So I'm going to try and take you through a very different talk around the screening for Risk of Sudden Death in the young. So I'm just going to try and put sudden death into into perspective for you. So we we have around 50,000 unexpected sudden deaths in the UK, some would estimate up to 100,000. But actually the recording of some Deaths are incredibly poor it's it's really inaccurate. The majority are due to coronary artery disease particularly in the older population and in the 1 to 35 year olds we think there may be up to 30, to 1500 unexpected sudden deaths per annum in that age group. And of those causes, one is cardiomyopathy, which is an inherited heart disease. Another is where the heart may appear normal after an autopsy is undertaken, which we've termed suddenly arrhythmic death syndrome, which is probably the the larger proportion of all. But there are many other causes in there, some of whom are of which are noncardiac. And I'll go through a little bit more detail later in the talk, but just to give you an idea of the sort of epidemiology we're thinking about in terms of the prevalence of disorders, hypertrophic cardiomyopathy, the most common cardiomyopathy one in 500 hundred and 20,000 people in the UK. Um, premature correlated disease, often due to familial hypercholesterolaemia another genetic heart disease 120,000. You've got Wolf Parkinson white syndrome, which is not genetic, but it's important. There is one of the potential risks long QT syndrome and not syndrome. I don't know how that got in there. Then one in 2000, that's still 30,000 Brugada syndrome, 30,000 Marfan syndrome, where we're looking at the most common cause of syndromic, a young thoracic aortic dissection. But there are other forms of aortic dissection also cause sudden death. And then one of the other cardiomyopathy say with mckenney cardiomyopathy still clocking in at several thousand in the UK population. So these are when you add those up, that's getting close to 300, 350,000 people with genetic heart diseases that may be placing them at high risk of sudden death and we have to identify them somehow. We can really I mean, short of screening everybody in the population. And we know that the screening committee in the UK is not interested in doing that because it's expensive. We can at least look at some high risk groups to start screening. And so of course, a symptomatic individual that may present primary care, particularly syncope and suspicious syncope. And I'll mention that at the end in particular, maybe a suspicious ECG finding somebody had their booth medical done and they've come up with some flagged findings that match the implicates risk or there may be family history. So a family history of premature sudden death or even unexplained sudden death, which is called sudden arrhythmic death syndrome. Um, where we've seen no evidence, as I said, of course, for the sudden death, even at an autopsy, or there may just be a family history of inherited heart disease, you may actually stumble across that when you interrogate the person more carefully. But I actually think in our population, people are less aware of the history of inherited heart diseases than the history of unexpected sudden deaths. And then there also athletes, which we'll touch upon as well, who are a bit higher risk for sudden death than the average. And those are the ones that tend to hit the headlines the most, as you would have seen with Christian Eriksen at the at the Euros a year or two back a few years back. There are some important considerations when we think about genetic heart diseases. Um, there's something called variable penetrance, meaning that the conditions are not always overtly present. So only in the proportion of individuals who carry them will they actually show signs of the disease, but they have susceptibility to it That may still be sufficient to cause sudden death in that individual or over time it'll develop. There's also other minor disease expression that may very difficult, may be very difficult to quantify, and often criteria that are generally accepted are not applicable when you're looking at a screening population. And the athletes heart in particular may have features that overlap with cardiomyopathy and sometimes even channelopathy, electrical heart diseases that make it difficult to diagnose. So I think looking at a cardiology referral to a specialist clinic rather than just a general cardiology list is important. If you want to get to the bottom of something accurately. And in fact, those are the international guidelines. Now referral to a specialist, a specialist service, the referral processes is complicated, but primary care is so critical and vital to the whole process. If there's been a sudden death, the coroner and the coroner's pathologist will be talking to primary care, and often it's primary care needs to guide the family after an unexpected premature death. Then it's primary care responsibility, often to pass the family on to cardiologists, paediatricians to geneticists. There may be sporting bodies doing screening, there may be schools doing screening as part of the cardiac risk In young screening program, for example, some of you may have come across and there may be the medicals that are just being done through executive care at Mayo or through