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Ok, I think we're live now. Um Good afternoon everybody. Good afternoon. My name is Christine. I'm one of the education leads for Manchester Pediatric Society. Um and with my really nice f one here, sweeter and she's gonna do a lovely session on pediatric cardiology today. Um Just to again, say thank you guys for coming. Um Hopefully you shouldn't take too much of your time. I noticed that some of you guys said before that um you were quite pleased with sort of our lectures, but you also said that you wanted some more osk things coming up and as a society, we're planning some things. So just make sure you're staying tuned on Instagram and on Facebook. Um we'll be posting some sort of ay content that we're gonna have sort of building up. Um So yeah, without any further ado I'm just going to close my camera and I'm gonna let sweeter sort of take it away. Yeah. Um, hi guys. Um thank you Christine for that introduction. I do recognize some of the names from last week. So thank you so much again for coming this week, er, for those who weren't there. I'm sure I'm one of the F ones. Um, I graduated from Manchester this July and I'm currently working on OBS and Gynae. Um, I'm just gonna share my slides very quick. Um, oh no, actually I think I'm just, there we go. Can everyone see that? No, we can't see at the moment. Ok. Just a second. What about now? Yes, we can see it. Ok, perfect. Er, so like Christine said, today's, um, topic of discussion is pediatric cardiology. Er, so before I get started, just a disclaimer, I'm an F one. So this lecture is purely at the level of what clinical year medical students like you guys should know or even if I ever say come in contact with an infant or an a adolescent and I'm thinking about cardiology, the level of knowledge that I should have um pediatric cardiology in itself is very specialist. Um but the good thing is at my level or your level, there's not tons, we need to know. There's only very few things we need to know. Uh So that's, that's a great thing. So let's just get started. So, um this is what we'd cover today to start with the case. So we've got a five week old female infant who was referred to the hospital because of wheezing poor feeding, poor weight gain during the last two weeks. So while the child was born, she was fine, but three weeks into life, she's developed wheezing, poor feeding and weight gain. Uh So what else do you want to know if you can pop into the chat? Let's start with, say the history. What do you want to ask the parent and I'll move to the chart so I can see what you're saying. Just pop into the chat guys. Anything you want to ask at all? Is there a fever? Yeah. Change in color? Kishen. When you say change in color. Is there anything specific you want to ask about to anyone? Really? On the um talk with Kham I'd love um if we could have done this like on zoom plethoric. Yeah, gray. Very good. The other one is blue as well. If they turn um blue because of cyanosis, any other inputs from anyone else guys, it doesn't even have to be specific to cardiology. Just a general pediatric history. What do you wanna ask the parents about? Ok. Give everyone another two minutes to pop stuff into the chat. Rashes. Yeah. What about, let's forget cardiology for a second. Some a child comes in with wheezing, poor feet and weight gain respiratory stuff we discussed last week. What symptoms do you want to ask about? Wet nappies? Yeah. And why would wet nappies be relevant? Birth history? How many weeks gestation? Very good neonatal sepsis N day. Very relevant. And on top of the birth history, something to be that would be useful to ask about is antenatal scans as well. If anything was picked up on the scans at all. Um As an extension of birth history, you can also ask about maternal history in the sense. Does the mom smoke or drink? Does the mom have any medical condition? Things like rubella diabetes, um high BP that might increase your risk of um certain conditions? Um So here, let's move on. So she was fairly well in herself up until three weeks ago, her neonatal examination, postbirth was normal. So that would be relevant because we know that once she was born. So on an antenatal screen, nothing was picked on our antenatal scan, nothing was picked up just after birth as well. The child was well, but it's three weeks into uh life that she's developed these symptoms. So we know this is a heart disease where she's got decompensation after a time period. So the reason this is important is because with congenital heart diseases, they present during three periods during their life. It's either just after birth or it's after a couple of weeks or months or they're fairly well during childhood. And once they become an adult or an adolescent, they develop symptoms. So examination wise, she's tachypneic. There are some sternal and intercostal recessions which would suggest respiratory distress, scattered wheeze pulses are normal. She's got a pansystolic murmur and her liver is enlarged. So, what do you guys think of this scenario? What do you think's going on? Don't just say congenital heart disease, cos that's what we're talking about specifically. What do you think is happening? So, like what do you think of the liver being enlarged? Why, why would that be relevant to someone presenting with respiratory symptoms and a murmur? Any responses? Christine? Um What I think um maybe the pansystolic murmur sort of pushed me towards more of a VSD sort of scenario. Yeah. So VSD ventriculoseptal defect. But what I was trying to get at more was the fact that this child's got a heart disease. But now the child after three weeks has developed heart failure and that's why she's got hepatomegaly. So this right sided heart failure is caused um sorry, the congenital heart disease has caused right sided heart failure that has in turn caused decompensation and hepatomegaly. So