Chapter 41: Cardiovascular Disorders in Children Nursing Care

Welcome to Last Minute Lecture.

This free chapter overview is designed to help students review and understand key concepts.

These summaries supplement not replaced the original textbook and may not be redistributed or resold.

For complete coverage, always consult the official text.

Welcome back to the Deep Dive.

Our mission here is to take these massive stacks of clinical data and, you know, turn them into something you can use right now.

And today we are really into the deep end.

We're wading into pediatric cardiovascular disorders.

Which is, I mean, it's just so dense and emotionally challenging for everyone involved.

It really is.

I think this area of nursing, maternal and child health cardiology is maybe one of the most demanding.

The physiology is just so complex.

The patients are tiny and the stress on the families is,

well, it's immense.

Exactly.

So to ground us, let's start with that classic scenario, the one that's just incredibly high stress.

You've got a new family.

They're about to go home.

Their baby was just diagnosed with a moderate ventricular septal defect, a VSD.

The most common heart defect.

Right.

And the cardiologist tells them they just need to watch the baby carefully.

So the parents, terrified, they turn to you, the nurse, and they ask,

what does that even mean?

What are we supposed to watch for?

And that question right there, that is the entire focus of this deep dive.

It's about giving you the knowledge from the fetal shunts all the way to the specific meds to not just answer that question, but to manage the whole spectrum of care.

And the scope of this field now is just fascinating.

We're not just talking about the congenital defects they're born with, like that VSD.

No, not at all.

We're also talking about acquired heart diseases that pop up in childhood.

And there's a growing emphasis on screening for inherited disorders that might not show up for years.

That's where the progress has been huge, right?

The genetic testing piece.

Oh, absolutely.

Yeah.

Think about genetic tests for conditions like Marfan syndrome or long QT syndrome or all the different cardiomyopathies.

We can diagnose these things so much earlier now.

Which means you can intervene earlier with lifestyle changes, with medications.

Or even implantable divinuses.

Yeah.

It's a whole different world of proactive care.

And the data shows it.

I mean, the outcomes are just, they're staggering.

It's something like 85 % of children born with CHD.

Even the most complex anatomical defects, yeah.

Yeah.

Are now surviving well into adulthood.

And that one statistic, that changes everything.

If these kids are surviving, the nursing focus can't just be reactive anymore.

It can't just be about the immediate crisis.

It has to shift.

It has to pivot toward chronic care, long -term wellness, and prevention.

Okay.

Let's unpack that shift.

Let's connect it to the big picture, to the national health priorities.

The healthy people,

2030 goals.

They really back this up, don't they?

They absolutely do.

It's this clear movement away from just focusing on adult heart disease prevention and toward really aggressive interventions starting in early childhood.

It's about building a better foundation from the start.

And when you look at those goals, they get really specific.

It's not just eat your vegetables.

No, it's not.

They're getting to the nitty gritty.

For instance, with food intake, the baseline was about half a cup equivalent per thousand calories.

The target is to get that up to almost a full cup equivalent.

That level of detail shows you they know that empty calories are a huge problem, that they're accelerating adult risk factors in kids.

And that leads you right into the goals for reducing childhood obesity.

It's comprehensive.

They're targeting kids from age two all the way up to 19.

So if you're a nurse in primary care, you are now on the charts.

And then there's the environmental piece reducing smoke exposure, which is huge.

The goals are aiming for 100 % of schools to be smoke free.

And they're pushing for laws to ban smoking in all public places, daycares, even home based daycares.

Yep.

Across all 50 states in DC.

It's a direct intervention.

You reduce that second and third hand smoke and you are improving cardiovascular health right away.

And finally, that very specific goal of reducing infant deaths related to birth defects, specifically CHD.

Right.

It highlights that even though we can't prevent most congenital heart disease, we can save lives with early diagnosis and really aggressive management right after birth.

So the takeaway for you, the listener, is that your role as a nurse here is just massive.

It's not just managing the defect in front of you.

Not at all.

It's being a core educator.

It's driving family wide changes in exercise, nutrition, and you know, your whole environment.

So if the goal is wellness, let's start at the beginning.

Let's start with assessment.

And how do you even begin to look for these really subtle signs of cardiac problems in a family history?

Well, the initial assessment, it has to be incredibly comprehensive precisely because the signs in children can be so, so subtle.

It has to be a multi -system assessment because heart failure isn't just about the heart.

It hits the lungs, the gut, the kidneys.

Exactly.

And that history gathering is probably the most critical first step.

And we're not just talking about the patient's history.

We're talking about the whole family tree, aren't we?

Absolutely.

You start with the immediate birth history, any maternal infections, drug exposure, any issues in that newborn period.

Then you dig deep.

Any parents or siblings diagnosed with cardiac disease before they were 55.

Any history of sudden unexplained death.

Right.

Or any known genetic disorders.

Things like Down syndrome or Turner syndrome.

Those are the classics.

Yes.

And recognizing those links is vital.

