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Echocardiography for Diagnosis, Prognosis, and Management in Hypertrophic Cardiomyopathy

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Summary

This medical on-demand teaching session will go through the importance and use of echocardiography when diagnosing, prognosing, and managing patients with hypertrophic cardiomyopathy (HCM). The presenter, a senior cardiology fellow, will also give tips on how to perform and report echocardiography readings according to the British Society of Echocardiography Guidelines. In addition, the presenter will discuss potential phenotypic imitations of HCM and red flags to look for in elderly patients. With this educational session, attendees will be able to gain useful information for delivering world-class care to their HCM patients.

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Learning objectives

  1. Explain the different etiologies of hypertrophic cardiomyopathy and the role of echocardiography in the diagnosis, prognosis, and management of the disease.
  2. Identify common red flags during echocardiography examinations associated with non-sarcomere-related hypertrophic cardiomyopathies.
  3. Demonstrate knowledge of European Society of Cardiology and British Society of Echocardiography guidelines for diagnosis and treatment of hypertrophic cardiomyopathy.
  4. Interpret echocardiogram results to diagnose hypertrophic cardiomyopathy, phenocopies, and age-related pathologies.
  5. Compare and contrast the advantages of echocardiography over other imaging modalities for the assessment of hypertrophic cardiomyopathy.
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The following transcript was generated automatically from the content and has not been checked or corrected manually.

hi and welcome. Everyone I heard is you've been wearing passionately for us to start. I do apologize sincerely. Been a slight air on my behalf. Very embarrassing, but it is what it is, and it's tech issues on Will Persevere foods. So do apologize on so higher month Miracle on before introducing are really wonderful speaker tonight. I first want to take the opportunity to really run through what the goal of this current journal club is because you're new to you might not be familiar. So the goal of his journal club. It's to share our expert knowledge across, then adjust the expert knowledge that we Concerta loss of energy to We can all work together it up it better. And our ultimate goal of delivering that world class character passions unfairly free is the really driving appreciation on interest in science and research. By doing that, will be able to generate future research collaborations across that the trust and then a Jess, we'll be able to drive in approved standards for free our research on ultimate fruits. You're a little bit new to Microsoft Life events me a little bit extensive Father's tech issues. How can you bend it, enjoy it on. There is some controls at the bottom of your screen and that allow you to pause there. Then, if you need to take quick bathroom back, you can even a just avoid him that even the video quality if you're joining from a low band with on Dwell, should not be able to join in from a video or auto perspectives. There is a lot of tree and a button which have displayed in you're still on your screen. If you click that, you can ask any questions at any point during the talk, but we'll get it was right there and with GM on you can after it's anonymous anonymously. Sorry or you can even enter in your names. We know who's asking you questions. If you would like to share it up on, I guess the next point of today is you didn't really come to join A Listen to me. Talk about my attack issue. Should came here, did it? Joining and listen to games on. Jim's is a deadly principal. Current physiologists lead at the bar talk sooner. He is education and research lead for correct physiology. On top of that he is the NIH. Our clinical doctor Research. He is an NIH, our clinical doctor research fellow. So I think it's great opportunity to welcome our speaker for tonight on. I'm just showing you that she, like, 19. Thanks. Governed community. Yeah. Care they with that? Yeah. Thank you for organizing tonight. Gavin on drank you for the 15 minute warmup. It was great. So I hope I've still got some some words after me, but I'm right. So thank you for organizing and think for the introduction. My name is James a senior cardio, because the oldest in parts heart center is government says on. But I've got I'm doing a fellowship, part time PhD and looking at investigating interventions in hypertrophic cardiomyopathy. And I'm an echocardio by trade. So this is an area that I'm very passionate about. So that's why I want to talk to you tonight. About thie importance of echocardiography. Been diagnosis prognosis. A management of patients with this condition out further do Hopefully I'll be able to. Good. So, as I said, we're gonna talk about the, uh, the importance of echocardiography in these three areas of hates again. I'll also talk about some Henson tips of how to perform and report to hate cmax with reference to the B S. C, the new newly released British Society of Echocardiography Guidelines. And if this time I've got my could tell, it's a bit about, well, it shows, um, nice phoenetical Ari a Shin or examples of Phoenix Typical reaction towards the end of the talk success in diagnosis so that this is the It's just a screen shop from the 2014 Europe in Society of Cardiology guidelines just to nicely demonstrate the different sort of etiology is behind hypertrophic cardiomyopathy and around about half to two thirds of patients with hep C Um uh that genetic causes from, uh, uh, genetics