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I mean, okay. Oh, hang on. I just want to share the power point screen, don't I? Let's go here and share, right? Okay. Bear with me while I get back to the beginning of the presentation. That would be helpful, wouldn't it? But I'll start by introducing myself. My name is Caroline Delahunty. I am a consultant pediatrician, and I've very much the majority of my career has been very much neonatology. So I've chosen to speak to you today around newborn circulatory adaptation and congenital cardiology, which I believed in your curriculum. Can I just check? First of all, that? Everyone can see the slides and can hear me. Yes, ma'am. Fantastic. Okay, well, I'm delighted to be here today with you. And really, my thoughts are with you so much. Please feel free to interrupt me. And I'm happy to take questions at the end. I will if I could ask Austin to monitor my time because I probably have too many sides, but we can stop at any point. So if we start by thinking about newborn circulation, So I'm going to talk to you about in utero circulation and the changes will then go on to think about the normal anatomy and then common congenital cardiac defects. I very much focused on the physiology here in this lecture. Because if you understand the physiology and can relate it to the clinical picture, it makes it much easier. I've not focused on management. Okay, this is a very complex looking side side, but as labeled at the bottom, I have fetal circulation on one side and then the the circulation post transition on the second on the second half of the picture. If we look at fetal circulation now, right, OK, I've got a pointer. So from the percenter, I've left the percenter out from the percenter. Oxygenated blood comes in through the umbilical vein. Um, it is the only vein that carries oxygenated blood. It comes through through the liver through the doctor stenosis. It feeds the liver through the paddock branches and the Katic sign sides and then goes into the inferior vena cava to enter the right atrium. Now in utero. Because the baby is not requiring to pick up oxygen from lungs, but still need some blood to go to the lungs for to develop the lungs for al Viola development, blood is not only feeding oxygen, it's feeding nutrients. The they're the majority of the blood doesn't go into the right ventricle. Small amount does. The majority, as a result of equal pressures, goes from the right atrium to the left atrium through something called the foramen ovale. There is a whole basically between the two ventricles between the two Atria. Sorry. So the blood goes across into the left atrium down into the left ventricle, flowing here, then up into the aorta to feed the rest of the body down through the descending aorta. In addition, we have a feed here where the blood that is coming up through the pulmonary trunk is feeding through the doctor's arteriosclerosis. The doctor's arteriosinusoidal very important, uh, channel because it becomes very important in a lot of congenital heart disease, and we use it quite often to sustain oxygenation. I'm going to go on before I talk about so during the changes at Earth. The first thing that happens when the cord is cut is that the baby starts to a rate the lungs, it cries. You want a newborn baby when you cut the cord to cry, because by crying, they're opening up their lungs. They're pushing the fluid that's in the alveolus back into the circulation and allowing air to enter. As a result of that, the pressures in the pulmonary tract fall and pulmonary circulation is established. So now we're in a situation where, as in utero, the pressure's on the right side of the heart and the left side of the heart have been equal. The pressure on the right side of the heart is lower now because the lungs are open, so it encourages the blood to flow into the lungs. So the right ventricle, therefore, becomes a low pressure ventricle. It's only pump into the lungs. It's not pumping around the body. The left ventricle has to pump around the body. It's a bigger pump and that these changes allow some of the adaptations to take place. That membrane, and that whole, between the right and the left atrium, will go back to the picture in the minute closes, the foramen ovale closes and the duct, the patent doctors arteriosclerosis is as a result of the labour surgeon prostaglandins that's taken place and the hormonal changes that have taken place. So I'm coming back to this diagram we've now acted right? Finally, cursor, This is your newborn post delivery or your adult circulation. Okay, so this right ventricle is now pumping blood up to the lungs. It's bifurcating here. It's not feeding into the aorta. It's going into the lungs to pick up oxygen. And this membrane has closed because the pressure of the left atrium being higher has shut it as pushed. It closed. So these two flaps start to come together and to close to stop blood going across