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Acyanotic Congenital Heart Defects

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This content provides a comprehensive overview of acyanotic congenital heart defects (CHDs), focusing on their types, causes, pathophysiology, clinical presentations, diagnosis, and treatment. It explains how these defects, characterized by left-to-right shunts or outflow obstructions, do not typically cause cyanosis but can lead to significant complications if untreated.

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what's up ninja nerds in this video

today we're going to be talking about

congenital heart defects it's going to

be a two-part series we're going to have

one on a cyanotic which is going to be

in this video and then we'll have

another video right after this on

cyanotic congenital heart defects let's

get started though so we talk about a

cyanotic and genital heart defects

they're defects within the baby's heart

that does not cause cyanosis what does

that mean so cyanosis is basically the

bluish discoloration of the skin that's

the concept here and these diseases they

typically do not cause cyanosis

therefore a cyanotic there is a little

nuance to that and we'll talk about it

called eisenmanger syndrome but for the

most part these conditions do not cause

cyanosis now let's talk about those

particular a cyanotic and genital heart

defects so we have something called an

asd that's an atrial septal defect it's

simple there is a hole within the inter

atrial septum and that's causing blood

to shunt from the left atrium the higher

pressure system to the right atrium the

lower pressure system because blood

always likes to go from areas of high

pressure to low pressure

so we call this a

shunt going from the left side of the

heart to the right side of the heart okay

okay

now if you think about that why would

that not cause cyanosis

the reason why is because the left side

of the heart is the oxygenated blood and

you're pushing oxygenated blood into the

right side of the heart which is the

deoxygenated circuit and the right side

pumps blood to the pulmonary circulation

the problem that causes cyanosis is when

deoxygenated blood is getting pumped

into the aorta but that isn't happening

in this situation okay remember that the

next one is called a vsd ventricular

septal defect it's a hole within the

interventricular septum that causes

blood to shunt from the left ventricle

higher pressure system to the right

ventricle lower pressure system pushing

oxygenated blood into the deoxygenated

right ventricle that's not going into

the aorta though okay so remember that

next one pda patent ductus arteriosus in

this situation there is a hole that is

staying open you know there's a

structure in the baby called the ductus

arteriosus it's a hole between the

pulmonary artery and the aorta whenever

the baby is born it should close off and

become the ligament arteriosum but in

this situation it stays patent open

causing blood to shunt from the aorta

the higher pressure system into the

pulmonary artery the lower pressure

system so we're shunting oxygenated

blood from the aorta into the pulmonary

artery again there's no cyanosis in

these situations

the next one is the endocardial cushion

defect sometimes called an

atrioventricular septal defect this

one's an interesting one so it's kind of

like a combination of asd and vsd so

there is an asd thus showing this hole

between the left atrium and the right

atrium that's where blood can shunt from

the high pressure left atrium to the low

pressure right atrium there's also a vsd

where blood can shunt from the high

pressure left ventricle to the low

pressure right ventricle all pushing

oxygenated into the deoxygenated not

going the other way

the last thing that's big difference

here with endocardial cushion defects is

that not only is there an asd a vsd but

if you notice the mitral valve and the

tricuspid valve there's only one

leaflet there so we're missing some of

the leaflets from the tricuspid and

mitral valve so there's abnormalities

within those leaflets as well

the big thing is this whole category

here are called your left to right

shunts meaning you're pushing blood from

the high pressure left side of the heart

whether it be left atrium to right

atrium left ventricle to right ventricle

or aorta into the pulmonary artery

all of these things you're shunting

oxygenated blood into the deoxygenated

circuit that doesn't cause cyanosis

where cyanosis comes is when you push

blood from the deoxygenated side of the

heart right ventricle to left ventricle

right atrium to left atrium or pulmonary

artery to aorta but that's not happening

what happens in this situation is you

push a lot of blood into the right side

of the heart which pushes a lot of blood

into the pulmonary circulation and the

end pathophysiology behind this is you

get a lot of pulmonary blood flow and

we'll talk about that later

the last one here that's kind of like a

little outcast kind of a random one that

we just add into this category of a

cyanotic because it would be in an

individual video is it's called an

outflow obstruction we're obstructing

the flow of blood

into the circulation and where that

actually happens is you have something

that's like a little stenotic or

narrowed or constriction ring of a

particular part of the aorta and what

that does is that obstructs the blood

flow down into the lower extremities and

so you get less blood flow down into

your lower extremities but all of the

branches of the aortic arch that give

blood flow to the head and the arms

you're getting good blood flow to those

so you have this differential type of

blood flow and sometimes you'll we'll

talk about this later you can have

shunting because sometimes correctation

of the aorta can be associated with pdas

and you can get something called

differential cyanosis we'll talk about

that later with the spiderman dude but

the big thing i need you to understand

the difference between these two is

these are left to right shunts this is

not necessarily a shunt it's an

obstruction of blood flow into the

systemic circulation particularly where

you have higher blood flow in the upper

extremities and upper body and decreased

blood flow in the lower extremities or

lower body okay that gives us the

categories the definitions if you will

let's talk about the causes of these

different types of a cyanotic congenital

heart defects so there's going to be a

couple here one two three four what i

need you to know we got patients who

have chromosomal abnormalities two types

down syndrome so that's going to be one

of your trisomy 21s

in that situation we see down syndrome

associated with these particular

conditions atrial septal defect

ventricular septal defect endocardial

cushion defect and pda

pda

the other chromosomal abnormality which

is within particularly the x chromosome

and y chromosome compulsion component

here so somebody has an absent y

chromosome this is called turner

syndrome and so they can have that

webbed neck they can have that shield

type of chest they can have very short

stature they have

coordination of the aorta so remember

turner syndrome coartation of the aorta

down syndrome asd vsd endocardial

cushing defect and pda

the next one is when the mother

unfortunately drinks large amounts of

alcohol they can develop fetal alcohol

syndrome that can alter the development

the growth of the fetus and this is a

particular one that you can see in

patients who develop asd or pdas

the next one is your torch infections so

these are intrauterine infections we

have chromosomal abnormalities fetal

alcohol syndrome and infections

intrauterine infections with the

emphasis being on one of the components

of this torch series so torch stands for

a bunch of different things you have

toxoplasmosis you have other you have

rubella you have cytomegalovirus herpes

there's a bunch of different types of

intrauterine infections but the most

important one is the r and that is the

rubella and so rubella can actually

cause vsds but the big one that i really

want you to remember that with is the

pda the patent ductus arteriosus and the

last one here is maternal diabetes so

diabetes within the mother there is a

potential increased risk of ventricular

septal defects so these are the big

things to remember for our causes let's

come down and talk about the

pathophysiology of these a cyanotic

congenital heart defects all right so

pathophysiology of these defects so

let's talk about the asds first so

there's a couple different components

here so first thing is we should have

the normal idea of what the inter atrial

septum is formed by so there's a couple

different types of tissues we're not

going to go into crazy detail i just

want you guys to get the basic just and

the normal situation here you guys see

that blue line there that blue line is

actually there's a septum that grows

down to make a part of the enter atrial

septum and what i want you to remember

is that blue line there is called the

septum primum the septum prime is

supposed to basically go through entire

length here from top to bottom now what

happens is

as it works its way down down down it's

supposed to get close to this actual

septus the septum intermedium here which

is basically the intermediate portion

between the septum of the atria and the

septum of the ventricles okay

but this thing is supposed to go all the

way down that's called the septum primum

as it goes all the way down it makes a

little hole in the top of it called the

ostium secundum okay

then after the ostium secundum there's a

tissue here that's supposed to come all

the way down here all the way down to

the bottom and cover up that ostium

secundum and this is that pink tissue

and that pink tissue is called the

septum secundum okay it's called the

septum secundum so you have the septum primum

primum

there's a hole that forms here after it

goes all the way down called the ostium

secundum the pink tissue that's supposed

to cover that ostium is called the

septum secundum

then normally there's a little hole here

in the baby called the

foramen ovale but whenever they're born

that should actually become a little bit

more of a scar tissue there and close

this area off becoming the fossa ovalis

so whenever the baby's born this is what

it should potentially be

now if you have a defect within the

formation of the septum or you don't

actually form the fossil values what can

happen my friends so first things first

if the septum primum does not come all

the way down it leaves a defect here in

the bottom where blood can shunt

what is that called well it's a defect

within the septum primum so we call that a

primum asd this is the less common one

the other one is the secondum asd what

do you think that's an issue with it's

an issue with the septum secundum it

doesn't come all the way down and block

off the osteosecundum so blood can shunt

from the left atrium to the right atrium

that causes that left to right shunt

the last one here is called the pfo now