A condition like DeGeorge syndrome, the 22q11 deletion, has a very high rate of really complex cardiac anomalies.

So if a child has a known genetic disorder, your forethought should be a full cardiac workup.

Now here's where the emotional side of this gets really intense.

The nurse's role is so much more than a physical exam.

Oh, it is.

A cardiac diagnosis is terrifying for parents.

They hear heart and they just panic.

They do.

And even young kids, they often get that the heart is important.

For the parents, their trust in their child's own body is just, it's shaken.

So the nurse has to be a teacher, explaining the anatomy in a way they can understand and really providing that emotional support.

And that role continues into the diagnostic procedures.

I mean, how do you help a family cope with an echo or even more a cardiac catheterization?

You demystify it.

You take the fear out of the unknown.

You explain that the echo is just taking pictures with sound waves, that the gap is a tiny tube to measure pressure, that education starts with you.

So if we know that emotional burden is real, how do we formalize that?

How do we translate it into our nursing diagnoses?

That's where the psychosocial diagnoses are just as important as the physical ones.

Physically, you have the obvious ones.

Reduced cardiac output or altered peripheral tissue perfusion risk.

And the metabolic side of things.

Malnutrition risk.

Definitely.

I mean, the energy takes just to keep a compromised heart beating and to breathe that fast.

The metabolic demands are through the roof.

If they're also getting tired during feedings, they're in a calorie deficit.

You have to document that.

But the psychosocial diagnoses, those seem to have the longest lasting impact, don't they?

They absolutely do.

We list things like knowledge deficiency or anxiety.

But the ones that signal real long -term risk are altered family processes and parenting impairment.

That's the inability to bond.

Because the parents feel like the child is just too fragile.

Exactly.

The sheer stress of the illness can make them afraid to even touch their own baby.

And if you miss those issues at the beginning, they don't just go away after a successful surgery.

They can lead to chronic family problems.

So moving into planning and implementation, it sounds like the priority is just education, education, education.

It's all about empowering the family.

By the time they go home, the parents need to be the experts on their child's specific heart anatomy.

And that's not just academic.

It's the foundation for understanding why they need to give the medications, why they need to go to follow -up appointments.

Yes.

And you also have to know the resources.

You have to be able to refer them to organizations like the American Heart Association, Conquering CHD, or Mended Little Hearts.

That peer support is something we can't replicate.

And part of implementation is also risk management for the things that can be prevented.

Right.

While you're managing the congenital defect, you're simultaneously trying to prevent the acquired ones, rheumatic fever, hypertension, and hyperlipidemia.

For rheumatic fever, the key is probably the simplest intervention in all of pediatrics.

Just treat strep throat adequately with antibiotics.

The autoimmune reaction that damages the heart valves is triggered by untreated strep.

So you have to educate parents to finish the whole course of antibiotics, even if their kid feels better after two days.

Okay.

Hypertension, we know it's on the rise, tied to lifestyle.

And this is where those healthy people, 20, 30 goals come right into the clinic.

As a nurse, you have to be advocating against high salt, against processed foods.

And clinically, we have to remember the guideline.

Routine blood pressure checks start at age three for healthy kids.

Not in adolescents.

No, you can't wait that long.

Now hyperlipidemia, this is one of those details where pediatric nursing is completely different from adult care.

A critical difference.

You do not restrict fat in infants and toddlers, ever.

That fat is absolutely essential for nerve myelination, for brain development.

So any kind of dietary fat restriction only starts after age two.

And the screening guidelines for high cholesterol follow that same logic.

Right.

Any child who is overweight, hypertensive, or has that strong family history of early heart disease needs to be screened regularly.

And the whole family has to be on board.

You can't put a child on a diet if the rest of the family isn't participating.

It just doesn't work.

Okay.

So you can't really understand any of the defects without having a rock solid foundation in the physiology.

So let's do a quick map of normal blood flow in a child.

All right.

So deoxygenated blood comes back through the superior and inferior vena cava into the right atrium.

Goes through the tricuspid valve to the right ventricle.

Then it's pumped out the pulmonic valve into the pulmonary artery, heads to the lungs, gets oxygenated, and comes back through the four pulmonary veins into the left atrium.

From there, it's through the metral valve into the left ventricle and then boom out the aortic valve to the whole body.

And here's a detail that's so easy to forget but is clinically vital.

The coronary arteries, the ones that feed the heart muscle itself, only get perfused during

the relaxation and filling phase.

Exactly.

And that's why tachycardia, a really fast heart rate, is so dangerous for these patients.

It shortens that diastolic filling time, which means less blood flow to the heart muscle itself, which can quickly lead to ischemia.

Okay.

Now let's get into the really fascinating part, fetal circulation.

Why is it so completely different from how things work after birth?

Well, there are two big reasons.

First, the lungs and utero are filled with fluid.

They're collapsed.

So the resistance to blood flow there is incredibly high.

So there's high pulmonary pressure.

Extremely high.

