Cram The Pance - S1E17 Congenital Heart Disease

Episode Date: March 26, 2021

Atrial Septal Defect, Coarctation Of The Aorta, Patent Ductus Arteriosus, Tetralogy Of Fallot, Ventricular Septal Defect review for your Pance, Panre and Eor’s.►Paypal Donation Link: https://bit.ly/3dxmTql (Thank you!)--- Support this podcast: https://anchor.fm/scott--shapiro/supportBecome a supporter of this podcast: https://www.spreaker.com/podcast/cram-the-pance--5520744/support.

Okay, so what we're going to be covering next is congenital heart diseases. So as far as the NCCA blueprint, the ones they want you to know is atrial septal defect, coerctation of the aorta, patent ductus, arteriosis, tetraliafalo, and ventricular septal defects. So those are the ones we'll go over today. Now, there's a couple of things I wanted to review before you get started, just to make sure that you're familiar with it. So some of these conditions are going to be cyanotic, meaning that you're going to have this non-oxygenated blood back into the body, and some of these are non-sionotic. So really quickly, we'll go over that.

So if you have a cyanotic condition, this is when the shunt or the hole in the heart is pushing blood from the right side of the heart over to the left and then it's getting pumped down to the systemic circulation. Those are going to be your cyanotic conditions. Now the non-sionic are when you have a shunt or a hole that's getting pushed, but in this case it's getting pushed from the left side of the heart to the right. So it doesn't have any the non-oxygenated blood on the left side of the heart pumping out to the systemic circulation. What's important to note is that even a non-cyonotic condition, which has a left to right shunt, can at a certain point, reverse if it's large enough, if it's been untreated for a number of years. So a left-to-right shunt can actually reverse to a right-to-left shunt becoming cyanotic. And this is due to pulmonary hypertension due to it being untreated for so many years

and the pressure reversing and switching it over to a right-to-left shun. This is known as Isommigris syndrome. So that's something that you should be familiar with. and let's go ahead and get started. That was the main thing that I wanted to go over before we get started. So let's start with atrial septal defect. Now, the way you're going to know if the conditions that we're speaking about are psionotic or non-sionic is by looking at the first letter of the defect. So atrial septal defect starts with an A. So it's non-sionic. How do I know that? Well, any condition that starts with a T, so tetralogy of flow, transposition of the great vessels, truncus arteriosis, anything that starts with a T, I want you to think of turquoise. So T for turquoise, and turquoise is like a bluish cyanotic color.

So if you see that the condition starts with a T, it's a cyanotic condition. If it starts with anything else, it's non-sionotic. So it's a really easy way to remember it. And you'll never forget that. So it starts with a Tertrology of flow. It's going to be, think of turquoise, think of cyanotic conditions. So anything that starts with a T is cyanotic, anything other letter, non-sionotic. So atrial septal defect starts with an A.

So this is going to be a non-sionotic condition. But remember, any of these can reverse in time if they're not treated. Eventually, you can have that isomiger syndrome. So it's possible to actually turn into cyanotic. As far as the boards, you need to know this is a non-sionotic condition unless they state otherwise. So atrial septal defect, just like the name implies. This is going to be an abnormal opening in the septum between the right and the left atrium. So there's a defect in the septum of the atrium.

So an opening in the right in the left atrium. And normally this is going to shunt from the left to the right. So a non-cyonotic shunt. As far as types, there's a few different types. only one I want you to remember, the only one that you'll need to remember, is going to be your most common type. And that's known as secundum ASD, so S-E-C-U-N-D-U-M, Secundum-A-S-D, or also known as Osteum-S-U-M-S-U-M. And this is about 70 to 75 percent of all the types of atrial septal defects. So that's really the only one you need to know. There are other types, but they're not going to ask you these. There's one known as

osteum-P-Rominary sinus. There's a few other types. But just remember, osteum-sicundum. That's the most common type. Now, as far as clinical manifestations in all of these conditions, clinical manifestations is not very specific, and it's because it's not very specific, it's not very important for the boards. The money is in the physical exam, which we'll go over. But clinical manifestations for all these are going to be pretty similar. Most atrial septal defects, if they're small, they don't really cause a lot of symptoms in infancy or childhood. If it's large enough, though, children may present with recurrent respiratory infections, failure to thrive, and other symptoms of heart failure. and it's more common for these patients to develop symptoms later in adulthood like palpitations, dysmia, syncope.