here. Because we now want the blood to come into the right ventricle and go into the lungs. And as a result of the prostate gland in surge, this duct closes as well. So there's various adaptations that take place. I hope that's clear, but I think as we go on, we'll understand further. I've given you a box diagram of the heart, actually, because sometimes people find looking at the red and the blue and all the shapes, the anatomical shapes difficult. Um, believe it or not, it's upside down because when we echo, we echo upside down, which seems rather odd. So you have your vena cava here, your venous return. You have a superior and inferior vena cava flows into the right atrium, flows into the right ventricle and goes up into the pulmonary artery. From there, it comes through the pulmonary veins down. Oh, lost marker. So don't worry. Comes pulmonary veins into the left atrium, left ventricle and out into their alter. So that's it all in a box format. I want to talk about how we classify congenital heart disease, and I don't know if anyone wants to put in the chat what they think about how you would classify it. Any thoughts? I'll move on because I've got a lot of slides. So you need to think about is the baby look at the baby? Is the baby blue or pink? Always a good start to use your eyes and see what's in front of you. The baby's blue that is cyanotic. Congenital heart disease, the baby's pink acyanotic and gentle heart disease. Right? We use our eyes, congenital heart disease. Believe it or not, it's quite easy. There's a lot of pattern recognition. It's not easy to fix, but it's easy to think about physiologically. The other type of congenital heart disease we can have is we can have rhythm disturbances. We can have babies born with a heart rate that runs too quickly. Too fast with a ventricle, a tachycardia, tacky arrhythmia. Or we can have babies born with a heart that is too slow with heart block and bradycardias. So looking at these, I'm going to list. And again, I could ask, Can anyone think of any pink heart defects? Acyanotic defects? The most common one. Now, I can't see the chat, which isn't very helpful of me. Okay, so there I've got a list. And don't worry about the list of acyanotic pink heart defects. The most common one is the one at the top, which is a ventricular sexual defects. The most common congenital heart disease in Children. Um, well, come to talk about it, the majority, ironically, get better without any intervention. You then can have very common also atrial septal defect. Patent duck. Just are curiosities is common in prematurity, but not particularly out with that. And then we're going to go on to talk about aortic stenosis where we have a were born with a narrowing in the aorta, which will affect the feed out to the body and can actually be very critical and can cause more death without being treated. Pulmonary stenosis, cooptation of the order and hyperplastic left heart. We're going to talk about these on the blue side. There aren't many things that meet blue, actually. But if you think about it, basically what I'm saying is you're either mixing pink and blue bloods together or you're not getting picking up enough oxygenated blood. So the only thing is that going to make you blue is if you can't feed into the lungs or you're mixing pink and blue blood together. So you look blue because you're mixing oxygenated and deoxygenated together and we have a list there. Don't go on to talk about these. Wanted to do this through cases to try to make it more clinical. I don't know if any of you have had any experience pediatrics yet in your medical school career career, so we have a three week old baby that comes in presenting with fast heart rate tachycardia, fast breathing. That's what you're going to see. The baby is probably going to look sweaty because of the breathing and the fast heart rate. Mom will tell you the little one is struggling to feed and hasn't been gaining weight because it's not feeding enough. Um, I've got to put a clinical thing in there, actually, with HEPATOMEGALY. When you examine the baby, you'll probably realize the heart is beating very quickly. You'll realize babies very breathless and working hard at the breathing. The chest will be lifting up and down because the chest is so compliant. We see the little ones sucking. It's skin in between. It's ribs. We call it intercostal recession. You don't see it so much in our adults, and we'll have a swollen liver. We'll have pattern megaly, and we may hear some preparations in the lungs with the lungs being wet. So this baby is showing signs of cardiac failure is in heart failure, which is exactly the same as cardiac failure and adults. And in fact, the management is the same. It's diuretics and fluid restriction, but we equally have to very much focus on nutrition and probably give a concentrated feed. Does anybody want to guess the diagnosis? When I've told you the most common cardiac