And second, all the gas exchange, all the oxygen, is coming from the placenta.

So the fetus needs a really efficient way to bypass those non -working lungs, and it does that with three critical chunks.

Let's start with the first one, the one that handles that really oxygen -rich blood coming from the placenta.

That's the ductus funosus.

The umbilical vein carries that highly saturated blood, and a little more than half of it just bypasses the liver completely through the ductus funosus and streams right into the IVC.

So it's like an express lane for the best blood.

Exactly.

And that highly oxygenated blood is then guided

by this little ridge inside the right atrium.

And it gets directed straight to shunt number two.

The formyl ovale, which is just a little flap, an opening between the atria.

That better saturated blood gets shunted right across from the right atrium to the left atrium.

Bypassing the whole right side of the heart.

It bypasses the right ventricle and the lungs entirely, which is crucial because the left ventricle then pumps that best oxygenated blood right out the ascending aorta to the brain and the upper body.

So it prioritizes the most important developing structures.

Precisely.

And that leaves the third shunt, the ductus arteriosus, to deal with the less oxygenated blood.

Right, the deoxygenated blood coming back from the upper body and the little of blood that does go into the right ventricle.

Well, because that pulmonary pressure is so high, almost 90 % of it gets diverted away from the lungs.

Where the ductus arteriosus.

Which connects the pulmonary artery directly to the aorta downstream from the vessels that go to the brain.

So really the fetus has this parallel circulation.

Both ventricles are pumping blood to the body and the lungs are just sort of on standby.

Exactly.

It's a brilliant system that has to change dramatically in the first few minutes of life.

So what flips that switch?

What are the key adaptations at birth?

It all starts with that first breath.

When the baby inflates its lungs, the fluid gets pushed out and the vascular resistance in the lungs just plummets instantly.

So the pressure in the pulmonary artery and the right ventricle drops way down.

Correct.

And now blood rushes into the lungs, gets oxygenated, and a huge volume of it comes rushing back to the left atrium.

Which raises the pressure on the left side of the heart.

For the first time, the left atrial pressure is higher than the right atrial pressure and it just slams that little flap of the forminoval shut.

Very immediately.

Immediately.

And the ductus arteriosus, its closure is triggered by chemicals,

the big increase in oxygen in the blood now, and the removal of the placenta, which causes prostaglandin levels to drop.

It constricts and closes within a few days.

Here's a critical warning though about the timing of that pressure drop.

Right.

Even though the shunts close fast, the newborn's pulmonary pressures continue to drop gradually.

They don't reach their lowest adult -like levels until about four to six weeks of age.

And that four to six week mark is everything, isn't it?

It's crucial because a small VSD that was totally silent at birth might suddenly start shunting a lot of blood and causing a loud murmur or even signs of heart failure.

But only once that pulmonary pressure has finally dropped low enough.

That delayed presentation just reinforces why we need such careful ongoing assessment.

Let's start with this screening tool that has just revolutionized how we detect critical defects, pulse oximetry.

Yeah.

This is now universal in the U .S.

for critical congenital heart disease.

It's done at 24 hours of life and again before they go home.

Walk us through the exact procedure.

This is a really high -stakes screen.

It is.

You need two separate readings.

One on the infant's right hand.

That's pre -ductal blood flow.

Blood that hasn't gone past the ductus yet.

Right.

And the second reading is on any lower extremity.

That's post -ductal.

And to pass, two things have to be true.

Both readings have to be 95 % or higher and the difference between the two can't be more than 3%.

And what's the protocol if they fail or need a re -screen?

Okay.

So if the saturation is between 90 and 94 % or if the difference is more than 3%, you re -screen.

You can do that up to two times an hour apart.

But if at any point the saturation drops below 90 % in either site, that's an immediate fail.

Urgent evaluation, probably an echo right away.

Let's focus on the general assessment, the things beyond the numbers.

You said activity level is the most important sign.

So what does a tired infant look like?

In an infant, the main activity that shows you their cardiac function is feeding.

You have to watch how long it takes them, how much effort it requires.

Do they need to stop and rest a lot?

And are they getting sweaty?

Yes.

If you see to a hypnea, that fast breathing or

diaphoresis, sweating on their forehead during a feeding,

that's a huge red flag.

It means their heart just can't keep up with the metabolic demand of eating.

For older kids, it's exercise intolerance.

They can't keep up with their friends.

And of course, growth.

Yep.

We track height, weight, BMI.

But a key point for kids with CHT is that a lot of them have their own normal curve, which might be a little lower than average.

You're watching for deviations from their own established curve.

Persistent failure to thrive is a classic sign of worsening heart failure.

Moving to the physical exam, let's start with what you can see.

Where's the best place to look for cyanosis?

The mucous membranes, the lifts inside the mouth.

That's where you'll see true central cyanosis.

You have to distinguish that from acrocyanosis, that bluish color in the hands and feet, which is totally normal in the first day or two of life.

And capillary resale.