sort of mutations in the Sock America proteins the big hitters on on the left hand side of the screen. Them and around 5 to 10% of the patients hate cm, maybe caused by other causes that other outside of the sarcomere. So so either syndromic oh infiltrated disease on around a third of patients. The cause is is yet to be identified. So, you know, negative genetic testing and no obvious red flags indicating former thrown a touch in a couple of show you a couple of examples of what we call Phenocopy. Is there something that looks, like hates Tiemann on Maybe with, you know, without further investigation, maybe erroneously defined a sock America extra. So hopefully you can see the three moving images that I can see What your parasternal long access to apical four chamber and a Palestine of short axis on, uh, several red flags that alerts us that to something else going on here So you can see in the personal access the apparent apical sickening It looks, you know, this could be a pickle. HCM is also a slight thinning and and brightness of the basal posterior wall, which is worth mentioning in the four chamber. The heart looks thick. There appears to be some speckling of the myocardium. There's, um, a true ventricular valve thickening certainly apparent on the mitral. That's, um, black by atrial enlargement as well. And what looks like reduce long axis function on in the short axis. There's this what appears to be a relatively discrete ball of back oh, brightness. Or it could be fibrosis and then have sort of mid uh uh, permit posterial warm. And this is. These are all red flags for Anderson family disease, and this is, ah, metabolic disorder, characterized by the by the patients not having a particular enzyme, which is, uh, for collector cities? A. I mean this causes. The absence of this enzyme causes accumulation of glucose finger leopards within tissues on replacement fibrosis and these that these are the hallmarks of F D disease on some properties. Another phenocopy, another thick heart copy of page cm. So this is again a typical four chamber on the left hand side, and that's, um, strain imaging or right on the left. Inside, you can see the Apical 14 with the speculum appearance of the myocardium. Again, there's a again atrioventricular about thickening some slightly more significant buy extra enlargement here, probably in some thickening. Also of the interaction will septum global longer to, you know, strain imaging. It's useful in this instance on. You can see that there's reduce strain at the base and mid level with relative sparing of the apex on, but these are all hallmarks, or red flags of amyloidosis. The amylase is an infiltrative disease where abnormal accumulation of amyloid protein causes the sickening of the secondary thickening of the myocardium. So again, red flags that are important to identify on elderly patients are worth worth mentioning. Here is well, so patients as they age and his diet dilatation of the aortic rusian ascending aorta can cause a a increase in the in the septum aorta and you angle, causing the basal second to bulge out in encroach onto the definitely outflow tract. So the on echocardiographically this get may give the impression of they isolated nasal septal thickening, uh, normal thickening, um, at the sort of elbow of the of the president myocardium pushing out on. Also, these patients may get secondary outflow tract obstruction due to this abnormal sort of bulging into encroachment on to the old beauty. But again, these patients do know have, uh, sarcomere in case CM. But it's red flags. It worth worth considering into this talk really is to is to is to focus on psych America CM, which is, uh, that that makes common have Editori cardiac disease. With the prevalence of around about one in 500 the general Population Imaging study, more recent imaging studies have shown that there may be higher degrees of prevalence in the population of higher degrees of unexplained hypertrophy that in the general populations we may be underestimating the problems of the disease. It's characterized by abnormal thickening community. That means you know a full thickness of above 15 millimeters in the absence of another cause, an isolated cause that could solely explain. It's such a such a aortic stenosis or hypertension. Proportion of patients will be at risk about verse events, while many will live a normal, normal life. Have normal life expectancy of proportion will be a risk of arrhythmic or embolic events. Onda Again a proportion of patients will have significant have symptoms. Exertionally symptoms ranging from, you know, mild dyspnea through to advance heart failure. So it's a disease characterized by thick and stiff muscle things. Muscle is impaired, unsurprisingly impaired, with impaired relaxation and diastolic dysfunction on echo that maybe obstruction to blood flow within the market. Within. Sorry within the left ventricle. Um, nothing current various levels, and it's a disease characterized by by hypercontractility, which contributes to the after mention on this has been been investigated and sort of under better understood recently is hate CM patients having hyperactive a shinin active mice and cross bridges compared to that of a normal heart. So it's a disease characterized by, but my card will thickening on. In order to diagnose it, you need to see it in the apartment. The point of putting this slide up his office. It's It's the magnetic resonance imaging slide. We may not see these 20 clearly on Echo, but this is just a demonstrator that there may be, uh, metal thing is, the hypertrophy may be hiding in in areas that unexpected areas in a lot parts of the myocardium. So it's a very important for assessment of the whole ventricle