pulse
pressure. Explain that. Why does the pulse pressure
widen with the PDA? Okay, so pulse pressure is
simply the difference between your systolic pressure
and your diastolic pressure. In a PDA, blood
is constantly leaking out of the aorta during
diastole, the heart's resting phase, and flowing
back into the pulmonary artery. So the pressure
in the aorta drops while the heart is resting?
It drops significantly. This causes the diastolic
blood pressure to be very low. So you might see
a blood pressure of, say, 100 over 40. That gap
between 140 is huge. wide pulse pressure. Got
it. Okay, now we need to integrate the pharmacology.
We mentioned endomethacin at the very beginning.
This is where it lives. This is its home. This
is the medical closure strategy. If we have a
premature infant with a symptomatic PDA, their
lungs are wet, they're in heart failure, they
can't get off the ventilator, we don't want to
do surgery if we can avoid it. So we give IV
endomethacin or another NSAA like ibuprofenylacine.
And these are NSAIDs. How do they work to close
the duct? So prostaglandins are the chemicals
that naturally keep the duct open in the fetus.
NSI's work by inhibiting prostaglandin synthesis.
You cut off the supply of prostaglandins and
the muscle in the ductus arteriosus constricts
and closes. But we have to be really, really
careful with endomethacin. I was looking at the
drug monograph. This is not a benign drug for
a tiny baby. No, it has huge risks. It works
by constricting blood vessels. That's great for
the duck, but it doesn't just work on the duck.
It can potentially constrict other vital vascular
beds, specifically the kidneys and the gut. So
the need -to -know safety checks are absolutely
vital here. Absolutely. Before and during the
administration of endomethacin, you are watching
two organ systems like a hawk. Number one, the
kidneys, is the patient peeing. Because endomethacin
can cause renal vasoconstriction, leading to
oliguria, low urine output, or even acute renal
failure. You're tracking urine output hourly.
hourly, and checking the creatinine before you
even start. And number two, the gut. You're watching
for any sign of GI bleeding, abdominal distension,
or bloody stools. Because if you restrict blood
flow to a preemie's already fragile gut, you
can cause a devastating condition called necrotizing
enterocolitis. NEC. That is a critical safety
point. So it's contraindicated in a patient with
an active GI bleed, suspected NEC, or significant
renal impairment. You have to check the labs,
the creatinine, the platelets before you ever
hang that bag. 100%. It's a non -negotiable safety
check. OK. That's a perfect cross -reference.
Now let's move to the, well, the scarier side
of the spectrum. The blue babies. decreased pulmonary
blood flow. Yeah, these are your cyanotic defects.
The core problem here is that the plumbing is
blocked on the right side of the heart. The blood
literally cannot get to the lungs, so it's forced
to find another way out. And that other way is
to cross over to the left side, unoxygenated,
and get pumped out to the body. Exactly. The
absolute star of the show here is Tetralogy of
Fallot, or TOF. It feels like every test has
at least one Tetralogy question. It's the exam,
darling. It really is. It's the classic cyanotic
defect. And it seems complex, but it actually
follows a pretty clear logic. It's four distinct
defects that occur together in one package. You
have to know the four components. OK, break them
down for us. The key problem, the one that drives
everything else, is number one. Pulmonic stenosis.
The pulmonic valve, the door leading out of the
right ventricle to the lungs, is narrowed and
stiff. It's an obstruction. A kink in the hose.
A kink in the hose. Because the right ventricle
has to work so hard to push blood past the stenosis,
you get defect number two. right ventricular
hypertrophy, the muscle gets thick and beefy
from constantly working against high resistance.
Like a bodybuilder. Exactly. Then you have defect
number three, a VSD, a hole between the ventricles.
And finally, defect number four is the overriding
aorta. The aorta is kind of shifted over and
sits right on top of the VSD, so it's getting
blood from both the right and left ventricles.
So it's grabbing both blue blood from the right
and pink blood from the left. Correct. And the
severity of the symptoms, how blue the baby is,
depends almost entirely on that first one, the
degree of pulmonic stenosis. That makes sense.