the reason we've got this case up here is at this level as a med student, you often will get even in your Aussies and stuff histories and sometimes it might not be obvious, it might look like a respiratory history. But you should remember in the back of your head that congenital heart disease is an important differential. It did come up in one of my Aussies. So yeah, um at this point, what investigations would you do? So this is not a trick question, cardiac investigations, what investigations would you order cardiac exam? Yeah, very good. And after the examination, what might you ask what E CG chest X ray echo spot on? So that's about it. So, moving on just to briefly talk about fetal circulation. Um What do you guys think is different in fetal circulation compared to adult circulation? So, you've got a little picture to help you. Um, anything, you know, at all bypasses the liver. Very good. Three bypasses two for the lungs. One for the liver. Yes, very good. Um So essentially like Anastasia and Kushen have said, uh one of the main things for fetal circulation is that you've got fetal shunts. So, one near the liver and two for the lungs and these fetal shunts help flo bypass the lung and the liver. The other thing is lungs in fetuses aren't functional. So the placenta is where fetal blood gets oxygenated. So the alveoli in fetal lungs are shut. So what essentially happens is you've got blood going through the descending aorta, which is mixed, mainly deoxygenated, but also a bit oxygenated, goes through the umbilical arteries into the placenta. It gets oxygenated, goes back through the umbilical vein, some of it goes into the liver and the remaining bit bypasses the liver and goes into the ductal veno ductus, vinosus, and then into the inferior vena cava from there, it goes into the right atrium. So that's where it comes into contact with one of the shunts, which is the foramen ovale in the atrium atrial wall, which lets blood go from the right to the left atrium. Now, the reason why this happens is because in adults usually it's on the left side of the heart where there is high pressure. But in infants, because the lungs aren't functional, it's on the right side of the heart where there is high pressure because of which blood shunts from the right to the left, the remaining blood goes from the right atrium to the ventricle into the pulmonary artery. And again, there is another shunt there called the ducts arteriosis. And because similarly, it's in the pulmonary circulation in the fetuses where there is high resistance of pressure, blood flows from the pulmonary artery directly into the aortic arch. Happy. So at birth, your alveoli expand and at that point, the pulmonary vascular assistance, which was before high starts to go down because of which your shunts close. The other thing that happens is your prostaglandin levels come down and the doctor's arteriosis close closes. So two things happen, alveoli, open your pulmonary circulation resistance goes down and the prostaglandins um levels also go down. Yeah. So moving on, why? Even though congenital cardiac diseases are quite rare, why are they important? So, one in about 10 stillborn infants have cardiac anomalies. Eight per 1000 live births have significant cardiac malformations, 30% of which would require intervention in the first year of life. Um And in terms of heart diseases in Children, they're primarily congenital. You can get arrhythmias and you can get things like rheumatic heart disease, infective, endocarditis, Kawasakis, but those are not primarily cardiac conditions as such. Well, infective endocarditis is, but the other two aren't primarily cardiac. There are more complications that happen because of another underlying pathology. So at our stage, in terms of heart diseases in Children, it's the congenital structural ones that we need to remember. Yeah. So what do you think are some risk factors for congenital cardiac abnormalities? I would love a bit more interaction on the chat because otherwise I feel like I'm talking to myself doesn't have to be right answers. It can even be guesses. What risk factors do you think causes congenital heart diseases, maternal alcohol use very good Down Syndrome. Anything else? Kitchen said Down Syndrome, any other chromosome and abnormalities? You guys can think about maternal smoking. Yeah, possible low folate? Ok. Um I'm Anastasia. Do you know why low folate might be linked to congenital heart disease, family history, prematurity? Yeah, very good. Does anyone know why low folate levels might be linked to congenital cardiac diseases at all? Cos that's actually new to me. I'm not sure myself might be good to look up later, I suppose. Moving on. So like you guys said fetal alcohol, smoking, but also warfarin therapy for drugs in terms of disorders, rubella diabetes and lupus can commonly increase your risk of developing heart diseases, chromosome and abnormalities like you guys have said Down Syndrome, Turners, but also Edward Noonans and Petals syndrome. So these are kind of the risk factors. You want to ask a parent about or rule out when you're thinking about congenital heart diseases? So how do you think a child with a cardiac disorder commonly presents or when do you think it's usually picked up or how is it picked up? Any ideas? Has anything been said on the chart? Christie. Um we've got an nay, I love them very good. So, on the antenatal ultrasound. So after birth, how do you think Children usually present? So one might be with heart failure like we saw in that case. Um What about other ways? So we've got poor feeding. We've got during a chest infection when a clinician listens to the chest and we've got may have no symptoms but might be cyanotic at birth and may present later on in development. Yeah. Um Yeah, sinuses at, yeah. The other common presentation is murmurs as well. So in terms of how they present, one is antenatally on ultrasound, usually