It's even possible to develop emboli with atrial septal defect, which can lead to potentially a stroke. So that's something that's important to know. Now on physical exam, this is what you really need to know. This is what they're going to ask you, and this is how you're going to differentiate between these. Now, a physical exam for atrial septal defect, you're going to have a systolic ejection murmur. It's going to be crescendo decrescendo. And it's going to be like a progressive increase and then a. slow decrease, progressive decrease. It's best heard of the pulmonic area, which is going to be

your left upper sternal border. And if you ever forget, like, where's the aortic, where's the pulmonic, where's the mitral, where's the herbs point? Just remember the sentence, all patients eagerly take medicine. All patients eagerly take medicine. And then that goes in order starting at the right upper sternal border. Aortic, pulmonic, herbs point, tric, tricuspid, mitral. So you remember those letters, all patients eagerly take medicine. You'll remember aorta. pylmotic, pulmonic, erbs, tricuspid, and mitral. And you just kind of go along the path there, right upper sternal border, left upper sternal down and across.

So all patients eagerly take medicine, aortic, pulmonic, herbs, tricuspid, and mitral. Okay. So again, this is a systolic ejection murmur, best heard at the pulmonic area. So left upper sternal border. Now, the other thing, even really more important than the murmur that you're going to hear, in these patients, you're going to hear a wide, fixed split S2. This is pathonomonic for atrial septal defect. If you ever see in a vignette, they describe a wide fixed split S2.

You need to be thinking of atrial septal defect 100% unless it's specified otherwise. But that's what you should be thinking of. And just really quickly to kind of understand this. So S2 is normally going to be split. And about 90% of patients you're going to have this split S2. It's a physiologic splitting and it's a normal finding. And like I said, in most patients, about 90% of patients it's a normal finding. So what happens is in S2, the aortic valve with inspiration closes before the pulmonic valve.

And why is this? Well, during inspiration, you have this increase in Venus return to the right side of the heart. So you have increased blood flow to the right side of the heart. What this causes because of this is the pulmonary valve delays its closure. So the aortic valve closes first, then the pulmonic because the pulmonic has all of this influx of extra blood on inspiration. that's normal physiologic splitting. That's what you should here. But an atrial septal defect, due to the abnormal opening between the atrium,

there's no difference in inspiration or expiration. It's always going to be this fixed splitting. So the splitting is never going to alter, whether it's inspiration or expiration. It stays the same. You're going to have this wide fixed split S2. So that's the main thing with atrial septal defect. The way I remember this, it worked for me. I don't know if it'll make sense to you.

But atrial septal defect is the only defect that starts with an A. So that makes me think, all right, what's special about the letter A? So the letter A to me looks like somebody kind of widely splitting open their legs. So if you think of like an A is the bottom part of somebody's legs, it looks like somebody has like a wide stance. And they're doing like a wide split with their legs. So that makes me think of wide splitting. And the other thing too is I think of the letter A.

And then it makes me think of the of an anchor. And an anchor is fixed in position. So that always worked for me. I just think of like a wide splitting fixed anchor. And that, you know, I visualize that with EA. So hopefully that'll work for you. Now, diagnosis. In all of these, you're going to diagnose with an echo.

That's going to be your best test. You don't even need to think about it. Echo is the testing for all of these congenital heart defects. So echocardiography, test of choice for diagnosis. It can detect the presence of an ASD determine its size. You can also do an EKG, chest x-ray, and all of these. It's not really going to be very specific. you may see some cardiomegaly on the x-ray, but really echo is the way to go. Now, as far as treatments,

most patients with a small atrial septal defect are observed because most of the time with a smaller atrial septal defect, less than 5 millimeters, they normally close within the first year of life with no intervention. So they'll spontaneously close within the first year of life. Now, if it's larger than that, if it's over 5 millimeters or the patient becomes symptomatic, they start having issues, then you're going to need to do some form of surgical correction. Now, with all the congenital heart diseases, the definitive treatment is going to be surgery. And don't worry so much about the specific type of surgery. They're not going to ask you about that.