the pfo is the interesting one the pfo

is because remember we said that this is

this is a little area right here it's

this little area right here where if i

imagine i actually took like a little

probe or something and i ran this probe

from this area to this area that is the

patent foraminal valve now normally that

wants to allow blood to go from the

right atrium to left hem in the baby

right because we don't want blood to go

to the lungs because the lungs aren't

functional you're in utero you're in

amniotic fluid the placenta is basically

the lungs in this situation

we want it to close though because we

want now blood to go towards the

the pulmonary circuit

but in certain situations

this actual pfo can stay open and this

may seem paradoxical but the pfo is

actually a little bit better at allowing

blood because it acts kind of like this

weird little septum it's actually better

at allowing blood to flow from areas of

the right atrium into the left atrium so

it's not necessarily kind of like an asd

it's important to remember that that a

pfo is actually completely different

from an asd those are defects within the

septum primum and septum secundum this

is a defect where it actually doesn't

fuse between this area and form the

fossil valve since how it stays patent

allowing for blood to be able to

potentially flow from right atrium to

left atrium now you might be like well

zack i thought it always goes from areas

of high pressure to low pressure it does

but whenever it tries to go this way it

kind of like kind of closes it off so

sometimes what happens is if you get

like somebody who gets developed like a

clot like a dvt

and it gets up into the right atrium and

it gets stuck for a momentary time and

the pressure rises up in the right

atrium it can actually pop that clawed over

over

into the left atrium and then it can go

left atrium left ventricle get pumped up

into the systemic circulation and cause

an embolus and so this is actually

called a paradoxical embolus and you can

see this with pfo so it's a big

difference to understand that

okay that's our asd so i want you to

remember secondum is the more common one

issue with the septum secundum primum is

the less common one

now the next one is your vsds now the

vsds actually form very interestingly so

here's going to be the ventricles right

so this is the ventricular wall here so

if you can imagine this is like the

right ventricular wall this is the left

ventricular wall and we call this

portion here this is like the septum

intermedium now what happens is from the

apex of the ventricles you have this

blue portion that moves its way up to

form a part of the septum okay that's

called the muscular portion of the

interventricular septum and then from

the septum intermedium there's this pink

portion that comes down and that's

called the membranous component of the

interventricular septum guess what we have

have

if you don't form the membranous portion

it's called a membranous vsd which

allows blood to shun from the left

ventricle to the right ventricle

if the muscular portion doesn't

completely form and sometimes this can

actually make it a big vsd or it can be

like sometimes you call like a swiss

cheese vsd because you can have multiple

pieces of it and it just completely

malforms but either way the muscular

portion of the vsd is an issue and that causes

causes

the left to right shunt it's actually

more common to see the membranous vsds

than it is the muscular vsds

okay we're moving on a roll here our

next one is the pda the patent ductus

arteriosus so remember i told you in the

baby there's something called the ductus

arteriosus it's the little hole between

the pulmonary artery that i'm tapping on

here and the aorta normally the whole

purpose is that in the baby you don't

want blood to go to the lungs and so

generally what you do is you shunt blood

in this way in the baby okay but then

what happens is when the baby is born

this should actually close off because

now you don't want this connection to be

open anymore and this closes off and

when it closes off

you now no longer have a connection

between the award and the pulmonary artery

artery

and this is called the

ligamentum arteriosum

but in certain situations as we talked

about above where someone develops a pda

it stays open

and then blood can shunt and remember

which in the baby when they're born

it's important actually preface this

when a baby is in utero

what happens blood flow doesn't go to

the lungs and so the pressure inside of

the pulmonary system is higher because

it naturally constricts the vessels

because we don't want blood to go to the

baby's lungs and so it's a unique

mechanism and so generally in the baby

the pressure's super high in the

pulmonary circuit that we can shunt it

from the pulmonary artery into the aorta

that's in the baby when it's in utero

when it's born what happens is the

pulmonary vessels dilate so now we want

blood to go to the pulmonary arteries

and so the pulmonary pressures drop they

drop so much that the pressure inside of

the artery is higher than the pressure

inside of the pulmonary arteries and so

we know that left sided pressures are

always higher than the right side of

pressures and whenever a person's born

and so this causes the shunt to go from

the aorta into the pulmonary arteries so

you guys get the point there so the

problem is we don't make the ligamentum

arteriosum we keep it open and we call

it a patent ductus arteriosus okay

the next one here is called the

endocardial cushion defects so the

endocardial cushion defects remember i

told you there's what's called that

septum intermedium

and what happens is from the septum

intermedium you actually form a portion

of the

mitral valve you form a portion of the

tricuspid valve you form a portion of

the interatrial septum and you form a

portion of the interventricular septum

if a patient who actually does not have

these endocardial cushions working

there's a defect within the growth of

the endocardia cushions are they going

to be able to make the interatrial

septum no so in this situation there is

no interatrial septum are they going to

be able to make the intraventricular

septum no so they're not going to have

it in a completely formed

interventricular septum are they going

to be able to have functional

tricuspid valve leaflets and functional

mitral valve leaflets no

and so what happens is this allows blood

to shunt into the left atrium to the

right atrium because higher pressure and

blood to shunt from the left ventricle

into the right ventricle because of a

higher pressure system and you can even

have reflux into the atria because you

don't have functional tricuspid and

mitral valve leaflets to block blood

flow that's a terrible situation to see

here big thing to remember here

going off of these is endocardial

cushion defects

usually this presents in children about

six weeks of age and same thing vsd

usually presents that with children

about six weeks of age the vsd and the

pda and even co-optation of the aorta

they can really present at any kind of

time or age in these patients so

remember to please don't forget that okay

okay

the next and last one here is that

obstructive type that's the coarctation

of the aorta what happens is the

co-octation of the aorta is you form

these little constriction rings and

what's really important for you guys to

remember is where the constriction ring

forms with respect to the ductus

arteriosus if it's in the baby but if

it's supposed to close off it should be

the ligamentum arteriosum right so we

say where is the constriction ring with

respect to the ductus arteriosus or the

ligament of arteriosum or in some

situations they may even have it open

a pda so where is it with respect to

that structure

so if the constriction ring is before

the ductus arteriosus or the ligamentum

arteriosus and we call that

preductal corotation of the aorta what i

need you to remember is this is more

common in

infants this is the infantile type of

coordination of the aorta and the

problem is is that all the pressure if

you're having this blood going through

here the pressure is gonna be having to

squeeze through this tiny little area

here and so the pressure in this area is low

low

and guess what the pressure in the

pulmonary arteries actually might be a

little bit higher that it causes blood to

to shunt

shunt

into the aorta causing a little bit of

mixing of blood and so you can get

cyanosis of the lower extremities but

have completely like normal blood flow

sometimes in certain degrees to the

upper extremities okay

postductal a post-ductal correctation of

the aorta is if you look at the

constriction ring it is after the ductus

arteriosus of ligamentum arteriosum this

is more common in the adult type so it's

important to be able to remember that a

pre-ductal is infantile a post-octal is

adult pre-ductal constriction ring

before the ductus arteriosus ligaments

marteriosum postdoctoral constriction

ring after the ligamentum arteriosum or

the ductus arteriosus okay i think we

got the definitions down i think we got

our causes down the basic

pathophysiology let's go over the

clinical picture now all right ninjas

let's move on to the next part so when

we talk about pathophysiology of these

asymmetric defects really i think one of

the big things is we're going to kind of

keep the obstructive one the coarctation

of the aorta since it's such a random

one we're going to talk about that a

little bit separately we're going to

really really focus a little bit more on

those left to right shunts which if you

remember what did i put there the

primary problem was that it was

increasing pulmonary blood flow we were

shunting a lot of blood into the right

side of the heart and pushing a lot of

blood through the pulmonary circulation

why can that be a problem let's think

about it if you have a lot of pulmonary

blood flowing through there you're gonna

have a lot of pulmonary pressures right

so you're gonna have these high

pulmonary blood pressures because you're

having a lot of pulmonary blood flow you

push a lot of pulmonary blood into the a

lot of blood into the pulmonary artery

you're going to increase your pulmonary

blood flow and increase your pulmonary

blood pressures

if you have high pulmonary pressures

what is the issue with this well think

about it

if i'm having a lot of shunting of blood

from a vsd

over right from left ventricle to the

right ventricle i'm having a lot of

blood being pumped into the right atrium

from the left atrium via an asd or

combined an ecd or i'm having a lot of

blood shunting into the pulmonary artery

via the pda all of those things are my

left right shunts either way the end

result is i'm pumping a lot of blood

into the pulmonary arteries i'm

increasing the pulmonary artery

pressures i'm increasing the leaking of

blood out of the pulmonary capillaries

if i cause a lot of like fluid to leak

out of the pulmonary capillaries that's

going to cause edema pulmonary edema and

that's going to alter the gas exchange

process leading to dyspnea tachypnea so