BUPA medicals, etc., and those may come straight back to primary care for action or may be referred straight on, But primary care is going to be integral because you're going to pick up the pieces afterwards as well. So really, really important to to have that understanding of the processes and know who you're going to involve. So let's think about a family history of unexpected death. The first step is a coroner's post-mortem. If there's been an unexpected death, that's a standard process in our country. And thankfully that's in place because there to exclude unnatural deaths, deaths due to foul play. But the coroner doesn't need to necessarily tell you exactly what the cause is, as long as they've excluded the unnatural causes. But there's a tendency now and there's a progression education amongst coroners that the actual cause of death may still be relevant to the family. And we need to drill down further and further into that and involve doing proper coronial autopsies with reports doing histopathology, where the expert autopsy may appear to play a particular role. At my institution at St George's, we have the foremost quality pathologists in the country and one of the foremost in the world and it provide and it provides a critical role in be able to determine the cause of death accurately. We also need to know more about the victim's history, which includes liaising but liaison between the coroner and eventually the cardiologist to see the family on their reported history and their hospital records. But often these unheralded deaths, they're less likely to be a family history and less likely to be a heralding of symptoms or history in these genetic heart diseases. We need to try and identify them earlier, if possible. And then there's another potential role called the molecular autopsy, actually pioneered by one of my colleagues at the Mayo Clinic, Mike Ackerman, which is post-mortem genetic testing, where you will be able to identify the cause of death by doing genetic testing in material obtained at the autopsy. And we're slowly getting through to to coroners and pathologists about the importance of that at the time of autopsy, such that there's a there's a new NHS pathway that works closely with pathologists and coroners. And so now when we do look at autopsy series and this is actually one that comes from Ireland, but it's very similar across the world, similar results in Australia and New Zealand, Denmark, a lesson of the UK because our data reporting recorded that if you have a look at potential inherited and genetic causes in the 1 to 35 age group, you'll see at the bottom there, there's HCM, DCM, ARVC, LDH They're all meaning heart muscle, disease, types of heart as the heart muscle disease, most of which will be genetically mediated. There's also premature coronary artery disease you'll see as the second tallest peak. And this aortic dissection, aortic rupture. And as I said, you will see a genetically mediated. But the biggest peak is, is this group sad? Sudden Arrhythmic Death syndrome, which is the one that's most likely to be associated with electrical diseases. So if we detect an inheritable heart disease that's known to be in the family, then we should screen or we know that's in the family to screen as per that condition. So if we're going to identify cardiomyopathy, for example, so hypertrophic cardiomyopathy, we need to screen family members according to that. So an ECG and ECHO is a basic screening tool. Same with that as a cardiomyopathy and often cardiac and genetic testing may play a role in refining the diagnosis. And then we can go on to stratify the risk for the individual. But this is a standard screening process. So here we have an ECG of an individual with deep t wave inversion laterally on BCG and also in theory and then voltage criteria for left ventricular hypertrophy on these immediately highlighting electrical left ventricular hypertrophy. And then we can do a transthoracic echocardiogram. We can immediately point out evidence of septal hypertrophy support of a classical presentation of hypertrophic cardiomyopathy. Now there's another condition which is close to my heart, forgive the pun, but arrhythmogenic cardiomyopathy or right ventricular cardiomyopathy that's actually a lot harder to screen for. You can see there's quite a panoply of tests required to screen families for this disease and often cardiac MRI and genetic testing. underplay a really important role. And I'm going to be mentioning genetic testing a lot of the time. It's if you are genetic heart disease, we actually understand the genomics better and better. And so this is an example of an individual here with you'll see ventricular topic's actually coming from the right ventricle. They've got t wave inversion anteriorly and this is a rhythmogenic Ventricular myopathy And they've actually presented with a VTE from the left. They have left ventricular involvement as well, which is classical nowadays for arrythmogenic cardiomyopathy. And then we'll go on and do a cardiac MRI. I don't think that's projecting particularly well. But on the on the left side you'll see an enlargement of the right ventricle there with a distortion in the outline of the right ventricle indicating aneurysms in the wall and then scarring over here on in the in the left ventricle. And