You just need to be aware, definitive treatment for all of these is going to be some form of surgical correction with some medications mixed in there, which I'll go over. But for atrial septal defect, observe if it's small, if it's larger, problematic, symptomatic surgical intervention. Okay, so that's atrial septal defect. Let's move on to the bottom half of the heart, ventricular septal defect. So ventricular septal defect, does it start with a T? It does not. So it's going to be a non-cyonotic condition. Of course, it can reverse an isomygreysmigris syndrome, but that's not what we're talking about today. So this is going to be the defect, a defect in the interventricular septum that leads of shunting of the blood from the left

to the right ventricle. So larger defects, of course, can result in significant left-to-right shunting that can result in pulmonary hypertension, and you can have that isomiger syndrome, which can reverse it. But generally, this is going to be a non-cyonotic left to right shunt. So that's, you have a hole in the, in the ventricles, in the septum there, and blood is being shunted from the left to the right side. Now, important to know, this is the most common congenital heart disease of childhood. So ventricular septal defect, the most common congenital heart disease of childhood. There's a few different types. I really only think you should remember the most common. which is perimenous.

Perrimbrinus, or type 2, it's also known as, is the most common type of ventricular septal defect, about 80% of VSDs in the USA. Other types, supercrystal inlet or posterior, don't worry about those. Just remember perimbrinous. Clinical manifestations, like I said before, are not very important for these, and it varies with VSDs depending on the size and the hemodynamic effects of the defect. Small VSDs are probably going to be asymptomatic in most cases. If it's larger, you can have these frequent upper respiratory infections, fatigue, and then the larger VSDs, like I talked about before, can actually have that reversal of the shant and turn into isomanger syndrome.

That's when you'll have the cyanosis and things like that. So that's going to be only in the large VSDs. Now, physical exam, again, is the important thing. So make sure you're paying attention now. Clinical manifestations, you can kind of zone out physical exam. Make sure you're listening. So this is what you need to know. Ventricular septal defect is going to have.

high-pitched hollow systolic murmur that's at her best at the lower left sternal border so high-pitched hollow systolic murmur at the lower left sternal border that's what you need to know for this um and diagnosis again is going to be with an echo so trans thoracic echo is going to be your single most important test to diagnose and classify the size and the severity of the the ventricular septal defect so a lot of similarities like i said before in the diagnosis and the treatment um EKG you can do as well. It's going to be nonspecific, but it may show some left ventricular hypertrophy. Now, treatment, it all depends on the size. Of course, like I said, with all of these surgery is going to be your definitive. But if it's small, you can just observe. Small VSDs

have a reasonable likelihood of spontaneous closure, usually within 12 months, just like an ASD. Now, moderate to large VSDs, you're going to go ahead and do surgical repair. And it's usually with a patch closure. Like I said, don't worry so much about the specific types. They're not going to really ask you that. Okay. Now, there's a few things that I think you need to know for ventricular septal defect. I have a way for you to remember it. And hopefully this helps you. So as soon as you see VSD, ventricular septal defect, think a very sharp dagger, VSD, very sharp dagger, chops a hole in your ventricle. So as you see VSD, think very sharp dagger, chops a hole in your ventricle. So chops is where the actual mnemonic is. So CH OPS chops. And what that stands for is

The C stands for common because this is the most common congenital heart disease of childhood. The H stands for both hollow systolic and high-pitched murmur. So the H and chops, hollow systolic and high-pitched murmur. O stands for observation, like I said, small VSDs may close on their own, so just observe. P stands for peri membranus, which is the most common type of VSD. And then the S stands for surgery. So chops is going to be common, most common congenital heart disease of childhood. H, holosostolic and high-pitched murmur, O, observation, P, perimberness, and S surgery.

So VSD, very sharp dagger, chops a hole in your ventricle. Okay. Moving on to Tetralogy of Phelow. So what does this start with? It starts with a T. Turquoise, cyanosis. So this is a cyanotic condition. And it's actually the most common cyanotic congenital heart disease.

So VSD was the most common overall. This is the most common of the cyanotic congenital heart diseases. So Tetrology of Phelow really consists of... of four different problems in the heart. So the first one is going to be a right ventricular outflow obstruction. So something's causing an obstruction in the right ventricle. The second thing is, and then in some of, sometimes this, instead of a right ventricular outflow obstruction,

they'll describe it as pulmonary stenosis. But right ventricular outflow obstruction, you'll see it really listed more on like up to date in some of the more legitimate places because it's not necessarily a pulmonic stenosis. It can be caused from a few different things, which we'll go over. right ventricular outflow obstruction. The second thing is right ventricular hypertrophy. And that's because of the outflow obstruction, which causes the right ventricular hypertrophy.