condition, I'm going to tell you what we find. First of all, sorry when we listen to the heart. We hear a murmur now. A murmur is turbulences. You're listening to abnormal heart flow. So you're hearing turbulence. We hear a murmur, which is an additional sound from turbulent flow at the lower left sternal edge. It's pansystolic in that it's encompassing, encompassing both heart sounds. We can feel the right ventricular right ventricle heaving because it's working harder because it's receiving more blood than what it would normally expect. But the pulses are actually normal. Any thoughts? Any thoughts coming up in the chat Ventricular septal defect. Fantastic. Absolutely Well done, right? So this is diagrammatic picture of ventricular septal defect. So again, I I've put a normal heart up on the right, the left hand side of your screen as you're looking at it. And then I'm showing you a ventricular septal defect, A hole in the ventricle here. So here we have a break in the ventricle and what's happening? Because, remember, the baby has taken three weeks to present because the pressures in the lung have fallen further. The left ventricle has a higher pressure. I keep losing this arrow, Don't I write? My left ventricle is a higher pressure, so therefore it's able to push blood across the whole into the right ventricle, so the blood keeps recirculating through the pulmonary arteries, causing pulmonary congestion. There's too much fluid in the lungs, are getting congested, which is what's actually given us are symptomatic presentation. Now, if that was a small hole, you wouldn't necessarily get pulmonary congestion, but you would find the auscultatory findings of the murmur at the left sternal edge. Um, so it's the size of the hole that determines if you go into cardiac failure or not. So big holes go into heart failure. Small holes can be a symptomatic. Moving on to a second case, we have a child being examined for an inter current viral illness. Common cause, Common time When you find murmurs and they can often be innocent just because the heart's beating quicker, you get flu murmurs. But here we find that we have a murmur at the upper left sternal edge. We have fixed split second heart sound, but we have normal pulses. Okay, what I'm showing you diagrammatically. Maybe I don't have a proper picture, actually, is that there is a hole between the right atrium and the left atrium. So this is an atrial septal defect. They are usually picked up there a symptomatic. Actually, they're usually picked up. Coincidentally, they do need repair because eventually you will develop an Isa manga syndrome in adults where you will have had overload of over time, you will have developed overload and pulmonary problems and high pulmonary pressure. Pulmonary hypertension in adulthood so they don't become symptomatic and in adulthood. So if you hear a murmur when your examiner child, at any point when they've come to you with their coughs, their coles there diarrhea and vomiting, you need to follow it up to make sure it disappears. If it doesn't disappear, you need to refer for assessment. So what I'm showing you is that because of that shunt across the left atrium, we feed too much blood into the parliamentary artery and eventually cause pulmonary hypertension in adulthood. Okay, so case three is an X 24 weaker who we can't get off the ventilator. So we've probably treated the respiratory distress of the newborn, but we're stuck. But when we examine, we find a continuous often called a machinery murmur. It does sound like a machine just rolling on below the left clavicle. Heart sounds are normal, but we've got bounding pulses. There's only one thing this can be. Does anybody want to shout out what they think it could be? It's a patent. Doctors arteriospasm this. So basically, this doctor's arteriosinusoidal, which is supposed to close in the 1st 10 days of life, often closes by. Day three normally hasn't closed because the baby doesn't respond to The surge is a prostate gland, and they don't have the receptors to close their dot um, and basically, because the blood is feeding from the aorta back into that pulmonary trunk and back into the pulmonary into the lungs, the lungs get congested. We get pulmonary edema, and we can't win the ventilator because the ventilator is control in the pulmonary edema. So again, we would think about giving diuretics, but we actually treat them with ibuprofen, believe it or not, for three days to try to close the dark. Or they may require, to go for surgical ligation, to close the doc to allow them to come off the ventilator and progress through to survival. Okay, this could equally be an adult, although I've said a 13 year old you want to think about your footballers that collapse on the pitch. People collapsing during exercise. A 13 year old girl has collapsed while running for a bus. Very common, actually, because quite often you'll think, Oh, adolescents, they faint, you know, but