Simple, but critical.

Should be less than two to three seconds.

Any longer than that suggests poor peripheral perfusion.

Okay, palpation.

We're feeling for the point of maximum intensity, the PMI.

Right.

In kids, that's usually at the fourth or fifth intercostal space, mid clavicular line.

If you feel it further out or lower down, that suggests the heart might be enlarged.

And you're also palpating for abnormal findings.

Let's talk about a thrill, a lift, and a heave.

A thrill is a vibration.

You feel it with your hand flat on the chest.

It's often described as feeling like a purring cat.

It's caused by really, turbulent blood flow.

And a lift or a heave?

The lift is a forceful contraction that just kind of pushes your hand up a bit.

A heave is even more forceful.

It's a sustained movement that lifts your hand up and out, and it suggests significant ventricular hypertrophy.

For auscultation, you need a system.

What's the protocol?

Quiet room.

And you have to use both the diaphragm for high frequency sounds and the bell for low frequency ones.

Start with the child sitting up and you move systematically.

Aortic area, then pulmonic, down the left sternal border, over to the apex for the mitral area.

Any practical tips?

Don't forget to listen on their back.

Some murmurs, especially innocent ones like the pulmonary flow murmur, can radiate to the back and you'll miss them if you only listen to the front.

Let's break down the sounds.

S1 and S2.

S1 is the closure of the mitral and tricustid valves.

That's the start of systole when the ventricles are ejecting blood.

S2 is the closure of the aortic pulmonic valves.

That's the start of diastole when the ventricles relax and fill.

And the classic clinical finding of volume overload is a fixed split S2.

Why does that sound so important?

Well, normally S2 splits a little when you breathe in.

A fixed split S2 means it's split, but it doesn't change with breathing.

And that is a huge indicator of right heart volume overload, which is most often caused by an atrial septal defect, an ASD.

Because the constant shunting of blood from left to right overloads the RV.

Exactly.

It makes the RV empty slower, which delays the pulmonic valve closure.

And that's what fixes the split.

Okay.

What about the extra sounds?

S3 and S4.

S3 is the Kentucky sound.

It's from rapid ventricular filling.

It can be totally normal in a healthy kid, but it can also be a sign of volume overload or a failing heart.

S4, the penicy sound, is atrial contraction against a stiff ventricle.

If you hear an S4 in a child.

It's always bad news.

Always pathologic.

All right.

Let's talk about murmurs.

The documentation has to be so detailed.

It does.

You have to document the quality.

Is it musical?

Is it harsh?

The pitch, the intensity, location, radiation, and critically, if there's a thrill.

Which brings us to the Levine grading scale.

Instead of listing all six grades, what's the one grade a nurse has to know?

The objective one.

Grade the fourth.

Because a grade the fourth out of the six murmur is the first one that is required to have a palpable thrill.

If you can feel that vibration, you know the murmur is significant.

Let's reassure the parents listening about the innocent murmurs.

Yes.

The most common one is the still murmur, usually in kids three and up.

It's vibratory, musical, and it changes when they change position.

The other one is the pulmonary flow murmur, which is a bit harsher and should be gone by about six months.

You have to be clear with parents.

Innocent murmurs do not turn into something serious.

I have to state that directly.

They do not become serious heart disease.

And the absolute red flags for a pathologic murmur.

Any diastolic murmur is always pathologic.

Always.

And that continuous machine -like murmur under the left clavicle in an infant.

That's the classic sign of a patent ductus arteriosus, a PDA.

Finishing up with diagnostics, what are the key labs you're looking at?

Well, you're checking cardiac markers like CKMB and troponin for muscle injury.

Inflammatory markers like CRP and ESR are key for things like Kawasaki or rheumatic fever.

BMP is your marker for heart failure.

As ventricle stretch, it goes up.

And in kids with chronic cyanosis, you'll see something that looks weird on the CBC.

Polysathenia.

The body is trying to compensate for the low oxygen saturation by making more red blood cells to carry what little oxygen there is.

It's a normal compensatory response.

On the imaging side, the Echo is the non -invasive workhorse, but the gold standard is cardiac catheterization.

Right.

And cath is invasive.

It's diagnostic.

You can measure pressures and oxygen levels.

But it's also interventional.

We can close holes, open up stenotic valves, place stents.

You have to prepare the family for the procedure and the radiation exposure.

And for older kids with exertional symptoms.

That's where you do an exercise stress test.

The goal is to recreate their symptoms, the chest pain, the palpitations on the treadmill, to see if they're truly cardiac in origin.

Okay.

Let's talk about the single biggest challenge in pediatric cardiology.

Heart failure.

So at its core, it's the heart's inability to keep up with the body's metabolic demands.

Right.

And to understand the failure, you have to understand the components of cardiac output, which is heart rate, time straight.

Pugil load is the volume of blood filling the ventricles.

Contractility is the squeeze.

And afterload is the resistance the heart has to push against to get the blood out.

And here's a huge pediatric difference.