The third thing is going to be an overriding aorta where the aorta actually shifts over to the right. And then finally, you're going to have a VSD, so a ventricular septal defects. So those are the four things. Right ventricular outflow obstruction, right ventricular hypertrophy, overriding aorta, the aorta shifts, and then a ventricular septal defect. Those are the four things. Now, just a little bit of an explanation about each and why it happens. So the right ventricular outflow obstruction, it can occur at multiple levels of the heart. And sometimes the obstruction is due to muscular bands blocking the flow.

Sometimes it can be due to the pulmonary valve. In some cases, the pulmonary valve becomes bicuspid and stenotic. So that can cause it as well. And that's sometimes why it's called a pulmonary stenosis instead. but it's not that, you know, it's not that specific to that. That's why it's more just right ventricular alpha obstruction. So that's the first thing. The second thing, due to this right ventricular outfall obstruction, the right ventricle is pumping harder against this obstruction. It has to work a lot harder. What happens when the heart works harder at hypertrophies. So that's why the second thing is

going to be a right ventricular hypertrophy, which is going to be the second finding. The third thing is going to be this overriding aorta, as they call it. So the aorta in patients with tetralogy flow instead of being anatomically positioned over the left ventricle to receive blood during systole, it's actually displaced and it moves over to the right and a little bit posterior, and it actually fixes itself right over the ventricular septal defect instead of being over the left ventricle. So it actually shifts over and actually is right over the ventricular septal defect, which is the fourth finding, which is a ventricular septal defect, which is straight to the point, just a hole in the ventricular septum like we went over before. And then the blood is moving

from the right ventricle over to the left. And that's why it's a cyanotic condition. And the reason why it is a cyanotic condition is because that right ventricle all of a sudden has all of this pressure, which is overtaking the normally higher pressure on the left. But because of this outflow obstruction, you have all this excess pressure. So it shifts it over to the left. And that's why this is a cyanotic condition and a right-to-left shun. Now, there are genetic components as far as the etiology of tetralogy flow. There's a few that you need to know, or at least be familiar. familiar with. About 15% of patients with Tetralogy of Fallow have one of the following. So Down syndrome

is associated with this, Trisomy 21, DeGeorge syndrome, which is chromosome 22 deletion. And then another one known as Allegyel syndrome, which is a genetic disorder. This one's not very common that can affect many organs, particularly the liver and you have this bio buildup. So those are the three things that you may see in a vignette that's associated with Tetralogy of Phelot. So Down syndrome, de George syndrome, or allegial syndrome. clinical presentation, again, don't put too much into this, but it all depends on the degree of the right ventricular outflow obstruction. The main thing that you need to know, particularly in infancy, is that patients will present with psionosis, like I said. And it's the main reason why

Tetralogy of Phelow is often described as blue baby syndrome, because these babies come out and they're completely cyanotic. They're blue. Another potential symptom or presentation, so this is probably the only important clinical presentation I would say to know is something known as TET spells. So TET spells, and you probably will see this at some point in a question. So what TET spells are is this transient occlusion of the right ventricular outflow track. So all of a sudden completely blocks the right outflow track.

And then these patients are going to have this sudden severe cyanotic episode. And normally, this is going to occur when, you know, young children like babies become agitated, start crying or in older children after they have some form of exertion like, during exercise. And in older children, they have this learned habit to improve these syndrome, to improve these tet spells. And what they do is when they have these episodes of psionosis, these tet spells, they actually squat. And this improves their symptoms. They feel a lot better. And why does that help? Well, as I talked about before, you have this cyanotic condition, this right to left flow because all the pressure on the right side is shunting the blood to the left.

Now, when you squat, you increase peripheral vascular resistance in the legs. causes the left ventricular pressure to temporarily rise because it's pushing against this resistance and it actually exceeds the right ventricular pressure. So you reverse the shunt temporarily, but you temporarily reverse the shunt from a right to left to a left to right because of all of this extra peripheral vascular resistance. And it decreases their cyanosis and they temporarily feel better. So that's really the only clinical manifestation I would say to really be familiar with. Now in physical exam, you're going to have a harsh systolic ejection murmur, which will be best heard at the left sternal border because this area is closest

to where the narrowed right ventricular outflow tract is. And this is where the blood's pushing through the stonotic area. And this is actually the murmur that you'll hear. Now you may wonder, why am I not hearing the VSD that these patients have? Well, it's because the area of the right ventricular outflow tract, the obstruction is actually going to overshadow that. It's actually much louder than the VSD. So this is normally what you'll hear. So Tetrology flow, listen for a harsh systolic ejection murmur. Best heard at the left sternal border. Now, diagnosis, surprise, it's going to be an echo.