from base to Apex. And this will be a This is the sort of first view that we often based with and echoes in the parasternal no access view on that. Someone has made some, uh, some measurements of the diastolic and I started parameters. So that basals except, um, the intra intracavitary dimension and the posterior wall on so that we've tried to measure the Internet trick. Except, um, I think that's probably the RV moderate, a band coming in and it's a suspicion of a power septal structure there as well, which we wouldn't include either of those two things in the match Wal fingers. And this is another case study of someone with It's not. It's not the best. First, in a long access to get off access, there's there's a suspicion of some septal thickening upon measurement. The sickening. There was any measure, at 1.4 so 14 millimeters. Again, it's a suspicion of a power septal structure there on, but we really do need some other views to confirm now. The Apical four chamber is a good cardiac function. The atria don't look too big. There's suspect suspicion of mid myocardial walls thickening on the septum. The short axis is not particularly useful in this case. Importantly, shadowing. It's very unclear and midget, and we really we rely on it so heavily in the assessment max of Oral Fitness, and when it's not useful, it could be quite difficult challenging to define these again. A patient or satis at C m. Other corresponding. See MRI images where you are very good on the cardio definition and can accurately measure maximal thickness on. There's, um, Basil under a septal, working around to the mid septum at the mid to about the mid level um so the inferoseptum in the mid level thickening. And then this was described some your Max wall thing. It's a 20 minute meters. Rather report to you and I the next these two, these lines I put on to sort of demonstrate. Oh Oh, yeah. Just to sort of illustrate that subtle, angular angle ambulation differences of the probe in echocardiography. Imagine that was a slightly off access to be chamber of four chamber to 10. But you may miss that the areas of maximum i podiatrist again underscoring that the importance of the short axis at the apex have been tricking rape X isn't often under appreciated area of the myocardium that that may hide hide on harbor LB. Hypertropy. But again, you've got 30 partner Long access. You're on the top left and then effectively slices of pineapple going down, going down all the way to the I'll be apex. So starting in the low mid cavity with evidence of any muscle presidents and then moving all the way down to the I'll be eight packs and want to recommend doing is, is to include a long a long pan of a clip like much like you would a assessment of aortic valve stenosis or a stenotic mitral about you might panther from from supervisor to improvise on. Do this at the apex in order to go from the point where the blood pool finishes So they're very, very tip of the mark of the cavity. I think panthers and come up just to make sure that I've made in a accurate assessment of the NBA packs in any areas that might be subtle differences in maximal fitness and the else or the B S. C guidelines are released very recently. Excellent, and they talk a lot about the pitfalls of localizing matter wall thickness of the different levels on. And unfortunately, in practice, of course, the Endocardial border is rarely a smooth, well defined. I want to find surface, so the's another highly recommend you read. These read these guidelines on they give hints and tips is to how to how to exclude septal structure of structures outside of the interventional, a septum that should not be included in that small fingers. Well, soon looking for you don't want to use contrast assessing. I'll be in effects appropriately, and if you have the benefit of having any CG before the the Echo cardio echocardiogram. And you can look for the telltale signs off the deep T wave inversion in the precordial leads with, perhaps, um, ST Elevation. And that might really push you towards looking. Assessing for LD aneurysm next 10. It is little importance of localizing Grady in within the within the cavity, so this could be a a mid cavity, the outflow tract. They may be simultaneously at both levels. Eliminate. Just be some flack separation in the context of a hyper dynamic cavity on this is important to define because it really does change the management strategy with these patients. So I recommended order of assessment, being starting with looking very thoroughly for the substrate just visually looking for the substrate cavity, obliteration or systolic anterior. Mention the much valve using color Doppler to assess any flow of normality. Looking for the absence of lamina flows were looking for looking for, um, turbulence in throughout the cavity, including the alveolopalatal on. Then are there any changes so you can use over the counter top plan and spectral Doppler it'd to look for for flare up? Neutrality is and then looking. Do they change their if they're normal at rest or just mildly abnormal. Do they change any dynamic changes of provocation? Such a such a? That's album. And so that's another case study. So you put the continuous wave through the cavity from there from the LVH packs, and you've noticed that the high velocity a jet it seems to be just over 4 m per second. Maybe up to five. Enough speak, Andrea. The next stage is to is to say right, we've we've recognized the high velocity jet. And where does he? Where do we think this comes from? So then we start our detective work. We start the I'll be a packs with a pulse wave walk through, so we take a clip of the pulse rate with the apex. There's a little bit of plaque celebration. This is an obliterative