Infants can't really change their stroke volume much.

That's right.

For an infant, the main way to increase cardiac output is to increase their heart rate.

But if the heart rate gets too high, they don't have enough time to fill and their cardiac output actually crashes.

When a child's heart starts to fail, the body kicks into this desperate compensatory mode.

Let's break down the two phases of that.

The first immediate response is the sympathetic nervous system.

Adrenaline.

It increases heart rate, increases the squeeze, and clamps down on the peripheral blood vessels.

Shunting blood to the brain and the heart.

Away from the skin, the gut, the kidneys.

It's a short -term fix.

The slower, more damaging response is the activation of the renin angiotensin aldosterone system, or RAAS.

This is the kidneys sensing low perfusion.

Exactly.

They release renin, which leads to angiotensin the sacchar player, a powerful vasoconstrictor, and aldosterone, which makes the body hold on to salt and water.

So it increases the volume, the preload.

Right.

And this is why it ultimately backfires.

The heart is already failing, and now it's getting overloaded with extra fluid and it's trying to pump against these clamped down blood vessels.

It just gets overwhelmed.

Let's contrast the signs of right -sided versus left -sided failure.

Left -sided failure is a backup into the lungs.

So you're going to see pulmonary congestion, fast -breathing crackles, shortness of breath.

Right -sided failure is a backup into the body.

So you'll see an enlarged liver, hepatomegaly, and in older kids, JVD.

And in infants, the signs are so much more subtle.

They are.

The goal of standard signs are poor feeding, getting tired easily, and that sweating with effort.

You might see puffiness around the eyes, periorbital edema, before you see it anywhere else.

The goal of management then is to stabilize them until you can fix the underlying problem.

It's all about decreasing preload, decreasing afterload, and increasing contractility.

Let's start with the classic inotrope, digoxin.

Digoxin gives you a stronger squeeze, a positive inotropic effect, and it slows the heart rate down, a negative chronotropic effect.

It's particularly useful in infants with single ventricle physiology, like HLHS.

But it has a really narrow therapeutic window.

It does.

And it's cleared by the kidneys.

The nurse has to know to hold the dose if the child's heart rate is too slow for their age and call the provider.

Afterload reducers.

The ACE inhibitors.

Like captopril or enalapril.

These are key for blocking that damaging RAAS response.

They stop the vasoconstriction, which lowers the afterload and makes it easier for the heart to pump.

And there's a crucial interaction with diuretics.

Yes.

You have to schedule them one to two hours apart.

If you give an ACE inhibitor and a diuretic at the same time, you can cause profound hypotension.

And finally, beta blockers, like carvetolol or propranolol.

These are great for reducing the long -term damage from all that adrenaline.

Propranolol is also the go -to for treating hypercyanotic spells into Tralogy of Fallot.

But there are some dangers with non -selective beta blockers like propranolol, right?

Yes.

It can cause bronchoconstriction.

So you have to be careful in kids with asthma.

And critically, it can both cause and mask the signs of hypoglycemia.

Parents have to be aware of that.

For nursing interventions, let's start with diuretics to manage the fluid.

We use loop diuretics like furosemide, but they waste a lot of potassium, which is risky.

So we often pair them with a potassium -sparing one like spironolactone.

Now for the most counterintuitive part of pediatric cardiac nursing, oxygen.

This is probably the single most important distinction you have to learn.

Oxygen is a potent pulmonary vasodilator.

It opens up the blood vessels in the lungs.

Right.

So if a child has a defect that already increases blood flow to the lungs, a left to right shunt, like a VSD, and you give them oxygen, you are making it worse.

You are lowering the resistance in the lungs, which increases the shunting, worsens the pulmonary edema, and can cause respiratory failure.

So oxygen can be actively harmful in these kids.

It can be.

You do not routinely give oxygen to children with increased pulmonary blood flow, even if they look like they're in distress.

You manage the fluid, you support their breathing, but you avoid the oxygen.

And finally, you have to address the malnutrition.

These kids have such high caloric demands.

So we increase the caloric density of their formula or breast milk.

We teach parents to do small frequent feedings.

And sometimes we have to use an NG tube to give them calories without them having to expend the energy to eat.

And you always have to support the parents.

You have to empower them.

Teach them CPR.

Teach them how to give them meds safely.

Teach them that it's okay for the baby to cry a little bit.

You have to help them avoid that fragile patient syndrome.

Let's get into the specific defects now, categorized by what they do to the blood flow.

It all comes down to pressure, right?

High pressure left side to low pressure right side.

Right.

So asianotic means a left to right shunt.

Oxygenated blood goes back to the lungs.

Cyanotic means a right to left shunt.

The oxygenated blood bypasses the lungs and goes to the body.

Let's start with defects that increase pulmonary blood flow.

Asianotic.

First up, patent ductus arteriosus PDA.

The fetal shunt just failed to close.

So you have constant shunting of oxygenated blood from the high pressure aorta into the lower pressure pulmonary artery.

