Computer generated transcript

Warning!
The following transcript was generated automatically from the content and has not been checked or corrected manually.

We'll start in about five minutes all right. I think we're pretty ecstatic, so we'll just start now, so yeah my name is Tanya, I'm gonna be f tooth at Wigan Infirmary and I'm going to talk about pediatric cardiology. Just a bit of an overview various different conditions. It's not all the conditions but just some of the common ones that you might be examined on, so just to start off with just going to discuss this a bit about the background, so fetal circulation, and what kind of adaptations that fetuses made to allow sort of good oxen, ization levels and um good some abilities to get rid of waste products and that can also lead on to what kind of congenital conditions we can have into a newborn babies and foetal cardiology essentially so um you have obviously you don't have a working pulmonary system or good developed lung system, so you have to have adaptations to be able to collect oxygen and to get rid of nutrients um to get rid of the waste products as well, so we have three adaptations commonly which are the three fetal shunts. So this sense essentially, Abel's blooded, bypass the lungs um and kind of former for circulation, so you can get the auction levels that you need, so you have the doctors uh read that's by myself a diagnosis, so it can can connect the umbilical vein to the inferior vena cava um so that basically means that you can bypass the liver, so the umbilical vein you can see on the right time, the the kind of the circulation and how well it's formed from that front. So you have the presenter, the blood goes from the present into the umbilical vein, bypasses the liver through the Doctor stenosis and goes into inferior vena cava, and then the blood goes from inferior vena cava to the right atrium. There's another another shunt from that point, there's the four main forum in a valley and the doctors arteriosclerosis, so the foramen a volley connects the right atrium to the left atrium, so essentially you can bypass the um sort of the right ventricle, the pulmonary circulation, and then you have the doctors arteriosclerosis, which contains, connects the pulmonary artery with the aorta so that can again bypass the pulmonary circulation at birth. Essentially when you have the first breath that allows the uh viola to expand, this decreases pulmonary vascular resistance, so that leads to a foreign the pressure of the right atrium and therefore the right atrium has less pressure on the left atrium. The left atrium can uh blood flows between the left issue to the right atria leads to the closure of the frame and a valley, so the pressure changes that you get with the first breath causes the the closure of the frame and a valley. Then you have um the first breath also increases the oxidation levels, which then causes prostaglandins in the future circulation to drop that causes the closure of the Doctors arteriospasm and then once the umbilical cord has been clamped at birth, that causes blood flow to stop through the umbilical vein and that closes the doctor stenosis shunt as well. So then as you can see, there's different kind of shows and different adaptations, so these can lead to congenital abnormalities in the cardiac circulation essentially as well. And overall they kind of split into two different categories. Two broad categories, so you have cyanotic heart disease and you have a cyanotic heart disease, so cyanotic heart disease as you can imagine is that you have high levels of deoxygenated blood in the systemic system and that can lead to cyanosis and this is usually caused by a right to left shunt, so essentially you have blood basically bypassing the lungs and going directly into the systemic circulation Rather than going through the lungs and what conditions are associated with these, the most commonly the trelegy of pallet and the transmission of great arteries. Then you have the a scientist say cyanotic heart disease, this is essentially left to right heart patient, so this is an obstruction beyond the lungs, so you still have high levels of oxygenated blood in the system, lower levels of deoxygenated blood in the circuit, systemic circulation, so you don't get cyanosis and the conditions are associated with this. Rvs, d. A. S. D. P. D. A. So VSD is ventricular septal defect, ASD, s. H. O, septal defect, pedia is patent doctors arteriosus so that's one of the ones that we talked about here. The doctors are your aosis and coarctation of the aorta, so these are kind of the ones I'll be talking about here. There are the heart conditions as well, but these are the most commonly tested, so two site off with cyanotic heart disease. You have tetralogy of Fallot, so this is a condition that's made up of four different pathologies you have VSD, ventricular septal defect, you get an overriding aorta and you also get pulmonary valve stenosis and right ventricular hypertrophy, so I'm going to talk about the pathogenesis of this as we go on, but some of the risk factors and some of these risk factors are very common with all of the cardiac