done between 18 and 20 weeks. The other common presentation is heart murmur. So might be picked up during the initial neonatal examination or six weeks later. But like you guys said, often asymptomatic, the other one is signs of heart failure. Uh So things like dyspnea, tachypnea, tachycardia, poor feeding. Uh The other one is symptoms of shock. So, hypotension that gray appearance or plethora like Ken previously mentioned, um tachypnea as well and sinois when the child becomes blue because of low oxygen levels. The important thing to remember though if is, if sinois is persistent, then it's nearly always a sign of structural heart disease. Yeah. And like we discussed previously, if you pick up anything from any of these different steps and you think this might be a heart disorder or a heart disease, then you want to do an ECG chest X ray and echo. So, murmurs, uh they're the commonest presentation of congenital cardiac disease and most murmurs are innocent. So it's in about actually, that is wrong. Not in 30% even more than 30%. Most murmurs are innocent murmurs and it's often heard during a febrile illness, like an upper respiratory tract infection or anemia. So you also have to examine a child after the illness and make sure and to see if the murmur is still there. And how do you think you would differentiate an innocent murmur or a benign murmur from a pathological murmur? So, what kind of symptoms signs are you thinking about? There's something called the five Ss. Has anyone heard of that at all? If it's heard during diastole? So that would be more pathological. Yeah. Anything else? So, so the five Ss would be a soft systolic murmur, asymptomatic and left sternal edge. So these features um make you think that it's more likely innocent. Plus they also shouldn't have heart added heart sounds or a thrill or radiation to anywhere else. Yeah. So if it's pathological, you're thinking it's pathological. It's us, it tends to be louder than two. So it's either loud with no thrill or a palpable thrill. Or you can hear it without a stethoscope. It also can be louder on standing. And the other thing is they can have symptoms like failure to thrive feeding difficulties, sinois, shortness of breath, et cetera. So these would be the different criteria as for why you will refer emerg as an emergency. If it's louder, louder than two out of six diastolic, it's louder on standing and if they have other symptoms. So moving on, can you guys think of how you might classify congenital heart disorders? Like what are the different types? Oh, someone said positional for, yeah, acyanotic. Yeah. And the other one would be cyanotic. Very good. So I'm someone can only that I'm someone that can only learn if I've got um these kind of flow charts and things. So I was quite, I spent a lot of time making this even though it shouldn't take that much time. So I hope you guys appreciate this. So broadly speaking, congenital heart disorders, you think AYO and cyanotic sinos cyanotic, like the name suggests, the kid usually turns blue because of low oxygen levels, Ayo, they don't have those symptoms. So there are a few different things on this slide. It's quite busy, but the ones that we need to mainly focus on is those in black. So one of the main causes or main types of acyanotic disorders we need to remember is left to right shunt. So that's when the blood goes from the left side of the heart to the right because of a septal defect or a patent doctor's arteriosis. And when patients have this disorder, they're often asymptomatic. Unless it's large or longstanding, then they can have signs of heart failure. So, the common signs of heart failure are shortness of breath, poor feeding, recurrent chest infections, like you guys have said before, tachypnea, tachycardia, hepatomegaly and cool paraphrase. The other one that you want to remember uh under cyanotic heart disorders is right to left shunt, which is the other way, which means deoxygenated blood mixes with deoxygenated blood. Hence why patients present with this classic cyanotic picture and along with cyanosis, they can also present with collapse and low oxygen levels. And there's two main conditions that we need to remember which is teratology of fallow and transposition of great arteries. Eisenmenger syndrome is something that infants or patients develop when they initially have an ayo disorder, like say atrial septal defect or ventricular septal defect. And over time because of decompensation, they end up developing um a cyanotic heart disorder and two other types, which I'll briefly mention, but you don't have to know in detail under a cyanotic. So one is when you get outflow obstruction, but the child is fairly well. So usually this is because of a narrowing of a valve, like you see with pulmonary or aortic stenosis patients are often asymptomatic. But with exertion, they can develop symptoms of shortness of breath, fatigue, dizziness. The other one is when they have outflow obstruction, but the child is quite sick, like seen in coarctation of aorta, which is when the aorta itself is obstructed and the blood flow is um not going through. Hence why the child gets quite sick. They can present with heart failure but also shock. So when you think shock, you're thinking of symptoms like hypotension or low BP, tachycardia, um tachypnea or even sometimes apneas, which means you might not hear any breathing or they're breathing too quick, hypoxia and they end up having metabolic acidosis as well because they're hypoxic, then they start having anaerobic respiration and because of that, your lactate levels go up and hence the metabolic acidosis. They might also present with lethargy, irritability and in coarctation of aorta specifically, they might have that gray appearance of the skin and floppiness of the limbs as well. Yeah. So you don't have to know all of it. The, but the main thing to take away is um left to right shunt for