It's just uselessly cycling through the line.

And the murmur is unforgettable.

It's a continuous loud machine -like murmur.

In preemies, we can use endomethazine to try and close it chemically.

Otherwise, it's catheter closure or surgery.

Next, the most common one, ventricular septal defect, VSD.

A hole between the ventricles.

Again, left to right shunt, increased pulmonary blood flow.

It creates a harsh holosystolic murmur at the left lower sternal border.

And this is where that four to six week timeline comes into play.

Exactly.

The murmur might not show up until the pulmonary pressures drop.

Small ones often close on their own.

Big ones that cause failure to thrive need surgical closure.

Finally, in this category, atrioventricular septal defect, AVSD, which is very common in one specific population.

Down syndrome.

AVSD is highly associated with trisomy 21.

You have holes in both septa and often a common AV valve.

It leads to early severe heart failure.

And the timing for repair is different for kids with down syndrome.

It is.

They are at much higher risk for developing irreversible pulmonary hypertension and faster.

So they often go for surgical repair by three months of age, which is earlier than other kids with AVSD might.

OK, now we shift.

Defects that decrease pulmonary blood flow, the cyanotic ones.

Let's start with transposition of the great arteries.

TGA.

In TGA, the plumbing is just switched.

The aorta comes off the right ventricle and the pulmonary artery comes off the left.

You have two totally separate parallel circuits that don't mix.

Which means immediate severe cyanosis at birth.

And survival depends entirely on having some mixing through the fetal shunts, the PDA and the PFO.

So the first thing you do is start a continuous infusion of prostaglandin E1 PGE1 to keep the ductus open.

What are the nursing alerts for PGE1?

It's a powerful drug.

The biggest side effects are apnea and hypotension.

Most of these babies will need to be intubated because the apnea is almost guaranteed.

And if that's not enough mixing.

They can do a balloon atrial septostomy at the bedside or in the cath lab to rip a bigger hole in the atrial septum to allow for more mixing until they can get to the operating room.

And the definitive surgery is the arterial switch.

Yep, the Jatine procedure.

Ideally done in the first two weeks of life.

They switch the great vessels back to where they belong.

And this is the critical part.

They have to move the tiny coronary arteries from the old root to the new one.

A big long -term risk is narrowing at that re -implantation site.

Let's move to the most common cyanotic defect.

Tetralogy of phallate.

There are four components.

One, severe pulmonary stenosis.

Two, a large VSD.

Three, an overriding aorta that sits over the VSD.

And four,

right ventricular hypertrophy from pushing against that stenosis.

And this is the defect famous for tet spells.

Describe what happens during a spell.

A tet spell is when the muscle under the pulmonary valve suddenly spasms and clamps down.

Often when the baby is crying or upset.

This acutely blocks off blood flow to the lungs.

Since there's a big VSD, the blood takes the path of least resistance.

Which is now right across the VSD and out to the body.

Right, a massive right to left shunt, causing profound sudden cyanosis.

And the immediate nursing intervention is the knee to chest position.

Why does that work?

By bringing the knees to the chest, you are kinking the hose to the body.

You are dramatically increasing the systemic vascular resistance, the SVR.

This makes it harder for blood to go out the aorta, which forces more of it to push through that tight pulmonary valve, improving oxygenation.

If the stenosis is too bad to fix right away, they might do a temporary shunt.

The Modified Bleyloctasic, or BT, shunt.

It connects the subclavian artery to the pulmonary artery to give them a stable source of blood flow to the lungs until they're big enough for the full repair, which is usually around 3 -6 months.

Okay, now defects that cause an obstruction to systemic blood flow.

Coarctation of the aorta?

CoA?

This is the narrowing of the aorta.

The key assessment finding is a difference in pulses.

You have to palpate the right radial and femoral pulses at the same time.

In Coal, the femoral pulses will be weak, absent, or delayed.

And the key finding with blood pressure?

A systolic BP in the right arm.

That is 10 mmHg or higher than what you get in the leg.

That's the hallmark.

Management is balloon angioplasty, stenting, or surgical repair.

What about aortic stenosis?

That's an obstruction to outflow from the left ventricle.

It makes the LV work really hard, leading to hypertrophy.

Kids often complain of chest pain with exercise.

You'll hear a harsh systolic murmur at the right upper sternal border.

Let's tackle the most complex of all.

Single ventricle defects, like hypoplastic left heart syndrome, HLHS.

This is where the left side of the heart is just too small to function.

The right ventricle has to do all the work, pumping blood to both the lungs and the body.

So it's a ductal -dependent lesion.

The ultimate ductal -dependent lesion.

If that PDA closes, the baby dies.

So immediate PGE1 infusion is mandatory.

All the blood mixes in the right atrium and the RV pumps that mixed blood out.

And the saturation goal is really unique and low.

It is.

We aim for a saturation between 75 and 85 percent.

It's all about balancing the flow between the lungs and the body.