abnormalities, essentially so, maternal infection during pregnancy, especially with rubella, increased aged mother, alcohol consumption, and pregnancy and gestational diabetes all increase the risk of developing this condition, so the pathogenesis of totality of fellow, so he has the four different conditions that we talked about, so the v sds ventricular septal defect. Essentially, you have a hole between the two ventricles, so a septum so that allows blood to mix between the left ventricle and the right ventricle and overriding aorta. Um So essentially what this means is that you have the ventricular septal defect and the aortic valve is directly above the VSD. So what happens is when you have contraction of the heart. The blood flows directly from the right ventricle through the aortic valve into the aorta, so essentially have kind of shunting the blood directly up through there rather than going through the left heart side. Um alongside you get the pulmonary valve stenosis as well, so again that promotes more blood, so if you have stenosis of the pulmonary valve, it basically means that more blood is shunted up towards the aorta up through the overriding aortic sort of arch and into the aortic system, systemic circulation. Um All of these, this base, all of these conditions cause pressure and strain on the right side of the heart, right side of heart muscles and you get right sided heart, have muscle hypertrophy so all of these things kind of essentially can cause heart failure as well and then this causes the right to left cardiac shin leading to the cyanosis so presentation, so most of the time it's present picked up during sort of antenatal scans, uh but you can also be picked up in the newborn baby check. Um So when you're doing um the newborn baby check you listen to the baby's heart and you can sometimes hear an injection systolic murmur and that might kind of prompt you to having sort of further investigations as to why this is why this um murmur is present. In the first place, we will also get cyanosis as it's a cyanotic heart disease and then as you get older, you might get things like poor weight gain, poor feeding, failure to thrive, and you might also get something called tet spells. So tech spells essentially are sudden worsening of the condition, So you get these symptomatic periods, where the right to left shunt to become significantly worse and that's usually either because there is an increase in the pulmonary vascular resistance or a decrease in systemic resistance, so for example, there might be a child who's exerting themselves crying or you're usually crying because they're quite young at this point, then they won't be walking around exerting themselves crying um that leads to a build up of uh CO2 and CO2 is a vase a dilator so that reduces systemic um resistance because it causes systemic razor dilation, and then that can lead to sudden periods of cyanosis, shortness of breath, and in the severe cases can lead to loss of consciousness, seizures, and potentially death as well, so investigations investigation for all of these conditions the gold standard is to do an echocardiogram as well just to see what kind of going on and that's how you'll be able to diagnose a lot of these conditions. That's a gold standard. Essentially, you can also do a chest x ray that might show right ventricular hypertrophy a sort of a boot shaped but that's not really diagnostic the investigations. That's gold standard and diagnostic would be an echo, so management in me, in eight you can give prostaglandins so that can maintain the doctors arteriosus, so it keeps that open keeps that shunt going, so that allows the blood to flow from the aorta back to the poor moriarty, so you can get a little bit of um approximation but the mainstay surgery is open heart surgery and kind of surgical repair of this. Um If someone comes in with a test spell um this space you kind of have to treat them as an a. T. V. Examination, to give them oxygen. Um you can give beta blockers that can relax the right ventricle, give them iv fluids that increases the pre pre load increases blood flow, um morphine Again that's just to sort of decrease respiratory drive, sodium bicarbonate that you get a compensate or metabolic acidosis uh and you can also give adrenaline to essentially increase the vascular resistance, so that's kind of helping with the the test spells that we discussed earlier. What kind of these symptoms are, so you but want to cause the opposite of what resulting in the text bell, so you want to increase um systemic resistance as well, so the adrenaline can help with that and that's basically it so that's tetralogy of Violet, so you have the you have the four pathologies that we discussed. The presentation can be cyanosis, phase, thrive, and heart failure symptoms um and usually it's picked up with an echo and you can get the mainstay of treatment is open heart repair, such surgery essentially, or we can give prostaglandins while you're waiting for surgical repair and then moving on to transposition of the great arteries so that is a second um cyanotic heart disease that will be discussing so transmission of the great arteries. The