ayo heart diseases and three main types and right to left shunt for cyanotic heart diseases and the two main types. Plus Eisenmengers happy. Is everyone happy? You guys have any questions at the moment? No questions at the moment. I'll let you know, I guess I put something on the shop. Perfect. Ok. Uh So just to start off with a cyotic heart diseases. Firstly, um patent ductus arteriosis. So that like we said, is a left to right shunt, which means it's the oxygenated blood that's mixing with the deoxygenated blood. And I'm gonna say something. Um Kisha, how would it present in an oy? Uh That's a very good question Kishen. Are you asking specifically to PDA or just congenital heart diseases in general? Yeah, it could be like different, Like you could have an examination session and somebody they cannot see like you had a murmur. What could that be? All right. Very good. So usually like with congenital heart disease in a history station, yeah, they could give you clinical signs but at least when I was in fourth year, they were more interested in you being able to pick up that it's a congenital heart disease. Cos I think when in my case, it was a child with wheeziness, breathlessness, sleepy, um like four weeks into birth and they didn't want you to think down just the route of respiratory and for you to pick up on the fact that this, this could also be a heart disease. It's more from that side of things, clinical signs. Yes, they could easily give you that in the examination station. But it's mainly picking up that when a child comes in with these symptoms. You also have to think about heart disease as a differential. I'm not sure if I've answered your question, but we can go through this in the end of the presentation. Yeah. Uh So moving on, like I was saying, with patent ductus arteriosis, it's usually your um oxygenated blood mixing with a deoxygenated blood. And here it's specifically from the aorta into the pulmonary artery. So it's often asymptomatic like most ayo heart diseases and the patent duc ductus arteriosis usually closes spontaneously. You hear this continuous crescendo, decrescendo, which means it goes high and then kind of goes low machinery murmur and it's heard loudest over the clavicle and it can present with those heart failure symptoms that we spoke about. And usually it's the prostaglandin levels that keeps the PDA open. So treatment wise, we give them drugs that help reduce the levels of prostaglandin, like indomethacin and ibuprofen, they may require surgery, but usually they tend to close spontaneously. And if they don't, if you give them the medication, it should help. So, moving on um atrial septal defect. So it's a defect in the atrial septal wall, quite self explanatory. So there are two types. It either happens in the septal secundum, which is the higher bit like you can see in that picture here or in the septal primum. Most of them are defects in the septum septal secundum, which is where the defect, it actually overlaps with the foramen ovale. One of the shunts, uh you can hear a soft ejection systolic murmur over the upper left sternal edge. And again, this is also usually asymptomatic, but they may present with um symptoms in adulthood like dyspnea, heart failure. The main thing to remember as well with atrial septal defect is they are at an increased risk of stroke as adults. Because when blood goes from the right atrium to the left atrium, it can pool in the left atrium and form clots which can then directly go into your brain. Um There is also a link between migraines and a patent foramen ovale. I'm not quite sure why, but apparently there is uh management wise, it's active monitoring and you might also then recommend that they get interventional radiological procedure like a percutaneous transvenous catheter closure. An open heart surgery would be like fourth line and it has lots of risks in itself and there's like a mortality risk of 5%. So less likely, but it is also an option and complications wise for ASD. The main thing to remember is stroke because of VTE risk and you can also develop af so moving on the third, um Ayo heart disorder is ventricular septal defect. So this is the commonest congenital heart disease seen in about 30% of patients. Again, usually asymptomatic. However, when you've got a large ventricular septal defect, they might present with heart failure and breathlessness. So like, like I've already said V ST is the commonest heart disease, but it's amongst the three Ayo heart disorders. V ST is the one that can likely lead to the patient having symptoms and it usually presents with a pansystolic murmur over the lower left sternal edge. And there is an association with Down Syndrome and Turners and it does close spontaneously. But if it is large and they do have recurrent symptoms, you might do surgery between 3 to 6 months, there is also an increased risk of infective endocarditis, uh because of which you might consider antibiotic prophylaxis. So I've talked quite a lot. So just to kind of summarize ayo heart disorders, there's three main types, atrial septal defects. Oh, sorry. Another question. Um Do they get a loud S two and V ST? They actually do. Yes, they do get a loud, do, I'm not quite sure why, but they do apparently do get a loud S two. It's also set on this slide. Um So like I was saying, there's um but II don't know beyond that why they would get a lou loud S do. Um So there are three main ayo heart disorders, atrial septal defect, ventricular septal defect and patent ductus arteriosus. Uh They're all usually asymptomatic unless it's either large or long standing out of these three VSD is the one that can commonly present with symptoms. Um And we've talked about the murmurs as well and the management. So, so moving on you on uh cushion asks if um the loudest too is due to developing pulmonary hypertension. Um I'm not quite sure. II don't wanna give the wrong information from my