If the stenosis is really tight, very little
blood can get to the lungs and the baby is profoundly
cyanotic. If it's mild, they might just look
a little dusky when they cry. They're called
pink tets. But the classic clinical feature you
absolutely need to know is the TET spell, or
hypercyanotic spell. Walk us through a TET spell.
What happens? What triggers it? It typically
happens during times when oxygen demand suddenly
exceeds supply. So think about it crying, feeding,
a painful procedure like an IV start, or even
having a bowel movement. Anything that stresses
the baby out. Any stressor. The infant gets upset,
adrenaline surges, and this causes the smooth
muscle in the right ventricular outflow tract.
The area right below that already tight pulmonic
valve to spasm, it just clamps down hard. So
the door to the lungs, which was already barely
open, basically slams shut. Effectively, yes.
So now almost no blood can get to the lungs.
All that blue blood in the right ventricle has
nowhere to go but right to left. through the
VSD, and out the overriding aorta to the body.
And the baby turns. Deeply blue. The oxygen saturation
plummets to the 40s or 50s. They become limp.
And they can even have a seizure or lose consciousness.
It is terrifying to witness. And the nursing
action. This is on every single exam. The very
first thing you do is what? You put the infant
in the knee -to -chest position. Or, if they're
an older child, they will often do this instinctively.
They'll squat down on the playground. Why does
that work? It seems so simple, but the physiology
behind it is actually fascinating. It's pure,
beautiful physics. By squishing their legs up
against their chest or by squatting, you kink
the femoral arteries in the legs. This dramatically
increases the systemic vascular resistance, SVR.
So you're increasing the pressure on the left
side of the heart, in the aorta and the left
ventricle. Exactly. You are making it harder
for the left ventricle to pump blood out to the
body. This creates back pressure. That back pressure
pushes back against the VSD. So it makes it harder
for the blue blood to shunt from right to left.
Yes. It reduces the right to left shunt and essentially
forces more blood to take the only other path
available through that tight, spasming pulmonary
artery to the lungs. It's a mechanical manipulation
of pressure. It's absolutely brilliant. It buys
you time. It buys you critical time. But then
pharmacologically, we treat those spells with
morphine and, from our medication list, propranolol.
Ah. Another cross -reference to our med stack.
Why propranolol? It's a beta blocker. It is.
It's a non -selective beta blocker. In this specific
case, it has a magical effect of relaxing the
smooth muscle spasm in that right ventricular
outflow tract, the area called the infundibulum.
It literally helps open the door to the lungs
a little wider. And the morphine. Morphine is
a central nervous system depressant. It calms
the child, which is crucial because crying and
agitation increase pulmonary vascular resistance.
and make the spell worse. It sedates them and
reduces the respiratory drive slightly, which
helps to break that vicious cycle of sympathetic
surge and hypoxia. So need a chest to increase
systemic resistance, propranolol to decrease
the pulmonary resistance spasm, and morphine
to calm the system down. Got it. What about tricuspid
atresia? That's another blue baby one. Tricuspid
atresia is basically a do not enter sign on the
heart. The tricuspid valve, the door between
the right atrium and the right ventricle simply
fails to develop. It's a solid sheet of tissue.
So blood can't get to the lungs the normal way
at all? Not at all. There is no path from the
right atrium to the right ventricle. So how do
these babies survive even for a minute? They
absolutely require two things. One, they need
a hole in ASD or a VSD to allow blood to mix
and get over to the left side. And two, crucially,
they require the ductus arteriosus to stay open.
That's the only way blood can get to the lungs
through the back door from the aorta. This is
the definition of a ductal -dependent lesion.
So if that duct closes? The pathway to the lungs
is gone and the baby will die. Which brings us
directly to the opener on our medication list,
oprostidil. Alprostidyl, also known by its brand
name, ProstenVR, is Prostaglandin E1. This drug
is a lifeline. Its sole mission in this context
is to maintain the patency of the ductus arteriosus.
It keeps that tunnel open until a surgeon can
create a more permanent solution. But looking
at the monograph for alprostidyl, this is a very
high -stakes medication. The side effect profile
is scary. It is. The number one absolute need
-to -know clinical pearl for alprostidyl is apnea.