past. But the the red flag here is that this collapse occurred during exercise. She wasn't just standing at the bus stop. She was running. She has a past history of getting out of breath on exertion, and I know you think asthma. But here it is important. When we listen to her test, we find an ejection systolic, so it's during sisterly. It's an ejection murmur at the right upper external edge. I haven't talked about Auscultatory positions, but it's the same as adults. So you have to think of where the blood is flowing and I'm going to give it away. And you see, you need to think about where the blood vessels are coming up. We hear a click clicks tend to suggest this valve. The valves are involved and they're stiff and they haven't to click, and the pulses are weak and you feel a plateau. Someone want to shout out the diagnosis. Pulmonary stenosis? No it's not, actually, and it's not because of where it's a very good suggestion. It's not because of where you hear it. You're hearing it on the right side. Um oh, gosh, you can't see my body, right? So if you think of the heart from the right ventricle, it's the pulmonary arteries Come up out the right ventricle. They go into the left side before they bifurcate the left ventricle. The aorta comes up and sweeps around the right to the left, down the back to go down the body. So the aorta comes up and the aortic valve is hered. An aortic stenosis is heared uh on the right side of the body. Pulmonary stenosis is heard on the left side of the body. Well, point that out. When we look at another diagram of the heart, the other clue here is it's stopping. The pulse is a week. You're not getting blood out to the body, so it's the aorta. So you have a narrowing of the aortic valve on echo. It looks like an apple core actually so narrowing of the aortic valve coming out from the left ventricle, we will come back to a diagram and I'll point out which will make your Oscar to tary point easier. Okay, so case five. This is a seven year old girl, very happy, very chatty. We often talk about them as being the cocktail party Children, Um, but noted to have a small elf like face. I don't expect you to get the syndrome. It's all right. She's developmentally delayed. What I'm pointing out here is congenital heart disease often goes in Children with that have syndromes during, uh, during the examination, it's the same story. You have ejection systolic murmur at the upper right sternal edge, and you've got weak pulse is she has Williams syndrome. I'm just highlighting if you have congenital heart disease, really. Look at that child. She may have a chromosomally problem. And in congenital heart disease these days in the UK, we check the chromosomes. Um, you look at her. She would have a thin lip, flat, mid face and a low nasal bridge. But as they grow, they really are the cocktail party Children. They happy they chat, but they've got learning difficulties. So it's important if you found a gentle, probably part problem. You think about that. They have the problems. Caroline, you've got about 10 minutes left. Okay? Okay. Okay. Um, this case here with the ejection systolic murmur at the left sternal area is your parliamentary stenosis. Takes you to that parliamentary valve and the pulmonary stenosis. This girl has a short web neck. When you look at her, can anyone think about shoutout what I'm thinking? Oh, actually, this one's a boy. Sorry. Right. So this is pulmonary stenosis. He's got Noonan syndrome when you look at him, so looking at him, you'll see his big ears. You'll see the shape of his eyes. He's got inner epic Catholic folds, and he's got quite down slanting eyelids and sort of full eyelids. And again, the space between his nose and his upper lip is large. And he's got a web neck when you undress him. And he's got wide space nipples. So it would make you think there is a chromosomally problem and you want to identify it or seek advice? Genetics, chromosomes. He's got Noonan syndrome. Okay, the here, you're doing a personal check, which we do on every baby. And you find a systolic murmur between the scapula. Normal heart sounds absent pulses in the lower limbs and swollen extremities. This is critical. You cannot feel the pulses in the lower limbs. You are not feeding blood to your femoral. Zell's Okay, so that's what you need to think. Well, I can't feel the pulse is not enough. Blood is getting through. This is a critical heart condition. That baby will die if we do nothing. That's why you must feel the femoral Xas part of your assessment. So she has an aortic cooptation. Equally, she could have critical aortic stenosis. There's two diagnosis here, isn't there? Well, there's there's three. Actually, the left side of her heart has a problem with outflow. So either your left ventricle, a pump is not working. You've got aortic stenosis, your valve can't open or you've got a narrow in in that aorta so physiologically, if you're not getting blood out the