the babies can actually be short of breath

breath

on top of that imagine you having a lot

of fluid that's accumulating within the

lungs it's congesting the lungs it's

causing them to become more fluid and

waterlogged it's harder to be able to

clear infections within those lungs and

it increases the risk of bacteria

thriving that can cause recurrent

bronchopulmonary infections because of

the congestion of fluid that's a big

thing to remember

here's another one that's really interesting

interesting

i'm supposed to have a lot of blood

flowing through the pulmonary

circulation but think about it the right

side of the heart is getting overloaded

with blood it can start to fail if it

starts to fail where's that blood going

to back flow into the liver and this is

one of the few signs that you can see of

right-sided heart failure which is

apatomy it commonly babies don't present

with ankle or pedal edema they rarely

present with jugular venous distension

they may present with some puffy eyes

but the most common clinical feature for

that right-sided heart failure is

sometimes just a swollen type of liver

or hepatomegaly that's some of the

things to think about so they can

develop right sided failure because of

the high pulmonary pressures and lots of

fluid accumulation pulmonary edema the

fluid logging causes bacteria to thrive

harder to clear them and recurrent

bronchopulmonary infections okay

next thing here

because of those high pulmonary

pressures think about what is the

kind of like an indirect result of this

remember every single time you're

supposed to be getting blood from the

pulmonary veins that go into the left

atrium right so it's supposed to come

into left atrium so my left atrium has

this blood if some of that blood is

getting shunted over

into the right atrium i'm losing volume

that i'm supposed to be putting into my ventricle

ventricle

if i'm getting volume into the left

ventricle but i'm shunting some of it

into the left ventricle

or if i'm supposed to be pumping blood

into my aorta but i'm shunting it into

the pulmonary artery either way i'm

reducing filling of the left ventricle

i'm reducing the amount of volume of

blood is being pushed out of the left

ventricle and i'm reducing the amount of

blood that's filling the aorta because

i'm pushing it all into the pulmonary

trunk or some of it into the pulmonary

trunk you get the point here right

i'm really reducing the total cardiac

output so the amount of blood that's

getting pumped out of the heart into the

systemic circulation is lower because

i'm either shunting it from the left

atrium to the right atrium showing it

shunting it from the left ventricle to

the right ventricle or shunting it from

the order to the pulmonary trunk that's

dropping my cardiac output

if you drop your cardiac output you drop

your blood pressure you don't perfuse

tissues and one of the big things is the

baby's skin they start to look pale they

have cool extremities that's one of the

big things the other thing

and this is one of the really

interesting ones is whenever your blood

pressure drops you create a sympathetic

reflex it's basically your body saying

hey the blood pressure is low we gotta

up the blood pressure somehow so your

sympathetic nervous system kicks in and

one of the things it does it says hey

i'm going to increase the heart rate and

so it increases your heart rate and

trying to increase heart rate you try to

increase cardiac output to try to

increase blood pressure but then the

baby's heart rate is just flying on top

of that sometimes you try to increase

the respiratory rate hoping that you can

actually increase the actual oxygen

delivery into the bloodstream that's but

that doesn't actually help it just makes

them to kipnic on top of that whenever

you have a high sympathetic overflow it

causes the sweat glands to be stimulated

and they cause a lot of diaphoresis

here's what's really interesting though

whenever there's a lot of sympathetic

activity it can actually cause the

patient to become more fatigued over

time and especially babies the way that

they present with fatigue is they don't

have the correct amount of energy to be

able to eat and so they have poor

feeding and then sometimes a failure to

thrive so these are big things to think

about that these patients can present

with and this could be kind of a

left-sided heart failure type of thing

where they're not being able to generate

enough cardiac output because they're

shunting all of it into the right side

of the heart increasing the pulmonary

blood flow and pressures but they're

dropping their pressures not perfusing

the skin and creating these sympathetic

reflexes so don't forget that okay

okay

on to the next part and this is the

worst case scenario for these babies who

develop a cyanotic congenital defects if

they have very this is the big thing to

remember usually you won't see this with

like small asds you really see this with

big vsds endocardial cushion defects and

sometimes pretty big pdas

you can get something called eisenmanger

syndrome if i were to really think about

the most important one to remember it's

really large esds that's the big one

that can lead to this

but with eisenmanger syndrome what

happens is you're constantly pushing blood

blood

into the right side of the heart

and if you're filling the blood

in the right side of the heart whether

it be via the

pda the asd or the vsd but i'll put most

of my emphasis on the vsds large vsds

you're pushing large amounts of volume

into the actual right side of the heart

the right side of the heart is going to

have to respond to that

and what it does is it starts trying to thicken

thicken

and become stronger

and so it leads to right ventricular hypertrophy

hypertrophy

on top of that here's where it gets

really interesting

do you see these pulmonary vessels here

whenever the pulmonary vessels are on

such hot under high high pressure

they have to respond and they do this in

a very interesting way because we're

pushing so much blood into the right

side of the heart whether it be left

atrium to right atrium right ventricle

left ventricle to right ventricle or

aorta to pulmonary arteries they're all

the pulmonary vessels are getting filled

with blood

when they get filled and filled and

filled with all of this blood they can't

tolerate that high pressure for long

so they have to protect themselves and

what they do is they undergo an intense

reactive vasoconstriction so they're

going to constrict their vessels that's

the first thing

second thing is they're going to thicken

the walls of the vessels when you

vasoconstrict the vessel you decrease

the aluminum when you thicken the vessel

you again cause this peripheral vascular

resistance the amount of resistance to

blood flow through that pulmonary vessel

to jack up so the pulmonary vascular

resistance is going to

increase significantly so if the

pulmonary vascular resistance in these

vessels increases

now the right ventricle is going to have

to work even harder to pump blood into it

and you know what's really interesting

is normally our left sided pressures are

supposed to be higher than our right

side of pressures we know that left

ventricular pressure should be higher

than right sided left atrial pressure

should be higher than the right atrial

pressure aorta should be higher than the

pulmonary artery we this is something

that we know

but if under a long period of time you

cause that peripheral vascular

resistance to increase so much

the right sided pressures can actually

start overcoming the left sided

pressures and when that happens

you now reverse the shunt

if the right atrial pressure is higher

you start shunting blood from the right

atrium to the left atrium that's putting

deoxygenated blood represented by blue

into the

oxygenated chambers of the heart that

can lead to cyanosis

i push blood from the right ventricle

into the

left ventricle i'm pushing deoxygenated

blood into the oxygenated chambers

if the pressure in the pulmonary artery

is high enough it can shunt blood into

the aorta that's pushing deoxygenated

blood into the oxygenated vessels

what happens now is now i'm pushing all

of this deoxygenated blood into the

oxygenated chambers of the heart the

left atrium left ventricular aorta now

the actual arterial system is getting

less oxygenated blood because of this

right to left shunting

when this happens when you go from a

left to right shunt

and you switch it over to a right to

left shunt that causes cyanosis

this is called eisenmanger syndrome and

it's because you put so much blood flow

into the pulmonary vessels it had to

respond vasoconstrict thicken the walls

increasing resistance causing the

pressures on the right side of the heart

to overcome the left pressures and now

switch and reverse the shunt right

atrium to left atrium right ventricle to

left ventricle and pulmonary artery into

the aorta and now we push deoxygenated

blood into the oxygenated circuits when

that happens you start to see this

effect of cyanosis so what can this look

like now i got a mixture of oxygenated

blood and deoxygenated blood moving into

my systemic vasculature one of the big things

things

as it presents with the cyanotic

findings bluish discoloration of the

skin the lips the oral mucosa sometimes

the sclera

all over the body and these are the big

big concerning findings especially like

central cyanosis

the next thing is over a long period of

time more of a chronic finding the nail

beds can actually become huge your

fingers can look like spoons and this is

called clubbing of the digits

the other thing is if you put a pulse

oximeter on you'll notice that their

pulse ox the saturation of oxygen is

much lower because we have a mixture of

deoxygenated oxygen in blood going

through the systemic vasculature

the other thing is whenever you have low

oxygen levels within the blood the

kidneys do not like that and the kidneys

respond and say hey

if there's less oxygen maybe it's a

problem because i have less red blood

cells but that isn't the issue it's the

problem with the heart you know what it

does the kidney gets stimulated and says

i'm going to release erythropoietin ipo

ipo tells the bone marrow make more red

blood cells because if we have more red

blood cells we can pick up more oxygen

deliver more oxygen to the tissues but

that is not the issue it's the problem

with the actual oxygen

being mixed between the chambers of the heart

heart

but nonetheless this will happen and

you'll try to make more red blood cells

so one of the findings of these babies

is that they will high numbers of red

cells within their hematocrit and this

is called polycythemia these are the big

big findings that i need you guys to

remember for eisenmanger syndrome which

is the reversal of the left to right shunt

shunt

now becoming a right to left shunt

okay let's move into the next thing if

we go through these and we talk about

these are all of the findings right that

we talked about that can happen with the

left to right shunts okay