They just stop breathing. Yes. It happens in
about 10 to 12 percent of neonates, and it usually
happens in the first hour of starting the infusion.
It has a direct suppressive effect on the respiratory
center in the brain stem. So you don't just hang
the bag, set the pump, and walk away. You never
ever walk away. You hang the bag and you stand
there with the intubation kit and ambu bag ready
to go. You must have ventilatory assistance immediately
available. You are essentially anticipating a
potential respiratory arrest. That's a huge safety
point. The monograph also mentions flushing as
a side effect. Yes, cutaneous flushing the baby
turning red is a very common side effect due
to its vasodilatory properties. But here's a
really important safety nuance from the source.
If you are running the infusion through an umbilical
artery catheter, a UAC, and you see intense flushing
just on the baby's face or upper torso, you have
to be careful. Why? Because it might mean the
catheter tip is in the wrong spot. It might be
sitting too high near the vessels that go to
the head and arms and shooting a concentrated
dose of this potent vasodilator straight into
the cerebral circulation. You have to be aware
of that. Wow. Okay. That is a fantastic clinical
pearl. Let's move to the next category in the
outline, the obstructive disorders. The kink
in the garden hose group. Right. So the classic
one here is coactation of the aorta. The aorta,
the main artery leaving the heart, is pinched
or narrowed. It's like someone stepped on the
garden hose. This usually happens right after
the arch, just past the point where the blood
vessels branch off to the head and arms. OK.
So if I'm visualizing the plumbing, the pinch
is after the vessels that supply the head and
arms, but before the vessels that go down to
the legs and lower body. Exactly. So you have
a massive traffic jam upstream from the pinch,
an empty low -flow highway downstream. Which
means you'd have bounding pulses and high blood
pressure in the arms. Yes. High pressure to the
brain, which can risk a stroke, and high BP in
the arms. And then if you check the legs, you'll
find weak or even absent... femoral pulses, cool
skin, and low blood pressure. Before extremity
blood pressure check. Exactly. This is the hallmark
diagnostic sign. If you see a nurse taking a
blood pressure on both an arm and a leg in a
newborn, they are screening for coarctation.
It's a standard screen in many nurseries now.
If the upper extremity BP is significantly higher,
like 15 to 20 points higher than the lower extremity
BP, that's a huge red flag. That makes perfect
physiological sense. Now what about the mixed
defects? The source material calls these incompatible
with life unless we intervene immediately. That
sounds intense. They are chaotic and complex.
The big one everyone needs to know is transposition
of the great arteries, TGA. It's a fundamental
wiring error. The pulmonary artery and the aorta
are swapped. They're connected to the wrong ventricles.
Completely wrong. The right ventricle, which
is supposed to pump blue blood to the lungs,
is instead connected to the aorta. So it pumps
blue deoxygenated blood back out to the bottle.
And the left ventricle. The left ventricle, which
has the nice pink oxygenated blood returning
from the lungs, is connected to the pulmonary
artery. So it pumps that pink blood right back
to the lungs again. It's two completely separate
parallel loops that never meet. The body gets
zero oxygenated blood. Unless you have a connection.
You absolutely need a VSD, an ASD, or a patent
ductus arteriosus to allow the blue blood and
the pink blood to mix somewhere. This is another
medical emergency where alprostidyl is the essential
bridge to surgery. You have to keep that PDA
open to allow for mixing. And the other big one
mentioned is hypoplastic left heart syndrome,
HLHS. This is one of the most severe and complex
defects. The entire left side of the heart, the
left ventricle, the mitral valve, the aortic
valve, the ascending aorta is severely underdeveloped.
It's tiny, sometimes nonexistent. It simply cannot
support systemic circulation. So the treatment
can't be a simple patch repair. No, these babies
are critically ill from birth. They require a
series of three very complex staged surgical
procedures, the Norwood, the Glenn, and the Fontan,
that happen over the first few years of life
to completely replumb the heart. or they need
a heart transplant. It's a very long and difficult
road. Okay, let's pivot now to the acquired heart
diseases. So again, these aren't structural defects
from birth, but inflammatory attacks on a previously
normal heart. Kawasaki disease is the big one
here. Kawasaki disease is an acute systemic vasculitis.