And this is another case where too much oxygen is dangerous.

Extremely dangerous.

Oxygen is a pulmonary vasodilator.

If you give oxygen, you drop the pulmonary resistance, and all the blood will steal away from the body and flood the lungs.

This causes metabolic acidosis and circulatory collapse.

Oxygen is a drug to be avoided in most HLHS patients.

The repair is a three -stage palliation.

It's a marathon.

It is.

Stage one is the Norwood procedure, done in the first few days of life.

It's a massive reconstruction where they build a new aorta and place a shunt to get blood to the lungs.

It has a high mortality rate.

Stage two is the bi -directional Glenn shunt at four to six months.

Here, they connect the superior vena cava directly to the pulmonary artery.

This allows blood from the upper body to flow passively to the lungs, taking a huge volume load off that single ventricle.

And stage three is the Fonten procedure at two to three years.

This is the final step.

They direct the blood flow from the inferior vena cava to the pulmonary artery.

At this point, all the deoxygenated blood is flowing passively to the lungs, and the single ventricle is dedicated only to pumping blood to the body.

Final sats are around 90 to 95 percent.

Shifting now to post -op care.

This is an ICU environment.

The prep work with the family beforehand is so important.

Pre -op care is all about reducing their anxiety.

You have to prepare them for what their child will look like.

The ventilator, the chest tubes, all the drips, the noise, it can be overwhelming.

And using resources like child life specialists.

Absolutely.

They can use play to teach the child about deep breathing and coughing using an incentive spirometer.

If they practice before surgery, they're much more compliant after.

Most of these surgeries use cardiopulmonary bypass.

What are the immediate side effects the nurse needs to watch for?

Bypass triggers a huge inflammatory response.

You can see massive fluid shifts,

generalized edema.

The child will be very swollen.

You'll also see decreased lung compliance and changes in their ability to clot.

In terms of perfusion, what's the single most important indicator of cardiac output after surgery?

Urine output.

Period.

If the heart is perfusing the kidneys, they'll make urine.

The target is 0 .5 to 1 millimil per kilogram per hour.

Anything less than that for a sustained period is a red flag.

Sometimes the surgeon will leave the sternum open.

Why?

An open sternal incision gives the swollen post -op heart room to expand without being compressed.

This requires meticulous sterile care to prevent infection until they can go back and close it a few days later.

We have to get them to breathe deeply even though it hurts.

So that means aggressive pain management.

Scheduled analgesia, PCA pumps.

If they're in pain, they'll take shallow breaths and they'll get atelectasis.

And chest tubes.

What are the key nursing interventions?

You have the three chambers.

Collection, water seal, and suction.

You need to make sure the system stays closed and below the level of the chest.

If you see continuous bubbling in the water seal chamber, that suggests an air leak.

And critically, you want to avoid aggressive stripping or milking of the tubes, which can cause lung damage.

Dysrhythmias are a huge risk.

Which is why they almost always have temporary pacing wires placed on the heart.

If the heart block or other rhythm issues don't resolve after about 10 days, they'll need a permanent pacemaker.

For discharge, what's the most important safety instruction for parents about handling their child after a sternotomy?

They cannot lift the child under the arms for about six weeks.

That puts way too much strain on the healing sternum.

You have to teach them to scoop the baby up with one hand behind the neck and the other under the bottom.

What about activity?

A return to normal, light daily activities is good.

But no heavy lifting, no competitive sports until the cardiologist clears them.

Even tummy time is restricted for a few weeks for infants.

And finally, we have to talk about the neurodevelopmental risk.

It's high, especially for neonates who have open heart surgery, kids with cyanotic heart disease, or anyone with a long hospital stay.

They're at risk for delays in cognition, motor skills, language.

As nurses, we have to push for developmental screening and early intervention referrals.

Let's focus specifically on cardiac cath care.

It's still a high -risk procedure.

Pre -procedure, you're teaching the child what to expect.

And you have to stress that afterward, they have to keep their leg flat and straight to prevent bleeding.

Post -procedure, it's all about two things, bleeding and perfusion.

Right.

For bleeding, they have to lie flat for several hours.

You're checking the dressing constantly, but you also have to check the tissues around it.

Is there a hematoma forming under the skin?

If you see active bleeding from the site, what is the immediate life -saving action?

You apply firm, continuous pressure directly to the site for at least 10 minutes, and you call the provider immediately.

You do not let go.

And for perfusion risk?

You're checking those distal pulses, the pedal pulse, the posterior tibial pulse frequently.

If you lose a pulse or the foot becomes cool or pale, that suggests a clot.

You have to notify the provider right away.

They might need heparin to prevent limb loss.

Okay, let's shift to acquired heart disease.

The number one cause in children is Kawasaki disease.

Kawasaki is an acute vasculitis, an inflammation of the blood vessels.

It's a diagnosis of exclusion, so you have to know the clinical criteria.

What are the criteria a nurse needs to have memorized?

A prolonged fever for five or more days plus four out of five of these symptoms.