So diagnosis will be made with echo. You can also do EKG's chest x-ray, which will be suggestive of tetralogy fallow, but definitive is going to be with your echo. But one finding that you need to be familiar with on chest x-ray is something known as the heart being boot-shaped. So B-O-O-T, like the shoe, boot-shaped. and the reason why the heart is boot-shaped is because the apex of the heart is actually flipped and it's upturned and you have this concavity of the main pulmonary artery don't worry too much about that but just know if you see a chest x-ray and they describe it as boot-shaped or they mention anything about the the heart being the apex of the heart being upturned you should be thinking of tetralogy of philoh so that's one of the few x-ray findings i think you need to be familiar with these i'll go for another one later in another condition but that's one of the ones you need to know now treatment there's

two things that you need to know for treatment. And that's surgery, like in all of them, that's your definitive. And then prostate glandins. So surgical repair you want to have early on in these patients normally within the first year of life. And so that's going to be your definitive treatment. And now prostate glandins. So why prostaglandins? Well, in newborns, when a newborn, when they're first born within the first couple of days, they have this ductus arteriosis, which is open for the first couple of days of life. So a newborns, a PDA is normally present for the first few days of life, then it spontaneously closes on its own. And the reason why you want to keep this open, and that's with prostate gland, and specifically Alprostodil is the one that's normally used. That's the specific

prostaglandin that's used because prostaglandins like Alprostodil keep this PDA open. And why do you want to keep the PDA open? Well, the PDA, these patients have this rife ventricular outflow obstruction. It's hard for them to get the blood oxygenated. They're having all these issues. getting blood pumped out and you have this PDA which is this connection between the the pulmonary blood and the aorta so this actually improves circulation by providing a secondary path for some blood flow to get past some of this right right ventricular outflow obstruction so it just provides additional pulmonary blood for increased O2 levels for these patients you want to keep the PDAs open in these patients just as a bridge to

surgery until they can be treated so this is going to be like supportive for them to make them feel better until they can have the definitive treatment, which is surgery. So prostate gland is particularly alprostodil, that's the main one used. And then surgery is going to be your way to treat. Now, there's a few things you need to know for Tetralogy of Fallow. I have a way for you to remember it. So remember the sentence, patients with Tetralogy of Phelow crave oxygen. Patients with Tetralogy of Phelow crave oxygen. So crave is going to be the word you want to use. And then Crave stands for Cyanosis. Remember, this is the most common cyanotic congenital heart disease. R stands for right ventricular hypertrophy and right outflow obstruction. Remember, those are two

of the four causes. A is going to stand for alprostodil. That's the first line prostaglandin used to keep that PDA open. And the other A is going to stand for aorta overriding. Remember that shifted aorta to the right. V and Crave is going to stand for VSD. That's the ventricular septal defect, three out of four abnormalities. Or actually, it's the fourth because we did aorta overriding. And then E is going to stand for echo, which is going to be your way to diagnose this. So again, patients with Tetralogy Ophalo crave oxygen. Cyanosis, R, right ventricular hypertrophy, and right ventricular outflow obstruction, A, Alprostadil, and aorta overriding, V, VSD, and then E ECHO. So that's going to be their way. You remember the things you need to know for Tetralogy of

follow. They crave oxygen. Okay, we'll only have a couple more left. We're getting there. Next one is going to be corotation of the aorta. So this is a narrow. of the descending aorta. It's normally located at the insertion point of the ductus arteriosis, and it's just distal to the left subclavian artery. So that's important to know for something later, which we'll go over. So the coerctation of the aorta is normally just distal to the left subclavian artery off the aortic arch. Now, it's more common in males, about 59% of males and then versus 41% of females. And there's two important associated conditions that you need to be aware of in patients with coerctation of the order. So the big one, particularly in adults, is bicuspid aortic valve.

So this is about 70% of patients with coerctation of the order will have this bicuspid aortic valve. And remember, the aerobic valve is normally has three leaflets. So these patients have a bicusp valve. So they just have two leaflets instead. So 70% of patients with corotation of the order will also have a bicuspid valve. Definitely needs to know that one. Then the second one that's associated with this condition that may come up in a vignette is Turner syndrome, which is present in up to 15% of patients with Turner syndrome. So 15% of patients with Turner syndrome will also have coerctation of the aorta. Now, clinical manifestation, again, not really that important. It varies in different age groups. Neonates may be asymptomatic. It all depends on the severity.