let's talk about some of these left

right shunts and some more specific

clinical features that you have to know

for your exam

for the asd when you're actually taking

the stethoscope listening to the chest

what are some things that you can find

so we know we have an s1 which is the

closure of the tricuspid and the mitral

valve and then you have the s2 which is

the closure of the aortic valve and the

pulmonary valve thus represented by a2

and p2 it's the second heart sound

composed of aortic and pulmonary valve closure

closure

when someone has an asd they have three

particular findings one is they have a

systolic ejection remember what does

that do to think about this really

really simply you have an asd you're

pushing blood we haven't had eisenmanger syndrome

syndrome

we push blood from the left atrium into

the right atrium you fill the right

ventricle more

if you fill the right ventricle more you

pump more blood

into the pulmonary vasculature

if you pump more blood into the

pulmonary vasculature you're having a

higher velocity of blood flow through

the flowing through the pulmonary

vessels that's going to cause

turbulence of blood flow that

precipitates a murmur and that is the

systolic ejection murmur okay it's due

to high pulmonary flow

the other thing is you develop a fixed

split s2 that's basically saying that if

you have more of blood that's going from

the left atrium to the right atrium

right atrium down to the right ventricle

now the right ventricle has more blood

than usual it already takes a decent

amount of time for it to get all the

blood out into the pulmonary vessels and

then close the pulmonary valve it

usually always closes after the aortic

valve but now you fill it with more blood

blood

and if you fill it with more blood now

it's got to pump all that blood out

which is going to take longer and then

it's going to take longer for the valve

to actually close even more so now it's

splitting the actual s2 the p2 component

becomes even further away from the a2

but here's the thing when we say fixed

it doesn't change during inspiration or

expiration it stays the exact same okay

that is the big thing to remember for

this one the last thing is you get this

diastolic rumble

and the whole point here is that as i'm

pushing lots of blood from the left

atrium into the right atrium i'm having

more blood flow

across the tricuspid valve if i have

more blood flowing across the tricuspid

valve from the right atrium into the

into the right ventricle that causes a

rumbling sound as it moves across the

tricuspid valve and that is called the

diastolic rumble and we hear the

tricuspid valve at that left lower

sternal border and so that's why we have

that one boom roasted vsd for the vsd

you have again s1 closure of the

tricuspid mitral and then s2 is the two

components here closure of the aortic

valve and the pulmonary valve

in this situation you have a whole low

systolic murmur that means that you can

hear the murmur the entire time real

quickly though actually before we come

back to this there's another thing here

with asd which is important to remember

there's a separate complication we

talked about a little bit before but you

can see this with particularly more

specifically pfos

rather than asds but i thought i'd

mention it here because sometimes you

can see this with really large asds or

if someone has a dvt

and they actually break off a piece of

that dvt it comes up the inferior vena

cava into the right atrium it can

actually quickly jump over from the

right atrium to the left atrium left

atrium into the left ventricle and you

can pump that up into the systemic

circulation up to the brain and cause

strokes you see this primarily with pfos

but with very large asds sometimes you

can see this okay

back to the vsd with this one again you

have holosystolic murmurs what is a

whole solid membrane means you hear the

murmur throughout the entire time period

of systole all this is is in vsds you're

having a lot of blood flowing

from the left ventricle into the right

ventricle and it's going to be bouncing

off of the

walls of the right ventricle that

creates a turbulence of blood flow that

turbulence creates a murmur and this

happens because when the left ventricle

contracts it generates more pressure to

push blood into the right ventricle and

so that's how you get the whole systolic

murmur it's important to remember though

the smaller the defect the louder the

murmur because we know that small

diameter causes more velocity more

velocity causes more turbulence more

turbulence makes the the murmur louder

so large vsds softer smaller vsds louder

remember that okay so hold the stock

murmur is due to the vsd then we have a

loud s2

think about this if i'm pushing a lot of

blood into the

right ventricle i'm pushing a lot of

blood into the pulmonary arteries the

pulmonary artery pressure is going to be

much higher whenever the pressure in the

arteries are higher it snaps those

pulmonary valves shut really really hard

and so it shuts them hard and it

actually causes the p2 component the

closure of the pulmonary valve to be a

lot louder than it usually is and that's

why we get a loud s2 but particularly

the p2 component is what's the actual

component that's actually causing that

loud s2 because of the high pulmonary

pressures because we're pushing a lot of

blood into the right side of the heart

pushing a lot of blood into the

pulmonary arteries increasing the

pulmonary artery pressures which is

shutting the pulmonary valve closed

really hard

the last thing is you have a diastolic

rumble so you're like oh okay that's

interesting why is this happening

here's why this one's interesting you're

pushing a lot of blood from the left

ventricle into the right ventricle

a lot of blood into the pulmonary

arteries a lot of blood from the

pulmonary arteries goes back into the

pulmonary veins

from the pulmonary veins they all empty

into the left atrium the left atrium is

going to have a ton of blood flowing

across the mitral valve if you have a

ton of blood flowing across the mitral

valve during diastole it's going to

cause the rumble but the rumble will not

be at the left lower sternal border it

will be at the apex where you actually

have the mitral valve pretty cool right

all right so that's the different

components of the vsd let's now come

down and talk about pda into cardio

cushion defect and then finish this off

with coarctation of the aorta as kind of

its own separate entity all right so the

next one is patent ductus arteriosus so

what happens with this one the real big

thing here to remember is as we kind of

like seeing here systolic ejection

murmurs the big things fixed split s2

diastolic rumble for that one holy

system loud s2 diastolic rumble at the apex

apex

for pda it's really the easiest one to

remember it's a continuous murmur so you

hear that throughout systole and

throughout diastole so if you look at it

it actually has more of a crescendo type

of fashion that you hear because as

you're having a lot of blood going into

the aorta a lot of it's shunting into

the pulmonary artery so it's going to be

having a lot of blood moving through

through that actual uh ductus arteriosus

the pain ductus arteriosus but it's

still going to be moving through

during diastole and so you have this

continuous machinery sounding or machine

like murmur that's the big telltale sign

buzzword term that you have to remember

now here's let's understand why is it

continuous okay

we know that the left sided pressures

are higher than the right side of

pressure but maybe what you didn't think

about is that during systole the aortic

pressure is still higher than the

pulmonary pressure during diastole the

aortic pressure is still higher than the

pulmonary pressure and i think that is

what's really interesting is that the

aortic pressure is always higher than

the pulmonary pressure regardless of

when the heart is in systole or when

it's in diastole so because of that

blood always flows from high pressure to

low pressure so in this time guess

what's happening blood is shunting where

from the aorta into the pulmonary during

systole during diastole blood is

shunting into the pulmonary artery

during diastole and that's why you get

that continuous murmurs because the

pressures are always higher in the aorta

than in the pulmonary during systole and

diastole pretty darn cool okay the other

thing here is again think about that pda here

here

another important finding is called wide

pulse pressures so imagine here the left

ventricle pumps a lot of blood into the

aorta right so when you pump a lot of

the blood into the aorta and then

actually during the systolic process you

get this point here this is your

systolic blood pressure this peak point

here okay

then what happens is blood flows into

the aorta

you go through a relaxation period

when the actual the heart goes into a

relaxation period

the actual pressure in the order should

drop down to about 80 and we're going to

call this the diastolic blood pressure

okay but remember what did i tell you

the diastolic pressure in the aorta is

always going to be higher than the

pressure within the pulmonary vessels

and so blood will actually shunt

into the pulmonary arteries

a decent amount during diastole

and what happens is because it's

shunting into the pulmonary arteries a

lot during diastole your diastolic

pressure drops pretty low and your

systolic pressure stays high

and so in these patients they have these

wide pulse pressure findings high

systolic blood pressure and a low

diastolic blood pressure because during

diastole the aorta's shunting a lot of

blood from the aorta into the pulmonary

artery and that's dropping your

diastolic pressure in the aorta because

you're shunting it into the pulmonary

artery therefore causing the difference

between the systolic and the diastolic

to be extremely large or wide in this

case that's one of the big things to

think about okay endocardial cushion

defect is a super random one it's

relatively easy to remember this one you

want to know why because it has all the

findings of an asd all the findings of a

vsd so because of that you would have a

holosystolic murmur at the left lower

sternal border that's because of the vsd okay

okay

you would also have a holosystolic

murmur at the apex this is why it's different

different

remember i told you that in these

patients they don't have great complete

valves so you see how we're missing that

one leaflet of the mitral valve like the

anterior leaflet of the mitral valve

because of that it's easy to pump blood

right into the left atrium

and because of that you're going to push

a lot of blood into the left atrium

during systole it can cause a

holosystolic murmur that is also present

at the apex and this is mitral

regurgitation that's a big one to

remember and then you get the systolic

ejection murmur here because again

you're pushing a lot of blood in from the

the

right left atrium into the right atrium

right atrium down to the right ventricle

or you're pushing a lot of blood via the vsd