body, there's three things it can be. Your ventricle doesn't work. Your aorta doesn't work or you've got a narrow in in the descending or aortic arch. Here. It's a co occassion. Okay, so what I'm showing you on this picture. What's highlighted with the blue ring is that your ascending aorta has a narrowing you have a cooptation. Picture here, shows a child return a syndrome with the web neck, but I'm going to move on. So again, similar thing. We've got a baby that's born who becomes pale and shocked with poor pulses. So again, you're thinking this baby It could be septicemia. I agree. But if we're talking about cardiac, this baby cannot get enough blood out of its body. Can't sustain it's BP here. I'm showing you a picture to schematically demonstrate that the left ventricle and the aorta, the LV and the a o are small. They're not developed. There is now repair for a hyperplastic left ventricle, actually, but it's multiple stages, and they have to go to a specialist center. But they don't all die the way they used to. Okay, I'm going to move on to watch, and we can always come back. Okay, so this is a common one. We have a seven month old baby previously noted to have a protruding tongue. Platin know. So you're looking at the bit newborn baby, and you're thinking this baby has dysmorphic features, slanting eyes, epic Catholic folds, short broad hands and a single Palmer crease. So I'm going to not ask, but you're thinking down syndrome she presents with poor feeding becoming breathless, she's puffy and she's got a fast heart rate, so she's gone into heart. Failure is very similar to about first case. She's out of puff. She can't feed on her heart's beating quickly because she's so congested. Clinical features of down syndrome on the picture. Um, that tongue sticking out is very common, and it's a clue looking at her antenatal history, because it's almost important when here you have a detailed 20 week scan, which is aimed at identifying antenatal cardiac lesion's, and this is one it would lead to. Um, you can normally pick this up anti in Italy, but postnatally you would. It presents within with heart failure in the first few months. There may be no signs because if the whole between the atria, the ventricle and the ventricular septum is so big and you've got so much mixing, you don't hear turbulence because it just all freely mix is making her eventually blue. So they get an A B s, a V S, D and atrioventricular septal defect very common in down's all down syndrome. Babies should routinely have an echo at birth or within the first fortnight to exclude this. It's a defect of how they're endocardial. Cushion has formed. It leads quite often to just a single valve. They don't have your separate tricusp id and mitral valve. They can have mixing across the atrial symptom. They can have mixing across the ventricular septum or it can mix across both. They can have both defects, and they can have other abnormalities. So here, schematically at the top on a you've got, um, whole here between your atria. A hole here between your ventricle. Okay, so they have a separate, but they've got normal valves. Actually. Hear your valve is too low and you've got a big hole, A big hole between the atria and ventricle. It's very much more atrial looking at it. Here. We've got a big hole in the ventricle and the valve is too high. So that's an atrial ventricular septal defect. They go into heart failure. They all require feed in support. Concentrated feeds, fluid restriction, diuretics. Before you surgically correct the defect, they try to take them to about six months before they surgically correct them. But it very much depends on our ability to manage them with diuretics and feed in all gentle heart disease. Diagnosis are made with the use of echocardiogram. We've not talked about investigations, but the same as adults. Chest X ray and echocardiogram. I am going to come. Okay, I got five minutes. Yes, yes, I think so. Ok, ok, we'll go back into another one. Um, actually, I'm gonna go back a few places and do a really interesting one. You can see I get excited over these things. Okay, Right. Transposition of the great arteries. I've told you the diagnosis before. Right turn, baby. She's blue immediately. There's not many things that make you blue immediately after birth. But if you think about it, you're obviously not getting blood to the lung. Okay, so you're not picking up oxygen. There's no respiratory distress. She's very comfortable. Probably because you're not actually had time to go into any form of heart failure anyway. But if blood is not getting to the lungs, if you think about it, you won't really go into heart failure. Um, because there's no congestion taking place. She's very comfortable, and you can't hear a murmur. I don't expect you to get this. So if you've got a blue blue baby with no murmur and no respiratory distress, there's actually only one thing it can really be. Do a X ray because we