They may have some poor feeding and things like that, really nonspecific. And then children and older infants may start to have some angina, some chest pain. They may have cold extremities. They may have clotication with physical exertion. So when they exercise, all of a sudden, they get this cramping mainly in the lower extremities, which is due to the decreased blood flow because it's pushing past this stenosed aorta. Now, adults, the classic finding is going to be hypertension. So that's the main one you need to know in adults. But again, clinical manifestations aren't that important or aren't really that specific for any of these diseases. So don't focus too much on them. but your physical exam is very important.

So this is what you really have to know. I'd say of like all the physical exam findings and all the things you'll learn in PA school, this is probably like the top 10. This is the one you really need to know that'll definitely come up and you'll definitely hear about it. So what's so important on physical exam about coerctation of the aorta? So you have this classic finding on physical exam and patients with coerctation of the aorta. And it's going to be hypertension, relative hypertension in the upper extremities. So higher blood pressure in the upper extremities.

and lower blood pressure or hypotension relatively, you know, compared between the two, and the lower extremities and also a delayed or diminished pulse in the lower extremities. So you're going to take their blood pressure in the upper arm. It's going to be like 140 over 90. You'll do it in the lower extremities, which you don't commonly do, but if you're suspecting this diagnosis, you would. And it's going to be like 90 over 70 or something like that. So hypertension, upper extremities, hypotension, lower extremities,

and then also a delayed or diminished pulse in the lower extremities. Okay, now why does this happen? If you understand it, you're never going to forget it. So you have to do like a quick review of anatomy in your head. So if you remember the aortic arch, you remember that on the arch of the aorta, the first thing that branches off is going to be your brachiocephalic that has the right subclavian on it, the right common carotid, then next branches off the left common carotid, and then finally you have the left subclavian artery. So you have all of these branches. And then you have the coerctation of the aorta. So it occurs after all of these branches off of the aortic arch. Now, what's important is the couple things that branch off before

this coerctation is going to be the right subclavian and the left subclavian artery. Those are important because both of those supply blood to the right and the left arm. Okay. Now, what you have to visualize here is think about a hose. And in the hose, you puncture two holes in the hose. So the water sprang up out of the top side of the hose of these two holes you made. Now, imagine pinching or clamping down in between those two holes. Now, the first hole is going to be where the left subclavian and the right subclavian are. So that's the first hole. And then after that first hole, you're pinching down on it.

Now, imagine that water. Think about the water. You clamp down after that hole. You're going to have much higher pressure in that first hole because you've clamped off. Water can't really get through that well. So it's backing up and it's spraying higher pressure there. Now, think about the hole after where you pinched or you have this corotation, we could say, of the hose. how high of the pressure do you think is going to be in that second hole?

It's going to be much lower. Less water is getting through. You have this stonotic area of the hose and the water can't get through as well. So the pressure is going to be much lower. It's also going to be delayed too because it takes a little bit longer to get there. It has to get through this obstruction first. So that's why these patients have higher blood pressure in the upper extremities and lower blood pressure and a delayed or diminished pulse in the lower extremities.

So if you think about it in your head, it makes complete sense. And that does help with certain. things to kind of understand why these things happen, rather than just kind of memorizing them, which sometimes we do as well. That's okay also. Now, you also may have a systolic murmur on physical exam, but honestly, who cares? The main thing you need to know is that hypertension, upper extremities, hypotension, lower. Now, diagnosis, of course, again, is going to be an echo, test of choice to establish

the diagnosis and the severity of the coerctation. You also may do a CT angio and an MR angio, so a CTA or an MRI. But this is really like a complementary diagnostic test in addition to the echo. And it's really for planned surgical intervention so they can get better anatomic detail. But echo is really all you need to diagnose this. Now, you don't need chest x-ray to diagnose, but you better know the findings on chest x-ray. Because these are another couple of the really, you know, those key terms that they like to talk about. Again, they're not going to give you the key term, but they're going to describe it and you better understand.