definition of this is you have the aorta and the pulmonary trunk are swapped, the attachment to this and that sort of the normal anatomy of the heart to the right ventricle pumps blood into the aorta and the left ventricle pump pumps blood into the pulmonary vessels, so you essentially get two different circulation's that don't mix essentially so you have the one circulation that's traveling through the systemic system, and that's the right side of the heart is travel, transferring, traveling through the pulmonary system, so you don't get to make sure that of the of the blood circulation, so you get cyanosis because you don't get good oxygenation of sort of oxygenated blood flowing through the systemic circulation and there are conditions that can be associated with this as well, so ventricular septal defect. Cooptation of the aorta and pulmonary stenosis can also be all be associated with transposition of the great arteries as well. So you have a presentation. Again, this is often picked up during pregnancy with antenatal ultrasound scans, but again least can cause cyanosis or cyanotic heart disease and similar presentation to the other tetralogy of Fallot, essentially so respiratory distress, tachycardia, poor feeding fairly thrive for poor weight gain, sweating, heart failure symptoms very similar and then again the investigation, gold standard investigation for this would also be an echo so management, um so you can if they have a patent doctors arteriosinusoidal, if they do have a ventricular septal defect that can initially compensate because it can help blood to mix between the two sifrit circulation's, prostaglandin infusions can be given as well to maintain the doctor's arteriosus. Again, that's helping to mix the two different circulation's or you can do open heart surgery, which is again definitive management as well, then we're moving on to a cyanotic heart disease. Um If you have any questions, you can just pop them in the chat as well, and I can answer them as we go along, so, acyanotic heart disease, so that's again so when you're not having the cyanosis side of things, so that's an obstruction past the lungs, so uh one of the conditions patent doctors arteriosclerosis, so definition of that is that basically you have the shunt initially in the foetus, you have the doctor's arteriosclerosis and that remains patent. Normally, this can stop functioning within 1 to 3 days of birth and within 2 to 3 weeks of life, we can close completely uh if it does fail to close, it leads to patent Doctor Sartorius. It was one of the heart disease, so genetics is one of the risk factors. Again similarly, rubella infection, so maternal rubella infection during pregnancy can be a risk factor and prematurity can be a risk factor. Risk well, so pathophysiology of the, of pd, A essentially pressure in the aorta is more than the pulmonary vessels, so basically the blood flows from the aorta to the pulmonary artery and that leads to a left to right shunt, so left to right shunt increases the pressure in the pulmonary vessels causing pulmonary hypertension that leads to right sided heart strain that leads to right side of ventricular hypertrophy and that can lead to a bit left ventricular hypertrophy and as a result of heart failure, so presentation so this can cause a murmur and the newborn baby examination so that leads to a continuous crescendo decrescendo machinery murmur, so that's very classic that's a buzz words and sort of examinations when the we have sort of mcq, uh continuous crescendo decrescendo machinery murmur would lead to is associated with the pd. A. Newborn babies, um shortness of breath, difficulty feeding, poor weight gain, um and repeated sort of respite lower respiratory tract infections might start making you think is it pedia as well. Investigation. Like I said again it's an echo that's a gold standard and the management so you can usually sometimes well sometimes you can monitor it for the, for the first year of life, just using echos um So as you're gonna every month or every other month, you can do an echo and see how they're getting on and see what if they're developing any symptoms essentially, but after a year, if this is not resolved by itself, um it would require surgical closure and surgical intervention at that point, but you can monitor it until the first year of age, then moving on to a ventricular septal defect so that's another branch of acyanotic heart disease, so ventricular septal defect is like, I mentioned already a little bit is essentially you have a hole between the two ventricles, so you have the septum in the middle and you can have a hole between these uh the two ventricles that can cause the blood from the left side of the heart and the right side of the heart to mix. Now, the size of the, the sort of the settle defect can can range. It can be um the entire septum can be degenerated, or they can be a very small one. Um Risk factors is often associated with Down syndrome and turner's, so that's something that you have to kind of, if someone if, if a child has been diagnosed with downs or turn ish, have to investigate them for b. S. D. S. As well so pathophysiology of