That's a fancy way of saying it's inflammation
of the blood vessels, the arteries, the veins,
the capillaries throughout the entire body. And
for reasons we don't fully understand, it has
a particular affinity for the coronary arteries.
And it's the number one cause of acquired heart
disease in kids in developed countries. It is.
That's a huge deal. So what's the need to know
for the exam? How do we spot this? The presentation
seems very specific. It is. The diagnostic criteria
are very clear. First, you need a high fever,
usually over 102 degrees Fahrenheit or 39 Celsius.
That lasts for more than five days and does not
respond to antibiotics or antipyretics. That's
the key trigger. Then you look for the associated
symptoms, and the mnemonic is Cree rash. Okay,
break down Cree rash for us. C is for conjunctivitis.
It's a non -exudative conjunctivitis, meaning
red eyes without any pus or drainage, just dry,
red, irritated eyes. OK. R is for rash. It's
usually the diffuse maculopapular rash, often
on the trunk. A is for adenopathy, which means
swollen lymph nodes, typically a single large
one in the neck or cervical area. And S? S is
for strawberry tongue. The tongue gets very red,
swollen, and bumpy, like a strawberry. You can
also see very red, cracked, peeling lips. And
finally, H is for hands and feet. You see swelling
and redness of the palms and soles, and then
later, in the subacute phase, you get this characteristic
peeling of the skin on the fingertips and toes.
Strawberry tongue is such a classic key word.
Now, the treatment for Kawasaki is really interesting
because it breaks one of the cardinal rules of
pediatrics. It absolutely does. We give aspirin.
Ooh. We are taught from day one, you never give
aspirin to kids. because of the risk of Ray's
syndrome, which can cause devastating liver and
brain damage, especially after a viral illness.
Exactly. But Kawasaki disease is the one major
exception to that rule. The benefit of its potent
anti -inflammatory and anti -platelet effects
far outweighs the risk of Ray's. We give very
high doses of aspirin initially to cool down
the inflammation and then a low dose for a long
time after. And we also give IV, right? Intravenous
immunoglobulin. Yes. High dose ID is the other
cornerstone of treatment. We're essentially flooding
the body with these pooled antibodies to dampen
the patient's own overactive immune response.
And the reason we do all this so aggressively
is to prevent the crash risk. The coronary artery
aneurysms. That's the ballgame. If you miss the
diagnosis or you treat it too late after day
10 of the fever, the child has a much higher
risk of developing these dangerous aneurysms
or weak bulging spots in their coronary arteries.
Which can lead to blood clot. a heart attack,
or long -term ischemic heart disease. It's wild
to think about. A heart attack in a toddler?
It happens. And that's why the long -term monitoring
with echocardiograms is so crucial for these
kids, sometimes for the rest of their lives.
OK. Then we have rheumatic fever. This is the
one that's connected to strep throat, right?
Yes. This is an autoimmune reaction that is a
delayed sequela of a group of a beta -hemolytic
streptococcus pharyngeal infection. It usually
happens about two to four weeks after an unfreeded
or partially treated case of strep. So this is
the kid who had a sore throat, maybe the parent
gave them antibiotics for two days, they felt
better, so they stopped the course early. That
is the classic scenario. The antibodies the body
made to fight the strep bacteria get confused
and they start to cross -react and attack the
body's own tissues, specifically the joints causing
a migratory polyarthritis, the brain causing
Sydenham's caria, which are these jerky involuntary
movements, the skin causing a rash called erythema
marginatum, and most importantly, the heart valves
causing carditis. So the prevention is simply
treating strep throat properly. Exactly. Prevention
is everything here. Finish your full course of
antibiotics. Penicillin is still the drug of
choice. And we diagnose it using something called
the modified Jones criteria. And briefly. Infective
endocarditis. This is a direct bacterial or fungal
infection of the inner lining of the heart, the
endocardium, or the heart valves. You have to
think about kids with pre -existing congenital
heart defects or prosthetic valves. They are
at very high risk because their blood flow is
often turbulent. And that turbulent flow can
damage the lining and create a rough spot. Exactly,
a rough spot where bacteria can stick and grow,
forming what's called a vegetation. But you also
have to think about the entry points for the
bacteria. Like dental work. Dental work is a
major one. Bacteria from the mouth can get into
the bloodstream during a cleaning or a procedure.