this will be picked up predominately by MRI rather than by echo. What about electrical heart disease? And that's ion channelopathy. That is another term for that long QT syndrome is the one you've probably most heard of. The most famous of the electrical heart diseases since was the first to be described and a range of tests is ECG echo just to make sure that you're not missing a phenocopy. Something that may be causing prolongation, but is actually due to structural heart disease, exercise, testing and ambulatory monitoring and really help with diagnosis and risk stratification. Genetic testing is so useful here as well in terms of making diagnosis and supporting investigation. And so here we've got an example of a patient with big peaked T waves, broad based t waves QT prolongation corrected for heart rate giving of a massive QTC. But many of these patients have much more subtle subtle changes in the QT interval that are less straightforward to diagnose, particularly when you start to look at families rather than the incidence cases that have caused cardiac arrest or sudden death. And just a reminder of some of the normal ranges over there. Now, this unfortunately hasn't projected well, but you can see it's poorly controlled epilepsy. So this is little vignette had a young woman in her early twenties had a long history of poorly controlled epilepsy and she'd never really had an ECG done. She'd seen lots of neurologists and she wasn't responding to multiple types of antiepilectic medication. You'll see that there are some changes here on the ECG, some T wave changes anteriorly, which are not ones that we would generally try to dismiss out of hand. And laterally, particularly the QT intervals starting to look quite long. You can't really see the end of the t wave defined very well. Um, implanted a loop recorder in her just to make sure because I was making the diagnosis and about a week later we got that anyway as a cause of her epilepsy. And in fact she responded incredibly well to beta blockers and and is done fantastically without the need for an ICD. And in fact, many of these patients can be managed very well with medical therapy rather than resorting to highly invasive treatments. Now, one of the other conditions that I look after is Brugada syndrome. As Steve mentioned, Brugada syndrome is another condition that predominately affects young male adults. And in fact, as you'll see in a moment, is probably probably the major cause for young, SADS deaths. These are these sudden deaths without any evidence of heart disease. And we diagnose that with a range of tests. And the Brugada syndrome is manifest in the right ventricular outflow tract, which is not always covered properly by the ECG. So we have to do particularly extensive and specialist EKGs to make diagnoses and to do this stratification. And sometimes you even have to do provocation testing with sodium channel blockers. And this is a particular one called ajmaline. You may never have heard of that. It's it's one that we use as a short acting intravenous drug. And again, genetic testing can play a role. But this is an ECG that if you saw an A&E, you'd think this person's having an acute MI. And you're right to want to exclude that first. But this is actually a cardiac arrest survivor who was 18 years old and had this pattern in v1v2 of this j point, and ST elevation with a curved pattern that's typical for brugada symptom called the type one pattern. And this is his brother who we saw a few weeks later where he had this ajmaline provocation test. And after 3 minutes of having an intravenous sitting channel blocker, he gets exactly the same pattern. So he's a carrier for the same condition and at risk of sudden death, although thankfully not quite as severe as his brother's. There's one other condition that you probably need to know about, but if you do come across it, it's going to be unusual because it's present in about one in 10,000 people, and usually in children. In fact, it's probably the most common cause for sudden cardiac death in children. Usually the child who's running in the playground and collapses and dies suddenly, particularly tragic conditions catecholamines polymorphic ventricular tachycardia, and we diagnose it with exercise testing, a Holter monitoring. But obviously most children, it's quite a challenge to do that. But there will be adults as well who are affected by it and carriers and they can pass it on to other family members and genetic testing is very good again for making the diagnosis and often the ECGs completely normal. You would never suspect that maybe bradycardia for age. And then when they exercise, they get a complex ventricular to be bidirectional, like to be polymorphic. Ventricular tachycardia is what ensues and then ventricular fibrillation. And this one thankfully terminated by a shock. And so this is one where we always say to any GP or neurologist who listen or can hear it is when you if you have a seizure running, that's not epilepsy, that's cardiac until proven otherwise. So any seizure during exertion is not a seizure, it's not neurological, is cardiac. And particularly in children. Just be aware of that because in our series of cases of sudden deaths, that was a one heralding factor that could have been preventable. So SADs where we have a sudden