it. Um So you have again increased pressure on the left ventricle typically flows from the left side to the right side, so you have because you have a sort of larger left ventricle and you have a large muscle side on the left side as well. Usually, this causes blood to go from the left side to the right side, so then it causes a left to right shunt so that can lead to sort of right side overload. Right sided heart failure, right ventricular hypertrophy, um and then that can lead to pulmonary hypertension as well. So again all of this all of this kind of put together so increased right sided sort of pressure. Right ventricular hypertrophy, pulmonary hypertension can lead to something called Eisenmenger's syndrome. Essentially this is where a left to right shunt forms, becomes a right to left shunt and cause cyanosis and that's usually because you have right sided heart pressure, increased pressure and that leads to that can be either due to the muscle itself hypertrophy or pulmonary hypertension leading to right sided muscle hypertrophy, so again it swaps the shunt around so that's when Eisenmenger's, that's what Eisenmenger's syndrome is it's just a swab from the left to right which is a that is associated with a cyanotic heart disease and then it switches to your right to left and that can lead to cyanosis and eventually can lead to soar cyanotic heart disease as well that is very rare, though so presentation. So again initially depending on the size of it can be symptomless. Um If it's quite small, it doesn't have to can be monitored and watched um and can close spontaneously, but again it can be picked up during antenatal scans or newborn baby checks with the murmurs but again similar symptoms you can have with other heart conditions is poor feeding, failure to thrive, um difficulty and sort of shortness of breath and destiny and tachycardia um and then the murmur that's commonly associated with VSD is pansystolic murmur most commonly heard on the left lowest border so that's what you'll hear that's kind of the buzzwords for VSD the in the examinations of my baby presenting the pansystolic murmur as usually a VSD management so like I said um small VSD s can be just monitored and they can just close spontaneously, but if it's not, if it's not resolving, but again the age of one you might need open heart surgery as well and you have to be careful because they can be, they can have an increased risk of infective endocarditis, so they have to be if they're having surgery, they have to have prophylactic antibiotics alongside it as well, next slide all right, and then moving on to the question are there. Any factors that may make a yes, so depend on the size of the VSD, essentially so, if it's quite big if it's involving the entire septum, it's very unlikely to close. If it's quite small, it's not very well asymptomatic and very small, it's more like most likely to close spontaneously, um but depends on the size of it, essentially imagine there's a septum between the two hearts. The whole, the bigger, the hole is essentially the heart. It will be for it to close spontaneously. If it is present a lot of the children that can a lot of children that are born and they have newborn baby. Examination, then you hear a murmur If they do have a VSD, it's quite if the murmur is quite pronounced it is you'd imagine the the defect being quite large. Um If you can barely hear a murmur or a lot of times, you might not be able to hear one because it's so small and that might just go spontaneously as well, so that's usually what kind of factors naked and less likely to cause spontaneous the size of the septum defect. I hope that answers your question, so you have the atrial septal defect so again similar to a ventricular septal defect, it's just in the atria now so you have a hole in the septum between the two atria, so again causes blood to mix, so again similar to the ventricular septal defect, you have higher pressures on the left side of the heart, so that causes blood to flow from the left atria to the right atria um leading to a left to right shunt, which then leads to blood continuously flowing to the pulmonary vessels and the lungs to get vaccinated. So therefore you do have a sand acyanotic disease because blood is still oxygenated, um again increased flow to the right side leads to the right side of overload, um right sided heart straining and then right sided heart failure, and then again this can lead to Ice Mangga syndrome as well because of what we said before, right sided hypertrophy and increased pulmonary hypertension due to the increased blood flow from the left side to the right side, can then reverse the shunt, so instead of it being a left to right shunt, it can become a right to left and cause cyanosis so that can lead to ice mangga syndrome as well, so there are different types of atrial septal defect and this is austin is the condom is the most common you have the patent foramen ovale. Because that's when that the one of the shots that we mentioned initially fails to close and you have the austrian premium, which is the least common. I don't think you need to know that in detail to be honest with you. That's