understanding, they get allowed us to anyway, depending. But I don't, again, I don't, I, I can have a look at it and maybe get back to you next week. But II don't wanna give the wrong information. So I'm not entirely sure. I'm sorry. So, moving on to um cyotic heart disorders. So before we get to teratology of fallow and transposition of great arteries just to briefly talk about Eisenmenger syndrome. So they usually develop within 1 to 2 years in large shunts or in adults. If it's a smaller shunt, they can develop quicker in pregnancy. And actually, the mortality is about 50%. So if there is a maternal history of a congenital cardiac disease, you want to get an echo. So just kind of briefly going over it. So if a patient or an infant has an ayo heart disorder, like we said, previously, multiple times, blood usually goes from the left to the right. But over time, when that happens for a long period of time, your pulmonary uh vascular resistance or your peripheral vascular resistance increases because of which then blood starts going from the right to the left. And when this happens, deoxygenated blood starts mixing with deoxygenated blood. Hence why the child can develop cyanosis. So even though they have an as cyotic disorder, initially over a long period of time, because of these pathophysiological changes, they can then develop cyanosis and usually they present with along with looking blue itself. They can also present with other symptoms like clubbing, a plethoric Rudy complexion because of polycythemia, which is when you've got increased levels of hemoglobin because the body is trying to um maintain those oxygen levels. Examination wise, they can have a raised G VP peripheral edema, right ventricular heave. So again, signs of heart failure and a loud S two. So, ideally, uh you want to prevent with early intervention. So even though ayo heart disorders are usually self resolving and not symptomatic, you want to make sure that they don't get to the stage where they develop Eisenmenger syndrome. Because once they've developed this syndrome, it's usually medically not reversible. The only thing that you can do is to get a heart lung transplant, but it has its own risks like high mortality. So, if a patient does develop Eisenmenger Syndrome, this is what you would do in terms of medical management. So oxygen, but again, that has nothing to do with overall outcomes. It's more just symptom relief in terms of treating the pulmonary hypertension itself. It's sildenafil, uh they might have arrhythmia. So you will treat them accordingly for that. For the polycythemia, you do venous section which is where you let them bleed and kind of get rid of the hemoglobin cells, um prevention and treatment of thrombosis as well with anticoagulation, things like Heparin Apixaban. And then you also want to try and prevent infective endocarditis with prophylactic antibiotics. So this is kind of the acute medical management. But when you develop this syndrome, it's not medically reversible. So moving on k um would people with ES and mens be on lifelong aspirin? I know. I'm not sure why. I guess with the thrombosis risk, I suppose you could be on lifelong aspirin. Um but in kids we usually try and prevent giving them aspirin except for one medical condition. Kawasakis because they can develop something I think called Reyes syndrome. So if possible, they might get other anticoagulation like Clexane. So heparin um Dox and things. Uh but I'll have to check whether you can give aspirin in eisenmengers specifically. So with other cyanotic heart disorders, teratology of fallow and transposition of great arteries. So they said, like we said, they develop synosis, but neonatal cyanosis usually presents in first week of life. And you can do a test called the nitrogen wash test where the infant is placed in 100% oxygen for about 10 minutes. And if the partial pressure of oxygen comes back, less than 15 kg pascal, you can be fairly certain, it's cyanotic, it's more than 20 you know, it's not cyanotic. But before you do this test, you need to make sure they don't have any lung disease and they don't have persistent pulmonary hypertension. Uh Again, you do also want to do blood gas analysis because oxygen saturations are not always reliable. And in terms, in terms of managing a sinus neonate. The first thing you want to do is stabilize their airway. So ABC airway, breathing circulation and then you might start, you, you will start prostaglandin infusion. So this is to try and keep those shunts. We were talking about PDA um Foramen ovale if they have maybe an ASD or A VSD defect open because that's what will help them survive. If they do develop SINOS, I'll talk about what I mean in a minute. And if you do give them prostaglandins, you also want to make sure they don't develop side effects. So you want to look out for things like apneas, jitteriness, seizures, flushing, hypotension and vasodilation. So with cyanotic heart disorders or if you see a child with blue skin, you do an echo and you think this kid's got um cyotic heart disorder, you want to stabilize the airways and then give prostaglandin infusion because that can help keep those shunts open and help them survive at least for the first few days before they can get surgery. So, moving on teratology of fallow. So there are four main defects you see with teratology of fallow, overriding aorta and it's moved more to the right as well. Uh The other thing is pulmonary stenosis or narrowing of that space in the pulmonary artery ventricular septal defect and right ventricular hypertrophy. So, teratology of fallow is where you see right to left shunting, which means deoxygenated blood is mixing with the oxygenated blood in terms of how severe teratology of fallow is. It depends on how stenosed your pulmonary valve is because if your pulmonary valve is not as stenosed, even if you've got mixing of deoxygenated and oxygenated blood, if the