vsd

into the

left ventricle to the right ventricle

you're filling up the right ventricle

pumping a lot of blood into the

pulmonary circulation if you're pumping

a lot of blood into the pulmonary

circulation high velocity is going to

cause turbulence of blood flow and cause

a systolic ejection murmur because

you're pushing a lot of volume of blood

into the right side of the heart

therefore you should get a systolic

ejection murmur

don't forget that

so all of these can be particular

findings that you would see during

systole and again because you have an

asd you're going to be pumping blood

from the

right side of the from the left side of

the heart into the right side of the

heart you're overflowing the right side

of the heart if you overflow the right

side of the heart it's going to cause it

more time for it to be able to contract

and push all the blood out into the

pulmonary artery which is going to cause

the pulmonic valve to close later when

it closes later it splits the actual

second heart sound and the p2 comes way

later than the a2 and because it does

not change during inspiration or

expiration it is fixed so again you can

see a lot of the findings of a vsd

and an asd with the only kind of

additional thing here is you can also

see the holosystolic mermaid the apex

and that's because you don't have a

well-formed mitral valve leaflet and you

can blow blood back into the left atrium

so these are big things to think about

for the endocardial cushion defect

let's go over here to the last one here

which is for all of this part here for

the coarctation of the aorta when we

talk about the coarctation remember i

told you there's two types there's the

pre-ductal that's going to be this this

diagram that we're going to talk about

and then there is the postductal this is

the one we're going to talk about here

what happens is with a preductal type of

situation here a preductal coarctation

what does that mean that means that the

constriction ring has to be

in front of or before that little pink tube

tube

the pda the ductus arteriosus the

ligamentum arteriosum right

so because of that imagine here what

happens you have the left ventricle it's

gonna pump blood into the aorta and

you're gonna have all this oxygenated

blood going to the right side of the

body to the head

going up to the head

maybe even getting into the left arm but

then oh

i'm hitting this little constriction

ring where i can barely get any blood to

squeeze through that tiny little

constriction ring and then i'm going to

have very little blood that's going to

be going out to my lower extremities so

a lot of

a lot of blood going into my upper

extremities head neck all of that but

less blood flow going into my lower extremities

extremities

guess what

if the baby has a pda open

guess what happens blood from the right atrium

atrium

goes to the right ventricle from the

right ventricle to the pulmonary artery

think about this i know this may seem a

little odd

if i have very little blood going into

this aorta what do you think the

pressure is in that little segment there

right after the constriction ring do you

think it's high or low i'm not getting a

lot of blood in that area so it's going

to be low but the pressure in front of

that constriction ring holy mama it's

going to be super high i'm going to have

very high pressure in front of the

constriction ring below pressure after

the constriction ring so if the pressure

in that little segment there is low

it's going to be easy for blood to flow

from the

pulmonary artery via the pda

into the aorta

aorta

and then now i'm bringing more blood

flow down into the lower extremities but

i'm bringing a mixture of decreased

oxygen to blood

in normal oxygenated blood there's a

mixture there there's a mixing of blood

and because of that my upper extremities

will get normal oxygen to blood there's

no shunting of this prior to that

constriction ring so all oxygenated

blood will go up to my head

and my arms thus represented here on the

spiderman looking dude with the red

blood all oxygenated blood to my upper

extremities and my head and neck

but then after the constriction ring all

of this blood now from this part of the

constriction ring afterwards and down is

getting a mixture of oxygenated and

deoxygenated blood so now my

torso and my lower extremities are

getting way less blood that is actually

completely oxygen and now it's mixed

and because of that we call this

differential cyanosis and this is

something that you can see in a

infantile or preductal type of

coartation of the aorta is they can have

differential cyanosis sinuses of the

lower extremities and kind of the torso

and then normal oxygenation no cyanosis

or bluish discoloration of the upper

extremities head and neck and that is

important to remember

the other thing that can happen here is

because you have this little

constriction ring imagine i'm trying to

push blood out of the left ventricle

into the order and through that tiny

little constriction ring if i try to

squeeze blood through that tiny little

constriction ring

small diameter high velocity high

velocity causes turbulence of blood flow

that's going to cause a murmur and it's

going to be during systole and guess

what it's a systolic ejection murmur and

when you hear it it's because it's

squeezing through that tiny little

stenotic area and the textbooks say that

this is a commonly is heard on the left

posterior hemithorax and again think

about it because you're squeezing blood

to the tiny little constriction ring all

right so the next part here is the

postdoctoral coefficient of the order so

again this is what you're going to see

in the adult type so what i really want

you guys to think about here is

postductal what does that mean so if we

think about it it's where the

constriction ring is with respect to the

ligamentum arteriosum or the ductus

arteriosus it has to come after so

there's our little constriction ring

what is the problem with this one so the

problem with this one is here's our left

ventricle right it pumps blood into the

aorta from the aorta it's able to pump a

good chunk of blood into the

vessels off the aortic arch

that go to the upper body so to the head

the neck the upper extremities all of

those are getting well perfused they're

getting a good chunk of blood flow so

because of that their actual pressures

in the upper body tend to be very very

high because this constriction ring is

narrowing the amount of blood that can

get into the lower extremities and so

the pressure just proximal to that

little constriction ring is going to be

crazy high and then the amount of blood

that's getting down into the lower

extremities you're not getting a lot of

blood because i got to squeeze the tiny

little constriction ring and that's

gonna drop the pressures that are going

down and perfusing the lower part of the

body so there's very high blood pressure

and perfusions to the upper body and

very low blood pressure and low

perfusion to the lower body now why is

that a problem

think about it if you have very high

pressures that are going to be going up

into the brain it can cause headaches it

can cause

an actual abnormal ringing sensation of

the ears called tinnitus on top of that

it can actually cause ballooning of the

vessels within the brain and these are

called bary aneurysms and if they

rupture they can increase the risk of

like intracranial hemorrhages and so

these are really really important things

to think about because of the high

pressure in the upper body the other

thing is if you have a high pressure in

the aorta now the left ventricle has to

work really hard to push a lot of blood

into that high pressure aorta so it has

to get jacked up it has to get thickened

what does that call when it has to get

thickened and so it can pump harder and

push more blood into the aorta that's

called left ventricular hypertrophy the

other thing is because all the pressure

in the aorta is super super high they're

going to have high blood pressure if you

take the actual blood pressure and a

cuff and actually put these on these

arms they're going to have super high

blood pressures so they're going to have

hypertension but the big thing here is

because of the high pressures in the

aorta and causing the left ventricle

hypertrophy it's going to cause so much

stress hard work hard demand of the

actual left ventricle that eventually it

won't be able to maintain it and it will

lead to left heart failure so that's a

big thing to think about now

the other thing is if we have low blood

pressure we're not perfusing the lower

part of the body we're not getting

oxygen we're not getting blood flow to

the lower extremity muscles they're

going to become very painful it's going

to cause them to not be able to perform

their normal contractions especially

when we're trying to exert ourselves

when we exert ourselves we utilize

oxygen within the muscles and if we have

very little supply to those muscles

because of the decreased profusion do

that dang constriction ring guess what

those muscles aren't going to get the

amount of oxygen that they need and they

demand during exertion and so this can

cause a pain in the lower extremities

called claudication also if you check

the pulses of their lower extremities

they'll have very decreased pulses and

sometimes pale and cold extremities of

the lower part of the body so that's a

big thing that's important to remember

another big telltale sign that you'll

probably see on your exams is that if

you check the blood pressures of their

upper extremities they're going to be high

high

and then if you check the blood

pressures of their lower extremities

they're going to be low

relatively significant difference here

so sometimes we call that a brachial

femoral delay the other thing is if you

check the break-in for more delay is

also very specific to the pulses if you

check the radial pulse and then you

check the actual like femoral pulse

and you look at the pulse difference

there the brachial pulse is going to be

very bounding and high and and more

rapid and then the femoral pulse which

is supposed to come early you're

actually supposed to be able to feel the

femoral pulse before you feel like the

radial or brachial pulse in these

patients the femoral pulse comes later

which is not a normal finding so if the

femoral pulse comes later after the

brachial radial pulse that means that

there's some type of delay there and

that's a brachial femoral delay so again

seeing a big pressure difference from

upper extremities lower extremities or

delayed femoral pulse coming way later

than the actual brachial pulse that is a

huge difference and a big thing to

remember here okay so that covers all of

the clinical pictures for these uh a

cyanotic defects let's now hit the

diagnosis all right so let's talk about

the diagnosis of these a cyanotic and

genital heart defects so one of the big

things to remember is you're gonna have

three particular tests an ekg a chest

x-ray and echo to be honest with you you

could potentially not even remember all