Bilateral conjunctivitis without pus, oral changes like a strawberry tongue or cracked lips, a rash, a swollen lymph node in the neck, and changes in the hands and feet.

Redness, swelling, and later peeling.

And the danger is coronary artery damage.

The vasculitis can cause life -threatening coronary artery aneurysms.

Treatment is incredibly time -sensitive.

You have to give high -dose IVG and high -dose aspirin within seven to ten days of the fever starting to prevent those aneurysms.

High -dose aspirin is unusual in kids.

Why here?

It's for its anti -inflammatory effect at first, and then a low dose is continued for its anti -platelet effect to prevent clots from forming in any damaged arteries.

They need follow -up echoes to check the coronaries.

Moving on to rheumatic fever.

This is an autoimmune response that happens a few weeks after an inadequately treated strep throat.

It can permanently damage the heart valves, especially the mitral valve.

We diagnose it with the Jones criteria.

What are the major symptoms?

Carditis inflammation of the heart, polyarthritis migratory joint pain, synonym cordia, those jerky movements, a specific rash called erythema marginatum, and subcutaneous nodules.

You need evidence of a recent strep infection plus a combination of those symptoms.

And management is long -term.

Yes.

You treat the acute infection with penicillin, but then they need long -term penicillin prophylaxis, often monthly injections, until they're at least 21, sometimes for life, to prevent another attack.

Next, cardiomyopathy, a disease of the heart muscle itself.

Right.

There's dilated cardiomyopathy, which is a big floppy weak heart.

Then there's hypertrophic cardiomyopathy, or HCM.

And HCM is the scary one.

It is.

It's a massively thickened heart muscle that has trouble relaxing.

It's the most common cause of sudden cardiac death in healthy young athletes.

Management is beta blockers, and critically, activity restriction.

What kind of activity restriction?

They have to avoid all highly aerobic, competitive sports, and isometric exercises like weightlifting.

These activities can trigger a fatal arrhythmia.

Finally, infective endocarditis, i .e.

an infection on the heart valves.

Its incidence is actually going up as more kids with CHD survive.

Treatment is a long course of IV antibiotics, usually four to six weeks, often at home with a PICC line.

And the prophylaxis guidelines have changed.

They have.

Prophylactic antibiotics before dental work are now only recommended for the highest risk groups.

Who is that?

People with prosthetic valves, a prior history of IE, or kids with certain types of unrepaired or residually repaired cyanotic heart disease.

A simple repaired VSD does not need it.

So we circle all the way back to prevention of adult disease in childhood, starting with pediatric hypertension.

Right.

Blood pressure in kids is based on percentiles for their age, gender, and height.

And a diagnosis requires three separate elevated readings on three different occasions.

And getting an accurate reading is so important.

It is.

You have to use the right cuff size.

You have to have the arms supported at heart level.

If you get a high automated reading, you have to confirm it manually.

Management is lifestyle first.

Always.

Weight management, diet, exercise, the whole family has to get involved.

Meds are only started if those changes don't work.

Second, dyslipidemia.

What are the screening guidelines for cholesterol now?

Universal screening is recommended for all kids, once between ages 9 and 11, and again between 17 and 21.

High risk kids should be screened earlier.

And management starts with diet.

Yep.

The child one diet after age two.

If that doesn't work, the more restrictive child two diet.

Stats are only considered if diet fails and the child is 10 or older.

And the root cause linking both of these is the prevalence of overweight and obesity.

Absolutely.

And the most important nursing intervention is to stress that this has to be a family effort.

You can't put a child in a healthy bubble in an unhealthy home.

The entire family unit has to change their behaviors.

This has been an incredibly deep dive.

We've gone from the womb all the way through to preventing adult disease.

I think the key takeaways for nurses are clear.

You have to master that multi -system assessment.

You have to recognize that the signs of heart failure in a baby are things like sweating while eating.

You have to understand the pressure dynamics that drive the shunts.

We learned about the critical timing.

The immediate PGE -1 for TGA or HLHS, that seven to 10 -day window for IV and Kawasaki, and the delicate balance of that three -stage palliation.

And maybe the most important lesson.

Not all low -sats need oxygen.

We started with that parent of the baby with a VSD, asking what to watch for.

And now we know the answer.

You teach them to watch for those subtle signs of volume overload, the fast breathing, the sweating.

You tell them to expect those signs to get worse around four to six weeks as the lung pressures drop.

And that brings us to our final provocative thought.

If you think about the incredibly complex multi -year surgical journey for kids with the most severe defects like HLHS,

how can we as nurses best support their parents in moving from a mindset of illness, seeing their child as a fragile patient, to a mindset of wellness?

How do we help them maximize quality of life, even when there are lifelong restrictions?

It is an ongoing psychological journey that requires all of our clinical skill and all of our empathy.

It's a challenging but really rewarding place to end.

Thank you from the team.

We hope you feel more prepared to take on this crucial part of pediatric care.