blood can get to the pulmonary artery and then to the lung, the severity won't be as worse. But if you've got pronounced pulmonary stenosis, then the severity of this condition would be worse and is associated with rubella. And other things you want to think about is maternal age, alcohol, diabetes. But the main one is rubella. When you do an echo, you also want to add on Doppler studies. Because for teratology of f, you want to know which direction the blood is flowing, usually picked up antenatally. Um it can present with an ejection systolic murmur which is loudest in the pulmonary area. And if you do a chest X ray, you can see this classic boot shaped appearance. So symptoms wise, sinois clubbing, poor feeding, poor weight gain. And they can also get something called tet spells when intermittently, they can have worsened symptom symptoms due to physical exertion, crying, waking. And the reason why this happens is usually what when you cry, when you are awake, when you exert yourself physically, your pulmonary vascular resistance increases and it puts pressure on the whole circulation system itself. Um And when you do get these tet spells, if they are severe enough, they can lead to reduced consciousness, seizures and potentially death. So in older Children, you might advise them to like squat and in younger Children, you might try and get the knees close to the chest um to kind of use the position to help relieve symptoms. So management wise, like we said previously, in neonates, it's prostaglandin infusion to try and keep the ductus arteriosis open. And the reason we're trying to keep the ductus arteriosis open is so that you can create an extra shunt for oxygenated blood to kind of get around the body. And the other thing you might think about is open heart surgery. But again, the mortality is 5%. But with teratology of fallow, it won't close on its own. So giving prostaglandins is more of a short term management. It's open heart surgery. That will be more long term. But however, if you do get corrective surgery, 90% of patients live into adulthood. Ok. So moving on um transposition of great arteries. So essentially what happens with this heart disease is your systemic circulation and your pulmonary circulation don't mix, which means you've got deoxygenated blood from the rest of the body going into the right atrium and right ventricle, but it goes directly into the aorta. So there is no oxygenation happening similarly, um with the pulmonary artery and veins as well, the blood from the pulmonary artery goes into the lungs, gets oxygenated, comes back into the heart through the pulmonary vein because of which again, you get right to left shunting and there is no oxygenated blood going to the rest of the body. Hence, hence why you get sinois. So this specific heart disease is associated with ventricular septal defect, but also cooptation of aorta and pulmonary stenosis. Uh the immediate survival depends on the shunt between the systemic and pulmonary circulation. So what that means is on top of the transposition of great arteries. If you've got another acyanotic heart disease, like an atrial or ventricular septal defect, that can help keep you alive because that creates this extra space for oxygenated blood and deoxy blood to mix. Instead of having these separate circulations a good way to think about it is, you know how I said in fetal circulation, your lungs aren't working. Hence why you've got these shunts that similarly in this condition, because you've got two separate parallel circulatory systems. If you've got these shunts, they help those two circulatory systems interact. They're usually picked up antenatally as well. And if they're picked up within the first few days of life, they present with sinois but also respiratory distress and poor feeding management wise. Similar to um teratology of fallow, you give them prostaglandin infusion to try and keep that PDA open, to keep that extra shunt open to help oxidated blood and de oxidated blood mix. Uh Definitive management would be balloon septostomy, which is when you insert a catheter into the foreman ovale and try and create an even bigger um atrial septal defect or the other thing you can do is open heart surgery. So just to summarize, um sorry, I've rambled on for very long. So please feel free to ask me to go back to slides or explain further if possible. Um Or if you want me to. So there's three main things you want to remember. Three main conditions you want to think about teratology of fallow transposition of great arteries and Eisenmenger syndrome. So all of them cause right to left shunting, which means deoxygenated blood mixes with deoxygenated blood. Hence why you get that classic blue presentation. Eisenmenger's usually happens when ayo disorders are longstanding or they're just large teratology of fallow and transposition of great arteries are those that start congenitally and usually you can give them prostaglandin infusion to medically manage for a few days, but they will need surgery eventually. Same. That's sort of the end of the presentation. A very quick whistle stop tour. Um Sorry if it wasn't entirely clear. Um I'm not sure if people have any questions at all before we move on because I think I did rush a bit. Did that make sense? Do you guys have any questions? Anything you want me to clarify? No. Are you guys happy for me to move on to the questions? Yeah. OK. So take your time to read this question and then um Christine, if you can get up the pole that would be useful. OK. Sorry, I'm still waiting for answers. I'll tell you when it starts rolling in. Yeah. Don't, don't worry if you guys don't know the answer, that's fine. Just try to work your way through and pick based on, um, what you can exclude. Um, we've got three. Mhm. Yeah, we've got threes. We've got threes. Ok. Very good. So that's the answer. Coarctation of aorta. So let's just go through the question very