That's why we give prophylactic antibiotics before
the dentist to certain high -risk patients, like
those with artificial valves or a previous history
of endocarditis. And the exam keywords for endocarditis.
The ones that always show up are ocelar nodes,
which are painful red, tender nodules on the
pads of the fingers and toes, and Janeway lesions,
which are painless, hemorrhagic, flat spots on
the palms of the hands and soles of the feet.
Okay, we've covered the defects in the inflammation,
but as you said, all roads can lead to heart
failure. How does heart failure look in a baby
versus an adult? It's so different. It is. In
an adult, you're looking for jugular vein distension,
JVD, or listening for crackles in the lungs,
but you can't really see JVD in a chubby baby
neck. In a baby, the signs are much subtler but
very distinct. The very earliest sign is often
unexplained tachycardia. The heart is just trying
to beat faster to keep up. Exactly, to compensate
for poor function. Then you'll see, to Chipney,
fast breathing. But the hallmark, the thing the
parents will almost always tell you about, is
difficulty with feeding. The sweating with feed.
That's the one. Scalp sweating. You have to think
about it. Eating is the most aerobic exercise
a newborn does. It's their marathon. If they
can't finish a bottle, if they get sweaty and
exhausted and start breathing fast after just
an ounce or two, that is activity intolerance.
That is a sign of heart failure. Which leads
us perfectly into part C of our deep dive. development
and milestones. How does all of this cardiac
stress affect the growing child as a person?
It has a massive impact. A baby is a metabolic
engine that is designed for one thing to grow.
If you are burning all of your available calories
just trying to breathe and circulate blood because
of a hole in your heart, you have no energy left
over to put on weight. This is failure to thrive.
So clinically, we're going to see them falling
off the growth chart. Right. They'll drop from
the 50th percentile for weight down to the fifth
percentile. And the nursing intervention isn't
just give medications. A huge part of it is nutritional
management. We have to fortify the formula or
breast milk to make it more calorie dense. Exactly.
We'll increase it to 24 or 27 calories per ounce
instead of the standard 20. We have to pack more
punch into every single drop they can take in.
And what about energy conservation? That's the
other side of the coin. It's so important. We
need to cluster our care. You don't want to be
going in and waking the baby up every 15 minutes
for vital signs if they desperately need to conserve
energy. You do everything at once. Change the
diaper. Check the vitals. Give the medicine.
And then feed them. And then you let them rest.
What about the psychosocial impact on the family
and the child? The anxiety is real and profound.
Imagine being a parent watching your child turn
blue during a TED spell. It's traumatic. And
for the child, there can be developmental delays.
They might hit their motor milestones late because
they're simply too tired to do tummy time or
learn to crawl or walk. So it's our job to monitor
for that. Absolutely. We have to monitor for
those developmental red flags, but also support
the family's coping mechanisms. We have to involve
the child in their own care as they get older,
and it's developmentally appropriate. Okay, let's
do a really deep dive now into the medication
module. We've touched on a few of these already,
but I want to get granular with the safety data
in these monographs because the exam questions
so often hinge on the side effects or the specific
nursing actions. Let's do it. So we've covered
alprosodil, the opener, and endomethacin, the
closer. Let's talk about furosemide lasix. The
classic diuretic. Fluid overload management and
heart failure. Right. It's a loop diuretic. It
works by inhibiting sodium and chloride reabsorption
in the loop of hemel in the kidneys. Bottom line,
it makes you pee out a ton of fluid. But for
the exam and for the bedside, you must remember
the push rule. You have to push it slow. Very
slow. The monograph explicitly states it. 0 .5
milligrams per kilogram per minute. Not just
slow, it's a specific rate. If you slam Lasix
the Valk push or you give high doses too rapidly,
you risk ototoxicity. Permanent hearing loss.
Yeah. From a diuretic. Yes, it can be transient
or it can be permanent. Can you imagine curing
a child's heart failure but leaving them deaf
because you were in a hurry with the IV push?