just um just for information, so the most common associated murmur with a. S. D. Is midsystolic crescendo decrescendo murmur. Um That's what's most commonly associated. So in, in reality, when you're doing a newborn baby check and you listen to the heart, you're not going to be able to tell exactly what kind of murmur. It is, so you'll be able to hear a murmur most of the time unless you're amazing and you can tell between the different kind of murmurs in a newborn baby. Most of the time, they're screaming anyway, so it's difficult to listen to the heart. So once you do listen to the heart if you just hear a murmur, you'd be referring them to get an echo and referring into pediatric cardiology anyway, but this is for example purposes you will hear a midsystolic crescendo decrescendo amendment um most of the time. Again, this is also picked up during antenatal scans or newborn baby examinations, but it may be a symptomatic in childhood, um But this is one of the sort of interesting cases where if it's quite small, you can actually go on to adulthood without any symptoms, but in adulthood it might present with dyspareunia, heart failure, or stroke, So what's interesting about this, which I'll come onto in a second, actually is it can lead to a stroke in a young adult um and I'll come onto that in a second, but yeah there's some of the other symptoms, it can have a shortness of breath, difficulty feed it in poor, bake in, in failure to thrive, and repeated sort of lower respiratory cuts, infections as well. So investigation, gold standard, doc, said echo and management. So if it's quite small similar to a. B. S. D, you can just do watchful waiting um It might close spontaneously, open heart surgery might be required, and that's kind of the definitive treatment for it and you can give anticoagulations to help with the complications which I'm going to go on to now. So like I was saying you can get a stroke in a young adult with ASD. So if it's quite small the ASD and they don't there's not many symptoms at childhood. They can present with symptoms in adults and young adults especially and it can cause paradoxical strokes. So essentially when you think when you when we get a DVT so someone without an a. A. S. D. When we get a DVT um it's most likely to form an embolus and go into our pulmonary circulation and that can lead to a. P. But with someone with an h. A. Septal defect. Um If they get a DVT, the embolus can actually go into the left side of the heart through the septum, so because of the defect in the septum that can go from the right side of the heart to the left side, and that can lead to a natural stroke as well, so um There might be a case of giving some anti coagulation too young adults to reduce risk of clots and strokes as well um can cause upon your hypertension and right sided heart failure as well and lead to like I was saying Eisenmenger's in room as well with ASD so that's quite interesting for that fact, so it's just if it's quite a small, it might not present insult childhood it can present in adulthood as well with you know, early onset heart failure or a DVT leading to a stroke. Kind of thinking of, didn't have congenital heart defect, they might want to do an echo for and then moving on to cooptation of the aorta. This is the last condition I'm going to talk about because there's multiple different ones, but these are the most common ones. So coarctation of the aorta is a common asymptomatic heart disease, so it's basically where you have there's a narrowing of the aortic arch usually around the doctor's arteriosclerosis, so this is commonly associated with telling a syndrome um So, if anyone, if you have, if you notice as a child and you would think so when you're on the postnatal ward, if you're doing things like baby checks. Um If you're suspecting if in the antenatal scans, they said there might be a genetic you know condition that's such a down syndrome or Turner syndrome. Um These children do have to be monitored because they can get congenital heart defects. Um Cooptation of the delta is one of them and this is one of the one of the conditions of sort of the heart conditions that you kind of can sometimes pick up on a newborn baby scan rather than an antenatal scan, So the risk factors like I said associate with Turner syndrome. Pathophysiology is you just get the narrowing of the a autumn, so it reduces the pressure of the blood flowing to the arteries that distal to the narrowing, but then increases the pressures um in areas that are proximal to the narrowing, so increases the pressures in the heart and the first three bunches of the heart as well. So in this, the presentation so you can get weak femoral pulses and newborn baby examination. So this is exactly why we help eight the femoral pulses, so when we do a newborn baby examination, there's a full head to toe examination that we do. Um This also includes palpating for femoral pulses, um and if they have quite weak femoral pulses um that's one of the red flags that you be thinking of do they need to get an echo. Do they need to be tested for cultivation of the autumn. Um You can do four limb BP um As well not commonly done, we can