quickly. So, you've got a two day old baby that has been discharged home the day after delivery following a normal routine examination. So first things first, usually if they had a cyanotic heart disease like teratology or fallow or um transposition of great arteries that wouldn't happen, they won't have a normal examination and go home. So again, you don't have that here, but just to point he suddenly collapsed and was rushed back to hospital. So he was pale with gray lips. So just these symptoms collapsing, being pale and gray lips. Can anyone think of what kind of medical pathophysiology is being described here? What, what would the word we use be called for collapse, pale and presenting with gray lips or what do you think's happening? We don't really have to get into the cardiac side of things. Just um any responses at all. Christine, anyone saying anything? Um Not at the moment. That's OK. That's fine. It was just so ok, we've got pink pee poor blood flow shock. Uh Yeah. So that's what we were getting at like shock. So collapsing suddenly and that classic like gray lips or pale um look because of hypotension is symptoms of shock. So again, then moving on the right brachial pulse could just be felt and the femoral pulses were impalpable. So this again is a classic symptom or presentation of coarctation of aorta where on one side, the pulses are not as pronounced as the other. Um the liver again being significantly enlarged suggests um right sided heart failure. Uh blood gasses show metabolic acidosis which suggests that there's raised lactate levels because of hypoxia. So you've got um both breathlessness or like hypoxia and sinuses as well as shock. So when you get these two together, you're thinking it's an obstruction that's affecting the neonate. Hence why it would be coarctation of aorta. Uh Moving on. He was ventilated, blood cultures were taken and antibiotics prescribed because they're thinking maybe sepsis. Um blood and urine samples were taken for amino acid screen and urine for organic screen. Now, the reason they are doing this is to rule out inborn errors of metabolism. That is when the child has a rare genetic disorder where they're not able to process um whatever the food they take. So they're not able to metabolize food and to kind of see if they've got any um abnormalities with the uh genetic abnormalities. That is when you do that amino acid screen, you might also do liver function tests. Um And it's also said that the femoral pulses remained impalpable, but with prostaglandin infusion, they got better and within two hours, he was pink and well perfused. So if we look at the options itself, sepsis doesn't classically present like this because there's no kind of reason why he could have caught an infection because there's no signs of a chest infection, urine infection, abdominal infection, none of that in bone marrows of metabolism. If that was the case, you might have seen a positive amino acid screen or um organic acid screen. Coarctation of aorta. It might be coarctation of AORTA cos there's signs of shock. Um pulses are impalpable and complete atrioventricular septal defect. That is more of an acyanotic heart disorder, which usually doesn't present with um such pronounced symptoms and again, pain and doctor's arterio as we spoke about, it's Ayano patients shouldn't present with symptoms of shock. Hence why it's three. So moving on next question, sorry, I've got a tendency to ramble on. So feel free to pop in the chat and say you don't understand or stop if I'm rambling on too much. Have you got any answers coming through Christine? Not at the moment, but I'll let you know. OK, I think we're doing OK for time. So we've got five at the moment. OK. There we go any more. Got a three. OK. Anything else coming through at all? Mhm. Uh So it's mainly five, a couple of threes. Ok, fine. So we'll talk through that. So actually the answer is one. So just reading the question again, you've got a four year old boy with three episodes of shortness of breath, dizziness, fainting after running. So I think the key thing here is running because he gets these symptoms when he's exerting himself and he's otherwise. Well. So if I go back to just a second, so this slide, usually when patients are asymptomatic, but then they get exertional symptoms, you're thinking about an outflow obstruction in a fairly well child. So you're thinking about narrowing of your valves. So usually your pulmonary valve or aortic valve. So that's what's happened with um this child here. Um And in terms of why it would be pulmonary stenosis and not aortic stenosis. It's because the ejection systolic murmur is heard over the left sternal edge or not, right. So, if it was right, then it would be aortic stenosis, same as you see in adults. Yeah. And if it was atrial septal defect, there might have been some indication um of asymptomatic patient, but developing symptoms either when they are ill or over a long period of time, not specifically with exertion. So the next question, this is just one of those things you just have to know. Um I'm not sure if there's any way we can work it out with logic. So don't worry about it and just guess if you can't um if you don't know the answer, any answers coming through Christine. Um. Oh wow. We've got mainly two. We've got a three and a five. Oh, ok. Mainly too. That's very good guys. I, to be honest, um, I didn't know this till I was prepping for this session. So great that you guys know this. Um, it's 1/4 question. We've got three more questions. So if anyone has to leave, feel free to leave and we'll send you the slides, er, but if you've got a bit more time, um, feel free to do the questions. Um, you've got three for four. Ok. Any more responses at all? Uh, we've got a one as well. Ok. Ok. Are you getting any more responses or are we kind of done? Mainly one and three? Ok. Fine. So it is actually one. So rheumatic heart disease, essentially