That is a can't miss safety item, a huge one.
And obviously... Lasix is famous for dumping
potassium. Oh, yeah. So you are constantly monitoring
for hypokalemia, watching for muscle weakness,
cramping, and most dangerously, cardiac arrhythmias.
And if that kid is also on digoxin, hypokalemia
dramatically increases the risk of digoxin toxicity.
It's a very dangerous cycle. OK, now let's contrast
that with Lisinopril. Lisinopril is an ACE inhibitor.
It works by stopping the conversion of angiotensin
I, angiotensin II. Angiotensin II is a super
potent vasoconstrictor. So by blocking it, the
blood vessels dilate. We use it for afterload
reduction. It makes it easier for the weak heart
to pump against the body's resistance. But unlike
Lasix, which dumps potassium, Lisinopril holds
onto it. Right. ACE inhibitors are potassium
sparing. So the electrolyte imbalance you're
watching for here is hyperkalemia. You have to
be really careful about giving potassium supplements
to a child who's on Lisinopril. And here's a
massive black box warning I'm seeing in the monograph
for Lisinopril. fetal toxicity. This is huge.
If you have an adolescent female patient with
a cardiac condition who is on Lisinopril, you
must be doing pregnancy screening and education.
It's known to cause oligohydramnios, low amniotic
fluid, fetal skull defects, and even fetal death.
That is a critical patient education point for
that specific demographic. I also saw a note
in the source about compounding Lisinopril. Yes,
this is a practical point. Lisinopril often only
comes in tablets. You can't ask a six -month
-old to swallow a pill, so the pharmacy will
often have to make a liquid suspension. The source
details an extemporaneous suspension crushing
the tablets in simple syrup. The key for the
nurse is to know that it needs to be shaken well
before every dose and stored properly. Good to
know. Okay, let's talk heparin. This one terrifies
me because of the potential for concentration
errors. It should. The source material highlights
a long and tragic history of fatal errors with
heparin in pediatrics. It comes in a catheter
lock flush concentration, which is usually 10
units per ml or 100 units per ml. That's just
a tiny dose to keep an IV line from clotting.
And then there is the hypotency injection, which
can be a 1000, 5000, or even 10 ,000 units per
ml. This is used for systemic anticoagulation
for treating actual blood clots. And the vials
can look very similar. They often do. Flushing
those two concentrations has killed neonates.
You think you're flushing a central line with
10 units, but you accidentally give 10 ,000 units
a thousand times the dose and cause a massive
intracranial hemorrhage. Always, always, always
double check the vial, read the label three times,
have another nurse check it with you. And for
monitoring a heparin drip? We monitor the APT,
or activated partial thromboplastin time. That's
the lab value we use to titrate the drip to a
therapeutic level. And another key safety point,
no IM injections for any child on Heparin. You
will cause a massive painful hematoma. Got it.
We mentioned propranolol for tet spells, but
the source says it's also used for infantile
hemangiomas. That seems random. It does, but
it's very common now. A liquid formulation called
hemangel is a first -line treatment for shrinking
those benign vascular birthmarks. But the key
safety pearl you need to know here is the risk
of hypoglycemia. Really? A heart medication causing
low blood sugar. Yes. Beta blockers can mask
the early warning signs of hypoglycemia, like
jitteriness or tachycardia, because they block
the body's adrenaline response. They can also
inhibit glycogenolysis, which is the body's ability
to break down stored sugar from the liver. So
if a baby isn't eating well and you give them
propranolol? Their blood sugar can drop to dangerously
low levels. So the hard and fast rule is you
administer propranolol during or immediately
after a feeding. You never give it if the child
is vomiting or not taking their bottles well.
That is a fantastic clinical pearl. What about
hydrolazine? That's a direct acting vasodilator.
It works right on the arteriolar smooth muscle
to make it relax. The big side effect you have
to watch for is reflex tachycardia. The body's
fighting back. Exactly. The blood pressure drops
because the vessels suddenly open up, so the
heart speeds up to compensate and try to maintain
cardiac output. It can defeat the purpose of
the drug if you don't anticipate and manage it.