So coerctation of the aorta on chest x-ray, you're going to have two. specific findings. You're going to have the first one that's known as posterior rib notching. So on a chest x-ray, if you look at the ribs in a patient with coerctation of the aorta, sometimes you'll see this notching on the posterior margin of the ribs. So like on the bottom of the ribs, you're going to see it. And it's this little curved out notches. And it's actually caused from all this increased arterial flow and these collateral vessels that are going right underneath the ribs that overlie the bottom margin of the ribs. So posterior rib notching on coerctation of the aorta. And the second one is something known as a figure three sign or just a

three sign like the number three. And this happens because if you look at the aorta on a chest x-ray, there's this indentation of the aorta. And this is the area where you have this, the coerctation. And then right after that, that coerctation, you have this post-denotic dilation where the aorta gets big. So you have a couple different areas where they have these indentations, and it actually looks like a three. So just remember, posterior rib-notching and a figure or three sign on corotation of the aorta of chest x-ray. Never going to diagnose it that way, but you need to know that for your exam. And then, of course, you can also do any KG.

You probably show some left ventricular hypertrophy, but not very specific or anything that you would normally need to know. Now, treatment, corrective intervention. Again, surgery, of course. So it's going to be this corrective intervention. Surgically, you can do a trans catheter intervention, like a balloon angioplasty, to open up that stenost area. and it should be performed early on, preferably in early childhood.

And really, you only do this in patients that have really severe stenosis of the area, they have systemic hypertension, heart failure, or just a really critical case. You're going to make sure that you have this intervention, whether it's surgery or a trans catheter intervention. And then just like in Tetralogy of Phelot, you're going to use prostaglandins as well, again, particularly alprostodil, will be used preoperatively just to maintain the patency of the ductus arteriosis and just to help them symptomatically, as well as using inotropic agents like dopamine, dopamine, all supportively just as a bridge to stabilize the patients until they can have

the operation to correct it. So overall, surgery is going to be your definitive and then you can also use things to bridge to surgery to make these patients more comfortable like your inotropic agents and your alprostodil, the prostate glandin. Okay. So this is the last part. We're going to go over, patent ductus arteriosis we are almost there so patent ductus arteriosis first let's really quickly review what the the ductus arteriosis is and its purpose so a ductus arteriosis is a connection between the aorta and the pulmonary artery in a fetus and it shunts blood from the pulmonary artery directly to the aorta it bypasses the lungs because in a baby in a in a fetus you bypass the lungs because the the fetus is getting oxygen from the mother's placenta. They don't need the function of the lungs.

But then after they're born, after birth within the first couple of days, the newborns obviously breathing, the lungs are functioning and oxygenating the blood. So they no longer need this ductus arteriosis. So it should close normally within the first couple days of life. If it doesn't, though, it's termed a patent, which basically just means open like patency, patent ductus arteriosis. So if it doesn't close within the first couple days of life, you have this PDA, this patent ductus or arteriosis, which we learn can be beneficial in some cases, but normally this isn't a good thing. Now, as far as epidemiology, you have a two to one female predominance. Prematurity is a big one. You'll also see a higher incidence in infants born at high altitudes versus sea level, which is

interesting. And it's really just because of the hypoxia, the lower O2 saturation in these higher altitudes, which can predispose them to a PDA. And then there's also an increased incidence in infants with congenital rubella, which is, doesn't seem that important, but I do remember getting a question on that in school. So you should probably just put that in the back of your mind and hopefully you'll remember that. Now, the patho behind this is really simple. And it's what we went over before. Remember when we wanted to keep a PD, we wanted to keep the ductus arteriosis open? What did we give them? What do we to maintain that PDA? We gave them prostaglandin. So it's the same reason. That's the patho behind this. So these patients with patent ductus arteriosis have this

continued prostaglandin E1 production. And that keeps the ductal patency because they have this continued prostate gland in E1 production. So it's a simple patho. That's why it's maintained in these patients. Now, clinical manifestations varies depending on the size, the length of the PDA, as well as the left to right shunting. And again, remember, what is this start with? It starts with a P, not a T, so it's non-synotic. But generally, like all of them, it's not specific. You're going to see some poor feeding, frequent low respiratory tract infections, some weight loss, nothing very specific. And again, just like all of the other ones, too, the non-sionotic, it is possible for them to develop isomeliger syndrome. So just remember that. It can switch the shunt, and they would have sinus.