of the ekg and chest x-ray findings that

is completely fine

i think the biggest most important

definitively diagnostic test is going to

be the echo you could actually have an

even better test in that which would be

cardiac catheterization but we don't

generally do those anymore they're you

know invasive and they're high risk and

they don't actually give us as much

diagnostic info as we could potentially

just again obtain with a non-invasive

study like an echocardiogram so big

thing to remember here you can get a

chest x-ray you can get an ekg they may

be somewhat helpful in the diagnosis but

again the big big most important test

here is going to be the echocardiogram

it's the best test it's the diagnostic test

test okay

okay

for ekg chest x-ray echo we're going to

go through each one of the actual async

defects and try to pick these out so

first thing here for the ekg

on asd you're overloading remember

you're pushing blood from the left

atrium into the right atrium you're

pushing blood into the right ventricle

it's going to cause the right ventricle

to have more blood when it gets more

blood it's going to have to try to be

able to accommodate so it tries to

undergo hypertrophy when it goes

hypertrophy it tries to make itself a

little bit thicker a little bit bigger

and because of that you can actually see

patients with right ventricular

hypertrophy because they're getting

thicker right atrial enlargement because

you're pushing more volume of blood into

the right atrium and sometimes as you

make the right ventricle thicker and

bigger it yanks and stretches on those

right bundle branches and it can

actually cause a blockage of the right

bundle branches so that's the big things

to see here potentially for the ekg

for the chest x-ray you're pushing a lot

of blood from the right side of the

heart into the pulmonary vessels you're

filling these bad boys and for the most

part for almost all the left to right

shunts asd vsd endocardial cushion

defect and pda what do you think is

going to be the common theme for all of

these on their chest x-ray

heavy pulmonary vasculature because

you're pushing a lot of blood into them

that's going to be the big thing that

you'll see with that one

and again because you are pushing a lot

of blood from the left atrium to the

right atrium you could make the right

atrial border a little bit larger on

asds all right but the big thing is the

echo and if you do the echo and you put

on the color doppler you'll be able to

see the shunt of blood going from the

left atrium into the right atrium for

the vsd again think about it your left

ventricle it's going to be pushing a lot

of blood from the left ventricle into

the right ventricle it could potentially

thicken the right ventricle over time if

it has to work really really really hard

because the pulmonary vessels are

getting overfilled they're undergoing

reactive vasoconstriction they're

thickening their walls they're making

the right ventricle have to hypertrophy

and if it does hypertrophy that's

indicative eisenmanger syndrome but the

more common findings is you're pushing a

lot of blood via the pulmonary arteries

into the pulmonary veins pushing a lot

of blood from the left atrium to the

left ventricle causing the left

ventricle to have to accommodate a large

volume of blood and have to get thicker

okay so it undergoes left ventricular

hypertrophy you're also bringing a lot

of blood into the left atrium so you're

going to cause the left atrium to

enlarge and so these will be the

particular findings that you would see

on the ekg for the chest x-ray you're

going to see increased vasculature and

you may see enlargement of the left side

of the heart so cardiomegaly

but again put the doppler on look at the

blood flow and look at the shunt going

from the left ventricle to the right

ventricle and you got your diagnosis pda

you may or may not see left ventricular

hypertrophy oftentimes their ekg is

relatively normal so that's one of the

big things to think about is that you

may not really see anything very helpful

on their ekg on their chest x-ray though

you'll see a lot of pulmonary

vasculature and again no right atrial

enlargement or anything of that nature

but again put the actual color doppler

on and you'll be able to see the

shunting of blood from the

aorta into the pulmonary artery okay

endocardial cushion defect this one's

actually really interesting it's the

only one that's just super random no

complete explanation it may be due to

the actual defect within the endocardial

cushions and where the av node is around

those endocardial cushions but these

patients tend to have a prolonged pr

interval and again i would say it's due

to some type of defect within the

development potentially the av node

there but again they tend to have

prolonged pr intervals on their actual

ekg and again you're pushing a lot of

blood from there left ventricle to the

right ventricle you're pushing a lot of

blood from there

uh left atrium into their right atrium a

lot of blood from their left ventricle

into the right ventricle you're

overflowing the right ventricle with

pressure and volume it's going to

undergo hypertrophy and thickening

therefore you may see right ventricular

pertrophy but again big thing here

that's just pretty much an out of the

ordinary one out of all compared to these

these

is that prolonged pr interval for the

endocardial cushion defect so again i

think the big thing here is that the

right side of the heart sent the right

atrium right ventricular hypertrophy for

asd vsd left atrium enlargement left

ventricular hypertrophy pda relatively

normal and then endocardial cushion

prolonged pr all of these have an

increase in pulmonary vasculature but

the most definitive diagnostic test is

see the shunting of blood from the left

atrium to the right atrium left

ventricle to the right ventricle aorta

to the pulmonary artery or looking for

any type of shunt of a vsd an asd and

abnormal tricuspid and mitral valve

abnormalities on there echo would tell

you that you have an endocardial cushion

defect okay let's come down to the

co-operation of the aorta all right so

the next thing here is coordination of

the order right so think about this one

for ekg because you are having higher

pressures potentially

inside of the aorta it actually could

cause the left ventricle to hypertrophy

so you may or may not see left

ventricular hypertrophy if it's more of

like the

adolescence adult type you probably will

see left ventricular hypertrophy but for

more of the actual infant tile types you

you probably won't see anything super

obvious but again i think one of the big

things here that actually differentiates

these from all the other chest x-rays is

that there's nothing to do with the

increased pulmonary vasculature

for this one you see something very

interesting so you see due to the

constriction ring here when you look at

the chest x-ray because of that kind of

constriction ring it gives like this little

little little

little

three sign if you will and so the little

three sign could actually be due to the

constriction ring that you can see from

the co-arctic aorta so that's one

particular finding that you can see in

the chest x-ray the next one here is

something very interesting whenever you

have somebody who has a quotation of the

aorta they're actually not perfusing the

distal parts of the aorta very well

which don't perfuse the intercostal

arteries as well and so because of that

you actually get like a diversion of

blood flow from like the internal

thoracic artery and some other branches

coming off of the

parts of the aortic arch and so it

sometimes it can cause the pulsations of

some of the intercostal arteries and so

this can actually kind of chew away at

the ribs and so sometimes you can get

something called rib notching and this

is just again the whole basic concept

here is that because you get decreased

perfusion of the descending aorta you

don't perfuse some of the actual

branches of the intercostal arteries

coming off of that and so the branches

that come off of the other components of

the aortic arch they have to feed those

intercostal arteries and then it starts

causing the pulsations of them and

starts chewing away at the actual

underlying portion of the rib causing

rib notching so you can see that with

coarcted aorta

but again easy definable thing here is

you can get a

echo and look to see if you can see a very

very

obvious identifiable stenotic lesion

another thing that's really important

here is sometimes these patients are

actually have a very high association

with a bicuspid aortic valve as well

okay so that would be the co-octation of

the aorta that would finish that one off

the last thing here that we should add

for these patients with a cyanotic

congenital heart defects as well as

doing this as just a newborn screening

for cyanotic congenital heart defects so

we're covering it here now but again

this can apply to the same thing when we

talk about cyanotic congenital heart

defects is we do something called pulse

oxymetry testing when we do pulse

oximetry testing we should also do

something called a pre-ductal and

post-ductal oximetry testing what we do

is we take the pulse oximeter and we'll

put it on the actual right hand and

we'll put it on the right foot and what

we're looking for is we're looking to

see if there's a very large difference

in the actual o2 saturation between the

right foot and the right hand if there

is a really big difference that tells us

that there's some type of potential

shunt going on here that's actually

causing this cyanotic defect and so

that's a big thing to remember is that

we use this as a part of a newborn

screening tool again we're comparing the

pre-ductal the right hand and the

postdoctoral right foot and it just

helps us to really kind of identify

where the mixing could potentially take

place and when we need to go and

actually get an echocardiogram of these

patients so again newborn screening they

automatically get pulse oximetry right

hand right foot if there's a large

difference sometimes like greater than

10 percent we should follow these

patients up with an actual

echocardiogram okay

now that we've done that let's talk

about treatment all right so let's talk

about the treatment of these asymmetric

defects so it's actually relatively

straightforward not too complicated to

be honest with you if they're small

defects especially like asds like if

they're really small defects we

typically don't do anything with them

because they sometimes will

close spontaneously but if they're

really large and they're actually

causing symptoms of like heart failure

like we talked about so they're having

right heart failure they're having some

of the concerning signs of maybe

decreased left ventricular cardiac

output all of those concerning potential signs of left heart failure right heart

signs of left heart failure right heart failure then we actually should actually

failure then we actually should actually go in and repair that so if they're very