do um so this is where you get high BP in the limbs supplying um supply from the arteries that come from the narrowing and lower BP and limbs that come after, so limbs that come, that are supplied by artery's come coming after. The narrowing can also get a systolic murmur um and some of the poor feeding gray or floppy baby or left ventricle fever As well so when you kind of put your hand on the left side of sign, you can feel the heart palpating against your hunt. Um These kind of things maybe make may think you especially with the femoral pulses being quite weak. That's one of the most common um presentations when you're doing the newborn baby, which I can kind of make sure that that's um that's still present, femoral pulses are present and they're quite strong. Management. Again, investigation for this will also be an echo as well like for any other heart commission. Management of this is more cases that they can sometimes a lot of the children can live symptom free until adulthood and that's when they start to get very sim, similar symptoms to what I mentioned before is things like this veneer, tacky cardia, shortness of breath, and early onset heart failure. If it's a severe cooptation of yale to we'll need surgery, we can also give plastic landing e um that can keep the doctors arteriosus open, but that's not a long term, so management is just until they can get surgery, so that is an overall sort of summary of some of the pediatric cardiology conditions. Um I've gone through them quite quickly, but the main thing you have to remember is that you ask they basically split into two broad categories so if I go back, it's acyanotic and cyanotic cart disease and a cyanotic heart disease, so acyanotic heart disease. Because, because you have a left to right shunt, um a sanity cart disease can become cyanotic. If the left, right shouldn't um swaps around because you get right sided heart failure and that's what's that's known as Eisenmenger's syndrome and then there's cyanotic heart disease, which is a right to left shunt, so that's because the blood is bypassing the lungs, you're not getting oxygenation of the, of the blood and therefore you get systemic high levels of um the oxygenated blood in the systemic circulation and therefore you get cyanosis. The most common associated conditions that the strategy of fallot and transposition of the great arteries and the most commonly associated conditions with a cyanotic heart disease are atrial septal defect, um ventricular septal defects, peyton, doctors, arteriospasm and cooptation of the also there are other conditions as well, so things like pulmonary valve stenosis and aortic valve stenosis as well, but I'm not really covered them today um just because I wanted to go through the most common ones that are examined that I found anyway. Um. And four of these conditions the mainstay investigation would be an echocardiogram and treatment can differ between kind of different conditions, Some of them you can monitor and watch and wait um but for most of them if it's symptomatic and if it's causing so um things like fairly to thrive and poor weight gain and children open heart surgeries. Usually the mainstay treatment for them as well okay, is there any questions This is a really quick one just to do um an overview of the congenital heart disease is and there's there's not much to it. Really I mean you just have to think of where is the blood going is it going from the right side of the left to the left side, or is it going from the left side to the right side, and that's usually kind of what it is. I mean we were taught with this by one of the pediatric consultants when I was in the pediatric um um one of the cardiology specialist and he did in about 10 minutes on a piece of paper, so it's very simple to remember, just know explain to two different conditions is there cyanosis is they're not cyanosis um and just you know which one's kind of correlate with which any questions yes. I think remembering as well about the paradoxical stroke um in a. F. D. Is quite important as well um It's something that can be on your differential. If a young person comes in with a stroke, a young adult who comes in with a stroke. Obviously, there are the conditions associated with us could be one of the differentials as well, okay, yeah so this is the paradoxical straight brilliant. Um If there are any questions, I think if we can fill this feedback form, I think um uh huh that be lovely. I think we'll do a few more pediatric based talks. I think I've done one already which is um newborn baby examination and uh pediatric history taking and pediatric abdominal conditions that are recorded as well, that you can go back and have a look through. If you're interested in either pediatric any or pediatrics as a whole, useful yeah, If we can fill out that feedback form, I can really appreciate it and I'm assuming that's normal questions that's fine yeah. I hope you don't okay great so these slides um can be sent out as well. I think um I'll speak to the doctors that kind of need this and I'll send them slide so that they can send out anyone who needs it brilliant ok well. Thank you for attending.