death with a normal coroner's autopsy, normal toxicology. So therefore, normal heart and is is unexplained. We suspect that we're going to find these electrical heart conditions and indeed, sometimes genetic testing will make that diagnosis for you. And we do a wide range of tests in family members, including MRI and ajmaline testing where needed and genetic testing. If we find something suspicious in the family. And if we do that process, if we go through that process, this is some data from back in 2018. This is the largest series reported so far, and it's from our group. We actually find that around just over 40%. So in this series, 42% of families, we have evidence of genetic heart disease, in particular the Brugada syndrome being the most common diagnosis. And most of these cases are of sudden deaths, young males dying in their sleep, which is the characteristic of Brugada syndrome. But we've got lots of these other conditions, including even Cardiomyopathies being detected in families, even when the autopsy has proven normal in the best of hands. But there's still this group of families where we don't find a cause and with genetic testing doesn't seem to get us anywhere. And this is where our research is directed to try and understand with that what is causing the sudden deaths in these individuals, because in the end, we want to prevent them. What about an athlete's? Well, they also have a a a risk of sudden death. And often those risk that risk of sudden death isn't necessarily related to their exercise. It's actually it often occurs when they're not exercising. So just to be aware that while there's a higher rate of exertional sudden death in athletes, actually their risk isn't necessarily always related to exercise, but we still see the normal heart, the electrical heart conditions being important and relative irrelevant in these conditions. But cardiomyopathy tends to take up a bigger slice of the pie than in other and then in non-athletes. And what is the risk of being an athlete? Well, it's about a relative risk of 2.5 higher risk of cardiac arrest than in more sedentary individuals. And this is data from the Italians. They put in a a programme of pre participation screening for individuals 14 years and older. It's controversial. There are many antagonists and many protagonists. It's not funded in the UK, but as you say, it's funded initially and it's really galvanised when we have these high profile sudden deaths that I've mentioned to you already, the screening process and that's been adopted in Italy and is adopted by some of the screening organisations, the charities in the UK is to do the family history physical examination and a resting ECG as a screening tool in the US. Traditionally it's stopped the physical examination and family history without going onto the ECG, but there are changes afoot in the US as well. But if you have positive findings then you go on to screen. You may be missing still cases. There, but if there are positive findings, there will be potential findings of cardiovascular disease that could be managed. And according to the diagnosis and some will be able to return to competition, but some won't. There are still going to be individuals who are having further examinations who have basically false positives in the screening process, and that's something that people have to be aware of when they go through any screening process. And they're asymptomatic individuals who would otherwise be not aware that they have a risk. And this is endorsed by the ESC, the International Olympic Committee, C four and with the other sporting organisations, including the RFU, etc., and slowly being taken on by others, but not by the screening committee in the UK. And this is historic of this is data from Italy showing the impact on athletes and non athletes for the screening over time. So it's a historical control. So not a case control study, but still giving some evidence for the reduction in risk over time. And that's probably the best study we have at the moment. Finally, suspicious syncope. So obviously we need to interpret syncope as to whether it's phase vagal or whether it's cardiac syncope with a more abrupt onset and offset. And sometimes that's going to be very difficult to understand. But often we may see evidence of hypoxic convulsions and seizures being the the pathway for diagnosis. Again, neurologists and cardiologists need to work together from that point of view, we can do things like tilt testing. We look at blood pressure response and heart rate response to tilting and to GTN provocation. This is a normal response and here you'll see the blood pressure and heart rate drop in response to just tilting without even the need to GTN and all this an old fashioned tool and it has its faults. I still use it for for examining the the symptomatic correlations in patients who symptoms who are reporting unexplained syncope. But of course just must remember that we don't forget to do an ECG even if you're suspicious, if you're vaguely suspicious that it's face a vasal vagal rather than others. Complete heart. BLOCK of Parkinson white syndrome. As excited as can cause and can cause cardiac arrest and syncope as well. So always be aware that there are acquired causes that may be doing so, not just a genetic. Thank you very much.