And, all right, so just like the other ones, turn your, you know, kind of close your ears for the clinical manifestations, but turn them back on for the physical exam. This one's really important, too. This is one you should definitely remember. So on physical exam of a PDA, patent. inductous arteriosis, you're going to have this continuous machine-like murmur. So it's this continuous machine-like murmur. And I wish I had a recording, but you will never forget it. When you hear it, when you're doing oskies, you're doing clinicals, they let you hear it. It literally sounds like, just like machine static.

It's this continuous machine-like murmur. And it's heard best at the pulmonic area. The way that I remembered continuous machine-like murmur, hopefully you're old enough to remember. There used to be something called a PDA, which is a personal digital assistant. they were Palm pilots. And I always remember PDA's personal digital assistants were little machines. They were little computers or little machines. And then I always remembered, okay, machine like murmur, continuous machine like murmur.

So you see PDA, think of little machines, personal digital assistant, and then you think of continuous machine like murmur. They may also have bounding peripheral pulses, so the strong throbbing pulses, as well as a wide pulse pressure. So if you're not familiar with what pulse pressure is, it's the difference between your systolic and your diastolic blood pressure. Normally over 50 is going to be a wide pulse pressure. The normal range is going to be anywhere from like 30 to 50. So if you take your systolic and it's 120 and your diastolic is 60, that's going to be a 60 pulse pressure and that's a wide pulse pressure

because it's over 50. So if you have like over 50, that's a wide pulse pressure and that's what you're going to see in these patients. They may have a wide pulse pressure as well as bounding peripheral pulses as well as the machinery. The continuous machinery murmur, do not forget. that that's the most important thing. Diagnosis, of course, is going to be with an echo, your best confirmatory test. Cardiacath and angiography are also considerations, but really only in the context of a more complex congenital heart disease. You can also do any KG, just like all the other ones, you're going to see like left ventricular

hypertrophy, left atrial enlargement, but not very specific. Now, treatment, okay, so I want you to think about this so you can come up with the answer yourself. So the problem is caused from this continual production of prostate glandin, which is keeping the ductus arteriosis patent or open. So you have this continuous prostaglandin production. So can you think of a common medication that inhibits prostaglandin? So prostate glandin inhibition will met as that. So it's ensigns. Ns are going to be your first line treatment to close. Stop the prostate glandic production and close this ductus arteriosis. So ibuprofen are in endomethcin

are the main ones that they use. So normally you'll give these patients. This is really the only one where definitive, technically it's your first line, definitive would still be with surgery, but really where medicine plays a much bigger role. So ibuprofen endomethicin is usually used for about 48 hours in the attempt to stop the prostate gland production, inhibit it, and close these PDAs. After about 48 hours, they're non-responsive to the insides. Then, of course, next step would be surgical closure with either a surgical ligation or a percutaneous catheter occlusion. Again, don't worry so much about the type. Just know that surgery, again,

is definitive, but medicine does have a pretty big role here and actually can be curative in some cases. So ns, and then definitive is going to be with surgery if enseds are if they fail to work. Now, that's it. We're done. I just want to do five quick questions. You can just kind of test your knowledge, see what you retained. So let's do five quick questions and then we'll wrap it up. So what is the most common type of congenital heart disease? Most common type of congenital heart disease. So that's going to ventricular septal defect most common type of congenital heart disease what are the four abnormalities seen in tetrology of flow four abnormalities seen in tetralogy of flow so that's going to be your right ventricular outflow obstruction right ventricular hypertrophy ventricular septal defect and

overriding aorta next one describe the murmur and patent ductus arteriosis you better not forget this we just said it so pda patent ductis arteriosis continuous machine like murmur. Four, describe the potential findings on a chest x-ray and a patient with coerctation of the aorta. So that's going to be patend duct, or I'm sorry, posterior rib-notching or a three or figure three sign. That's on coerctation of the aorta, chest x-ray, posterior rib-notching, or a figure three or three sign.

All right. Last one. What is the most common type of atrial septal defect? Most common type of atrial septal defect. That's osteum secondum. that's secundum osteosacundum okay that's it uh we're just under 40 minutes there that was a long one but there's a lot of material i hope the the pneumonics are helping and and these these podcasts are helping overall and thank you so much as always for the reviews i do so much appreciate it

and i also want to let you know that if you haven't checked out my youtube page yet please do i think it'll help a lot i have a lot of visuals um for a lot of things i go over that makes it a lot easier to understand so thank you so much as always um good luck in good luck on your pants your Pannery, your EORs, and good luck in PA school.