like our rights rays. And what we're seeing is something called an egg on the side. So if you think about that is supposed to be an egg, I've never been 100% convinced she has been intubated. She's got an ET tube sitting at the top because we always want to protect the airway and try to optimize oxygenation by taking over the breathing. But we go trek Oh! Oh, I think something's gone wrong. Here. Hang on. Sorry. What is the right picture? Sorry. Right. OK, OK, we go to Echo, and what we find is that the arteries have switched over. What I'm showing you here is that the aorta is coming off the right ventricle. Okay? It's not coming off. The left is coming off the right. When we follow that round, we follow around an arch and the descending order and off the left ventricle. We've got the commonly artery coming off, which then bifurcates to follow through to the lungs, so we track it through so the arteries have switched over. It's called transposition of the Great Arteries. It's a fantastic thing on echo because it's very characteristic. Um, so the water is coming off the right side of the heart, the pulmonary arteries coming off the left. So unfortunately, they're not the and the problem is the veins from the pulmonary artery continue to go back into the left atrium. So you've got a parallel to parallel circulation's, actually, So the oxygenated blood just keep circulating round into the lungs. It doesn't go out around the bodies. The baby's blue. So we have to We have to Do we have to keep that duct open to try to get some mix in to try to get some red blood to go down the aorta. So we give them a drug to maintain the patent, see a prostate infusion. This is lifesaving. Ideally, what you really want is that nature has been kind to us and put a hole between the two ventricles and given them a ventricular septal defect which will allow mixing okay and give us some pink blood going into the blue and up around the body. You also don't want the form and a valley too close. So what you do is you transfer them urgently to a cardiac center. They feed a balloon into the heart, and they rapture this this membrane to stop it close in. It's called an atrial septum. Eah, Septostomy. It's done with the balloon. Okay. And then they will plan to switch these arteries over and being very simple here. But so this to the right ventricle. And so this to the left ventricle before, Before they do that, they've got to make sure this left ventricle is strong enough to pump around the body. And if it's not, they don't do that. They allow the baby to remain blue, and they tie a band around the pulmonary artery to keep the left to strengthen up so that this poor left ventricle gets resistance training before it switched over. It's called an arterial switch procedure. Right? I'm going to I'm going to stop here. You can see you can talk for hours on this, but I think what I want to emphasize is don't be frightened by gentle heart disease. Think physiologically and I've tried to give you a very physiologically based talk. Relate your clinical findings to your pathology. And I always say, Learn from every case. If you've examined a baby or a child found a murmur, find out what they found and always refer for specialist opinions. If in doubt. Okay, any questions? We don't have any questions in the chat. Yeah, but now I should be able to see the chat, shouldn't I? Oh, hang on. Okay. The chart up. Okay. Just Well, while we're waiting, if anyone does have any, any questions? Please, Please, please give us feedback. We rely on the feedback to keep the medical. The online medical school going. I've put a link in the chat. Um, and the the it's pediatrics lecture. Um, with doctor Caroline Delahunty, third of November 2022. But all those, all those details that are in the chat. Um, if you just bear with me, I'll try and transfer, uh, the certificate over for those of you who need verification that you've attended, uh, lecture. But I'll try and put that in the chat. Now. Thank you for that feedback. Honestly, because I keep worrying that pediatrics, when in the situation you're in just doesn't seem your priority, but it's such a fabulous specialty. I can't recommend it enough. I have been I've been a consultant in 21 years, and I felt very privileged to work with parents, Children and babies because it's the most precious thing in their life. It's amazing. Specialty. I'd love to encourage you Will. Okay. And, uh, I saw there was a question about the certificate. I'm just uploading that two. The chat Now I hope I'm right. Um, Austin, I think it would be beneficial if people did the feedback first and then right at the end. Yeah, well, I've already put the the, uh, feedback links in the, uh, in the chat. So if we give them another five minutes or so to do that and then post certificate. Okay. Thank you. And again, if anyone has any questions, please put them in the chat or, um, mute yourselves. We have a few more minutes left