And a weird administration quirk. The source
notes that it can change color if it comes into
contact with metal. That's a strange one, but
good to know for drawing it up. You might need
to use a filter needle or just be careful to
avoid metal contact. And it also mentions a rare
but serious lupus -like syndrome as a potential
reaction with long -term use. Okay, let's move
into our final section, Part E Synthesis. Let's
connect all these dots. Mention the push -pull
of potassium. Yes, this is a classic exam scenario.
You have a child with heart failure who is on
a regimen of digoxin, furosemide, lasix, and
lisinopril. Furosemide makes you lose potassium,
lisinopril makes you retain potassium, and digoxin
toxicity is massively potentiated by low potassium.
So the nurse is a tightrope walker. You are the
tightrope walker. have to be monitoring the potassium
levels constantly because tipping too far in
either direction has a dangerous impact on the
other drugs. If the K plus level gets too low
from the Lasix, the Dagoxin can become toxic
even at a normal dose. If the K plus level gets
too high from the Lisinopril, you're at risk
for fatal arrhythmias. Balance is absolutely
everything. Exactly. You have to understand how
all three drugs interact. And let's synthesize
the opener versus closer one last time to really
drive it home. Let's do it. You have a profoundly
cyanotic newborn. You get an echo, and it shows
TGA or tricuspid atresia. The plumbing is either
crossed or completely blocked. You need mixing.
You need that ductus arteriosus open. So you
give alprostadil. You give alprostadil? Yeah.
And you watch for apnea. You have a premature
newborn who is struggling on the ventilator.
They have wet, crackly lungs, a machinery -like
murmur and bounding pulses. The duct is stuck
open and it's flooding the lungs. You need it
closed. You give endomethacin. You give endomethacin
and you watch the urine output and look for any
signs of gut bleeding. That is the 80 -20 right
there. If you understand that one concept, you
can answer a dozen different questions correctly
and safely. Absolutely. And let's just revisit
the heart failure regimen, the classic triad
of medications. Right. It's often digoxin plus
a diuretic, like Lasix, plus an ACE inhibitor,
like Lisinopril. Think of it in terms of what
each one does for the tired pump. One. Digoxin
increases contractility. It makes the heart pump
harder and more efficiently. We're improving
the squeeze. The anotropy? Exactly. Two, Lasix
removes excess fluid. It reduces preload. We're
making the heart pump less volume, which reduces
its workload. Three, the ACE inhibitor dilates
the blood vessels. It reduces afterload. We're
making it easier for the heart to pump against
the resistance of the body. Harder squeeze, less
volume, less resistance. It's all about improving
efficiency. It's pure mechanics. The heart is
a pump. We are just manipulating the variables
to make the job easier for a tired, overworked
muscle. As we start to wrap up, I want to look
at the outro thought in our outline. We've mapped
the plumbing, the inflammation, the consequences,
and the chemical toolkit. But what about the
long term? And that's the final provocative thought
we want to leave you with. We call these surgical
procedures repairs, not cures. That's a powerful
distinction. It is. A child who has a tetralogy
of phallate repair at six months old. What happens
when they're 30? They don't just have a normal
heart. They have scar tissue in their heart from
the surgery. They might have a leaky pulmonary
valve that will eventually need to be replaced.
They are at a lifelong risk for arrhythmias because
the electrical conduction tracks may have been
cut during the initial surgery. And the Kawasaki
kid. That kid who had Kawasaki at age three.
They might have permanent coronary artery scarring,
even if they didn't develop full -blown aneurysms.
They need to monitor their cholesterol and their
heart health much more aggressively as adults.
because they have a pre -existing condition baked
into their blood vessels. So pediatric cardiac
nursing isn't just about saving the baby today.
It's about setting the entire trajectory for
the adult they will one day become. That's it,
exactly. You are preserving precious heart muscle
for a lifetime of use. Every prompt intervention
you make, every correct medication dose you give,
every time you catch a subtle sign of heart failure,
you are preserving function not just for tomorrow,
but for decades to come. That is a really powerful
place to leave it. You've officially taken the
deep dive into pediatric cardiac nursing. Now
go save some tiny hearts.