what happens is when patients have a streptococcal infection. So those who did say three, you are partially right, you initially have a streptococcal infection and then if you say have it repeatedly or sometimes you're just unlucky, you might then develop a autoimmune reaction to it. And that is what rheumatic fever is. So, rheumatic fever itself is not the actual infective period, but it's what you develop after as a hypersensitivity reaction. And if you've had rheumatic fever multiple times, you end up damaging your heart valves and that's what leads to rheumatic heart disease. Yeah. So when you acutely de and you can develop symptoms like myocard of myocarditis, endocarditis, pericarditis. So, yes, you are right. If you said three initially, you have that infection, but the actual heart disease is because of damage to the valves and it's irreversible and usually you need a valve replacement. So, next question, I think this, we went over in the presentation. So hopefully it doesn't take you too long to answer. Um Marks will this be covered? Next? Block in rheumatology? Um So I didn't do that. Uh They do cover, I think they ii mean, I remember, I think I, we went through it briefly but I'm not sure if you have a case on it, they will cover kind of the criteria for it and the different symptoms patients can present with. Yeah, but I'm not sure in rheumatology itself, you might have, do you have a case on pediatric rheumatology at all? Um I just meant like on the UK MLA. Oh yeah. So yeah. Rheumatic fever is probably the UK MLA. We don't go over an M SK. Yeah. So. All right. OK. I'm not sure what the question. All right. OK. Sorry. Um For question five, it's mainly three. but also two and four. OK. So like you guys, most of you guys said, so the commonest congenital heart defect is um ventriculoseptal defect. Um What is the most likely age an infant with a large ventricular defect might present with um heart failure symptoms? Again, this is what we sort of went over today. Any answers coming through at all? Christine. Um Sorry. So for question six, it's split the two top answers two and three and also um somebody thinks one. So, ok, so it is three. So about three weeks to four weeks is when they usually get it. Um One day you might see that more with cyanotic heart diseases. So those with um things like teratology of fallow transposition of arteries, uh not so much with um ventricular defect. So this is the last question. Uh This is a little bit tricky. I'm not sure if you guys have already covered this. So don't worry about it. If you're not aware, we can go through it but just attempt it. Um So people say an aspirin, OK. Um Fine. Um OK, we'll go through this. So I can, I can kind of appreciate why people are thinking aspirin. So just reading over the question very quickly, you've got a five year old boy who's come in with a persistent fever, strawberry tongue peeling skin. So that kind of desquamation of the skin and swollen lymph nodes. So, at this stage, what um diagnosis are we thinking about? Can anyone pop it into the chart? Um Kawasakis. Oh, very good. So, Kawasakis, um so one of the things with Kawasakis is you want to rule out coronary artery aneurysm, hence why he gets the echo. So, in terms of the prophylactic medication, you are right, you also give them aspirin. However, aspirin is to reduce the risk of um thrombosis itself. It's not for coronary artery aneurysm. So for coronary artery aneurysm, you give them IV immunoglobulins. Yeah. And as you guys have already picked up labetalol and clopidogrel not really relevant to this scenario. Um So that's pretty much the end of the lecture. So just kind of answering, I think I'm not sure if it was Kishen that asked that question about how you might get things in Aussies. So I think one of the stations that I'm not, I didn't have it, but the other badge in my year had it was to do a cardiology exam on a child. But you do the normal cardio exam. And then I think the question they were asked is if a patient of say, I think the child was like eight years old comes in with a murmur and is fairly well, then um what do you think is the likely cause? And the answer was innocent murmurs. Um So that you have to examine them after they've come out of the illness or the anemia is um resolved. And when I was doing my OSC, like I said before, it was a history from a parent of a child that's about a month old that's unwell with dyspnea, breathlessness. Um Those heart failure signs and you had to pick up it was congenital heart disease. You didn't really have to give a specific diagnosis just that it is potentially a congenital heart disease and you would do those, um, investigations. Yeah. Christine. Um, yeah, just ask a clarifying question just regarding the IV immunoglobulins. Um, just asking if they bind to IGA. So it doesn't bind to the endothelium of coronary arteries. You know, I'll be at the physiology of it. I'll be very honest. I'm not sure you do ask some very smart questions, but I'm, I'm really not sure about it. I can have a look. Did you say IG what, what was his, what did he ask again? I can't see on the chart. Just does the IV immunoglobulins bind to IG A so it doesn't bind to the endothelium of the coronary arteries. II can't, I can't tell you. I'm not entirely sure. Sorry about that. Do you have any idea Christine? Um It sounds smart because I know Kawasaki is an IGA mediated vasculitis and IV go off the IGA. So, but I don't know the exact um path of um does anybody have any other questions? Thank you so much for coming. Um Sorry if I couldn't answer some of your questions, it's quite specialist. So my knowledge is mainly limited to what I had to know in fourth year as well. I don't think I know anything beyond what I learnt then. So, um but yeah, thank you so much for coming. Do you mind staying on just for a second if that's ok? I'm just gonna wait for things to ahead. Thank you guys.