go in and repair that so if they're very large defects sometimes like greater

large defects sometimes like greater than one to two centimeters or they're

than one to two centimeters or they're causing symptoms then we should actually

causing symptoms then we should actually go in and surgically repair that or

go in and surgically repair that or sometimes we actually put in we'll do

sometimes we actually put in we'll do like a transcatheter closure take a

like a transcatheter closure take a catheter go on the one inside and then

catheter go on the one inside and then just pull and it creates like a little

just pull and it creates like a little patch pretty cool

patch pretty cool vsd same thing if it's like small

vsd same thing if it's like small they're not really causing any problems

they're not really causing any problems they're asymptomatic they sometimes will

they're asymptomatic they sometimes will close spontaneously and remember small

close spontaneously and remember small vsds loud murmurs large vsds very kind

vsds loud murmurs large vsds very kind of soft members but if they have a large

of soft members but if they have a large vsd and they're actually having symptoms

vsd and they're actually having symptoms you should surgically or transcatheter

you should surgically or transcatheter close that one the other thing is that

close that one the other thing is that sometimes vsds are more likely to

sometimes vsds are more likely to develop some of the actual chf symptoms

develop some of the actual chf symptoms because not only are you pushing a large

because not only are you pushing a large volume blood from the left ventricle to

volume blood from the left ventricle to the right ventricle you're pushing a

the right ventricle you're pushing a large pressure from the left ventricle

large pressure from the left ventricle to the right ventricle and so sometimes

to the right ventricle and so sometimes these patients can definitely develop

these patients can definitely develop left-sided heart failure and right-sided

left-sided heart failure and right-sided heart failure and so you might need to

heart failure and so you might need to medically treat them in the entire until

medically treat them in the entire until you can get that surgical closure

you can get that surgical closure and so we would try to maybe do

and so we would try to maybe do diuretics to pull off some of the fluid

diuretics to pull off some of the fluid if they're developing a lot of pulmonary

if they're developing a lot of pulmonary edema or a lot of actual uh hepatomegaly

edema or a lot of actual uh hepatomegaly we would actually do afterload reducers

we would actually do afterload reducers so that we can try to push a lot of

so that we can try to push a lot of blood out into the systemic circulation

blood out into the systemic circulation and maintain a good cardiac output and

and maintain a good cardiac output and sometimes inotrope so we can get that

sometimes inotrope so we can get that left ventricle to squeeze as much blood

left ventricle to squeeze as much blood out of the left ventricle and into the

out of the left ventricle and into the aorta because we just don't want it

aorta because we just don't want it shifting over into the right side of the

shifting over into the right side of the heart

heart so this would be the big things to do

so this would be the big things to do again endocardial cushion defects these

again endocardial cushion defects these ones they don't actually survive very

ones they don't actually survive very well and all these patients you can't

well and all these patients you can't wait you just need to close them it

wait you just need to close them it doesn't matter if it's small if it's

doesn't matter if it's small if it's large that these patients need these

large that these patients need these surgically fixed so endocardial cushion

surgically fixed so endocardial cushion defect it doesn't matter they have to

defect it doesn't matter they have to get surgically fixed for asds and vsds

get surgically fixed for asds and vsds if they're small asymptomatic they close

if they're small asymptomatic they close spontaneously if they're large you

spontaneously if they're large you surgically transcatheted close them but

surgically transcatheted close them but for ecds endocardia cushion defects

for ecds endocardia cushion defects surgery in all forms it's important to

surgery in all forms it's important to remember that and again because you can

remember that and again because you can have large vsds asds and valve

have large vsds asds and valve abnormalities these are patients are

abnormalities these are patients are high risk of left and right sided heart

high risk of left and right sided heart failure so again pull fluids off if they

failure so again pull fluids off if they have pulmonary edema try to be able to

have pulmonary edema try to be able to reduce the afterload of the aorta so

reduce the afterload of the aorta so that you can push blood in the left

that you can push blood in the left ventricle into the aorta and not into

ventricle into the aorta and not into the right side of the heart

the right side of the heart and again ionotropes that just maintain

and again ionotropes that just maintain a good squeeze of that left ventricle

a good squeeze of that left ventricle for the other one which is the patent

for the other one which is the patent ductus arteriosus the pda

ductus arteriosus the pda in that situation we want to maybe leave

in that situation we want to maybe leave it alone if it's a small one if it's a

it alone if it's a small one if it's a very small defect not having any kind of

very small defect not having any kind of symptoms of heart failure then okay we

symptoms of heart failure then okay we can leave it alone it'll price close

can leave it alone it'll price close spontaneously but for the very large

spontaneously but for the very large defects or potentially symptomatic or in

defects or potentially symptomatic or in pre-term infants that are having

pre-term infants that are having symptoms and issues we actually should

symptoms and issues we actually should close this and how can we close this

close this and how can we close this really interesting so there is a

really interesting so there is a molecule called prostaglandin e1 and

molecule called prostaglandin e1 and what prostaglandin e1 does is it

what prostaglandin e1 does is it actually keeps the duct open okay so it

actually keeps the duct open okay so it keeps the duct open

keeps the duct open in these pre-term infants where we

in these pre-term infants where we actually want to close that duct we

actually want to close that duct we don't want to keep it open anymore

don't want to keep it open anymore we can actually give them drugs like

we can actually give them drugs like indomethacin and ibuprofen you want to

indomethacin and ibuprofen you want to know why

know why you know what happens is arachidonic

you know what happens is arachidonic acid you it by an enzyme called

acid you it by an enzyme called cyclooxygenase cox

cyclooxygenase cox works to convert arachidonic acid into

works to convert arachidonic acid into prostaglandin e1 and prostaglandin e1

prostaglandin e1 and prostaglandin e1 keeps the actual duct open

keeps the actual duct open if we give somebody endomethacin and

if we give somebody endomethacin and ibuprofen that inhibits the

ibuprofen that inhibits the cyclooxygenase enzyme they cannot

cyclooxygenase enzyme they cannot convert arachidonic acid into

convert arachidonic acid into prostaglandin e1 prostaglandin e1 levels

prostaglandin e1 prostaglandin e1 levels fall if they fall they can't keep the

fall if they fall they can't keep the pda open

pda open and it closes that's a pretty cool

and it closes that's a pretty cool mechanism right so that's the concept

mechanism right so that's the concept here that we would use in these pre-term

here that we would use in these pre-term infants is that if they're large or

infants is that if they're large or they're having any symptoms we can start

they're having any symptoms we can start them off with endometriosis and

them off with endometriosis and ibuprofen and then eventually surgically

ibuprofen and then eventually surgically close it okay so pda if it's small

close it okay so pda if it's small asymptomatic leave it alone likely will

asymptomatic leave it alone likely will close spontaneously if it's large or

close spontaneously if it's large or they're having symptoms and pre-term

they're having symptoms and pre-term infants we can do endomethane ibuprofen

infants we can do endomethane ibuprofen to inhibit the cycle oxygenates drop the

to inhibit the cycle oxygenates drop the prostaglandin one and start trying to

prostaglandin one and start trying to close it and then if that doesn't work

close it and then if that doesn't work maybe six to 12 months later we'll

maybe six to 12 months later we'll surgically close that

surgically close that okay there's a really really important

okay there's a really really important point here that we have to talk about

point here that we have to talk about sometimes

sometimes pdas can keep certain patients alive

pdas can keep certain patients alive that may seem super weird but it's

that may seem super weird but it's important in certain types of cyanotic

important in certain types of cyanotic congenital heart disease we actually

congenital heart disease we actually want to keep the pda open because it

want to keep the pda open because it might be the only thing that's keeping

might be the only thing that's keeping the baby alive because it's helping with

the baby alive because it's helping with mixing of blood we'll make more sense of

mixing of blood we'll make more sense of this when we get into the cyanotic

this when we get into the cyanotic congenital heart disease lecture but

congenital heart disease lecture but what i want you to remember for right

what i want you to remember for right now to just trust me is that we would

now to just trust me is that we would keep the pda open by increasing that

keep the pda open by increasing that prostaglandin e1 level so prostaglandin

prostaglandin e1 level so prostaglandin e1 level keeps the pda open give them

e1 level keeps the pda open give them infusions of prostaglandin e1 to keep

infusions of prostaglandin e1 to keep that bad boy open

that bad boy open and if you do that you would be keeping

and if you do that you would be keeping it open for certain types of conditions

it open for certain types of conditions and we'll explain these in that lecture

and we'll explain these in that lecture but the conditions i need you to

but the conditions i need you to remember is a coartation of the aorta

remember is a coartation of the aorta because if you remember we talked about

because if you remember we talked about this just a little bit ago the

this just a little bit ago the corotation of the order they're getting

corotation of the order they're getting no blood flow to their lower extremities

no blood flow to their lower extremities if they have a very bad corotation

if they have a very bad corotation you can at least get some blood flow

you can at least get some blood flow into the aorta even though it may be

into the aorta even though it may be deoxygenated you're still getting blood

deoxygenated you're still getting blood flow to the lower extremities and that

flow to the lower extremities and that is important so in a correct aorta we

is important so in a correct aorta we need that another condition called

need that another condition called transposition of the great arteries

transposition of the great arteries another one called hypoplastic left

another one called hypoplastic left heart syndrome another one called total

heart syndrome another one called total anomalous pulmonary venous return and

anomalous pulmonary venous return and tricuspid atresia i know this may seem

tricuspid atresia i know this may seem like very random and like useless

like very random and like useless information and just

information and just bare memorization right now but i

bare memorization right now but i promise we will make sense of it in the

promise we will make sense of it in the congenital heart disease lecture but

congenital heart disease lecture but these are the conditions you have to

these are the conditions you have to keep the pda open because it might be

keep the pda open because it might be the very thing that keeps them alive

the very thing that keeps them alive long enough to get these things

long enough to get these things surgically fixed

surgically fixed okay

okay the last one here for these actual

the last one here for these actual diseases and we'll talk about the

diseases and we'll talk about the complication of how we treat that very

complication of how we treat that very scary eisenmenger syndrome is

scary eisenmenger syndrome is co-octation of the aorta so for the

co-octation of the aorta so for the infantile type what was that one the

infantile type what was that one the pre-duct the constriction ring comes

pre-duct the constriction ring comes before the ductus arteriosus the pda the

before the ductus arteriosus the pda the ligament and arteriosum in those

ligament and arteriosum in those situations what did i tell you

situations what did i tell you keep that dang pda open so you can

keep that dang pda open so you can perfuse their lower extremities even

perfuse their lower extremities even though it's low oxygenated blood you're

though it's low oxygenated blood you're still getting some perfusion we need

still getting some perfusion we need that so you keep the actual pros the pda

that so you keep the actual pros the pda open by giving them prostaglandin e1 so

open by giving them prostaglandin e1 so you're going to give them prostaglandin

you're going to give them prostaglandin e1 levels

e1 levels okay the next thing is we're actually

okay the next thing is we're actually going to surgically resect it so then

going to surgically resect it so then wherever that actual that little

wherever that actual that little constriction ring is we're going to cut

constriction ring is we're going to cut that little constriction ring out and

that little constriction ring out and pull that out and then end to end

pull that out and then end to end anastomosis of those situations okay so

anastomosis of those situations okay so coarctation aorta and the infantile type

coarctation aorta and the infantile type keep the pda open so you can perfuse the

keep the pda open so you can perfuse the lower extremities even though they'll be

lower extremities even though they'll be cyanotic you're still perfusing them and

cyanotic you're still perfusing them and so in those situations give them

so in those situations give them prostaglandin e1 to keep the pda open

prostaglandin e1 to keep the pda open and then surgically resect

and then surgically resect in the adult type

in the adult type we actually can go in

we actually can go in and balloon open we can actually take a

and balloon open we can actually take a balloon we take a catheter and we take

balloon we take a catheter and we take and put a balloon in there and then

and put a balloon in there and then inflate the balloon to stretch open the

inflate the balloon to stretch open the constriction ring and then we'll throw a

constriction ring and then we'll throw a stent in there if we throw a stint in

stent in there if we throw a stint in there you can actually get blood flowing

there you can actually get blood flowing to lower extremities and if that doesn't

to lower extremities and if that doesn't work you can actually again surgically

work you can actually again surgically resect an end to end anastomosis for

resect an end to end anastomosis for that as well so these are the things

that as well so these are the things that we'll do for cortisone but the big

that we'll do for cortisone but the big one i need to remember is for the

one i need to remember is for the infantile type keep the pda open how do

infantile type keep the pda open how do you do that prostaglandin e1 and then

you do that prostaglandin e1 and then you can resect it surgically and then

you can resect it surgically and then for the adult you can either balloon

for the adult you can either balloon stint it and then if that doesn't work

stint it and then if that doesn't work you can also surgically resect it

you can also surgically resect it okay let's talk about the last

okay let's talk about the last particular finding here which is

particular finding here which is eisenminger syndrome that's scary scary

eisenminger syndrome that's scary scary thing that we want to try to treat these

thing that we want to try to treat these before we ever get to this because if

before we ever get to this because if you get to this point all of the

you get to this point all of the surgical procedures are not going to be

surgical procedures are not going to be helpful and the patient als

helpful and the patient als ultimately will need to get a heart

ultimately will need to get a heart transplant so let's talk about that one

transplant so let's talk about that one all right so the last one eisenmanger

all right so the last one eisenmanger syndrome so this is that again that

syndrome so this is that again that scary scary one that you don't want the

scary scary one that you don't want the patients to ever get to because again if

patients to ever get to because again if that right side of pressures overcome

that right side of pressures overcome the left side of pressures now you

the left side of pressures now you reverse the shunt right to the left now

reverse the shunt right to the left now these babies got cyanosis

these babies got cyanosis at this point surgical procedures are

at this point surgical procedures are not going to be helpful medical therapy

not going to be helpful medical therapy won't probably be very helpful as well

won't probably be very helpful as well at this point you need to probably get

at this point you need to probably get the patients a heart and lung transplant

the patients a heart and lung transplant that's super sad right they're little

that's super sad right they're little babies but what can we do in the interim

babies but what can we do in the interim to kind of get them that heart and lung

to kind of get them that heart and lung transplant sometimes what we can try to

transplant sometimes what we can try to do is if you remember what was the

do is if you remember what was the problem with eisenmanger syndrome their

problem with eisenmanger syndrome their pulmonary vascular resistance was son of

pulmonary vascular resistance was son of a gun it was so high right because

a gun it was so high right because they're squeezing and their walls are

they're squeezing and their walls are thickening what if i try to reduce the

thickening what if i try to reduce the pulmonary vascular resistance so i can

pulmonary vascular resistance so i can try to get some kind of like pulmonary

try to get some kind of like pulmonary blood flow through what could i

blood flow through what could i potentially try to help with with that i

potentially try to help with with that i can give these patients pulmonary

can give these patients pulmonary vasodilators and that'll actually be

vasodilators and that'll actually be things like you can get things like

things like you can get things like milranone or like sometimes inhaled

milranone or like sometimes inhaled nitric oxide and i can just relax those

nitric oxide and i can just relax those vessels a little bit so you can actually

vessels a little bit so you can actually get some kind of pulmonary blood flow

get some kind of pulmonary blood flow and that would potentially be somewhat

and that would potentially be somewhat helpful until they get the ultimate

helpful until they get the ultimate treatment which is going to be a heart

treatment which is going to be a heart and lung transplant

and lung transplant ninjas in this video we talk about a

ninjas in this video we talk about a cyanide congenital heart defects in the

cyanide congenital heart defects in the next one we'll cover congenital hope you

next one we'll cover congenital hope you guys enjoyed i hope it made sense and as

guys enjoyed i hope it made sense and as always until next time

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Acyanotic Congenital Heart Defects