cavity. That's nothing. Doing their come up to the mid cavity is a similar story where you've got this sort of triangular shape, relatively low velocity job in context of hypodermic function. You come up to the outflow tracks, and something's definitely changed has been a step up in velocity. You got aliases, the pulse width Doppler signal and wrap around, so walk back a couple of clicks to just into the mid cavity. And what we've got now is a classic lobster claw trace and shows that it is absolutely classic for outflow tract obstruction, where you've got the mid systolic drop in velocity justice to the point of septal mitral contacts. And I mean systolic. Drop in velocity Justice does that to the point of settle mitral. Several contact is classic for a protracted, but we also need to rule out any contamination with mitral regurgitation because in in A in a cruel twist for the echocardiography, as you got worsening outflow tract obstruction that is often secondary, worsening mitral regurgitation that is due to the outlook the systolic absolutely mention the mitral about. So this is through the continuous way same patient, continuous way through that through the cavity and this time angulated of the mitral valve and as a sort of relatively pop indicator to baseline jet. Very high velocity measured as we angle the probe alignment through somewhere in between the two, you get a sort of hybrid of early peak, and there's something else going on. There isn't and then has become through to the outflow tract. You can see very clearly that absolutely classic sign for Scimitar shape jet trace, I should say, for after tract obstruction way, it starts relatively perpendicular up to 2 m per second and then on then conclave to the right trace. I'm so I'm very confident that this is true appetite obstruction not contaminated with mitral vegetation on. It's a decent going, Grady in of over 100 mg stomach you on the notes of mitral contamination. It's incredibly. This is a tool you can trick you can use where you you look at the terminal portion of the of the trace and just say great quickly. Does it look perpendicular to the baseline? If it is, it's probably mitral regurgitation in a medical measuring a different patient. Now you can see how the continuous wave is. Is isa a line through the mitral valve is not really near the outflow tract, Um, but it's a slightly funny shaped face. You've got the hybrid, and now you've got a bit of perpendicular in a bit of a bit of angulated, but angulations towards the outflow tract. True outflow tracts. There's a change in shape off the trace on Do you conceive how the terminal portion of the jet deviates from the perpendicular and get I'm very confident that this is a true Grady int of hung in 40 minutes of mercury. So sticking my 66 m per second and this patient so you can your free for prognosis. This is the, um you may be familiar with this is is that and the hep C um, risk SED calculator. So this is a a tool. A calculation tour developed by cost and cost is O'Mahony on colleagues on it's been adopted by the European Society of Cardiology in their guidelines on echo features very prominently in this tool, it's it's a capital to calculate patients predicted risk of sudden cardiac death over five years, not one. You're over five the next five years. And like I say, echo features very prominently with three out of seven variables, um, being continuous, echocardiography variable to get maximal Think so millimeters left atrial diameter and millimeters on maximal lvot. Radiant. Excuse me at rest or with ourselves and I've got a couple of examples just to show you how the measurements we make an have a real implication for for a patient. So let's look at this one. We got 40 year old patient that's more thickness of 16 millimeters left atrial diameter of 45 millimeters on maximum recorded out food Factory in of 60 millimeters of mercury. Yeah, no two family history of sudden cardiac death. Yes, they had some anesthesiologist and know toe unexplained syncope. And they have ah predicted risk of STD it five years that falls within the intermediate range between four and five. Sorry, four and six is is intermediate. But let's say we get that patient to do a proper about salvage this time. Hum, we demonstrated out for track grading of 120 minutes of mercury, so we doubled the the big radiant with with appropriately done about Saba Andi. Um, this is pushed the SED risk up. Predicted acidy risk up with a high risk level now. So it where where, according to the recommendations and I CD should be considered. So obviously that's going to inform the discussion with it and a shared decision making process around I CD and implant, which is no without risk itself. Another example again, A 40 year old patient with the maximal fitness of 18 millimeters of mercury 18 millimeters left atrial diameter of 45 millimeters outflow tract, you know, 60 and the same as before. For the binary eso, they have a high risk. According to these these parameters, this is patient would be considered in the high risk category above six, the center of the next five years. But unfortunately, the Echo cardio for included Thie moderator band in the maximum well, the wall thickness and it's actually a true well, the warfarin. This was 15 on the Imagine Obliquely across the left atrium and overestimated left atrial size. So actually the true measurements mean that this patient has a predictive risk of in 40 in intermediate range. So is not put up Teo strike fear into the arch back a cardiology visit Teo, just Teo underline the job you do is important and that the measurements you make have a real impact or patient care like management. Even I'm sorry I'm recovering with a sore throat, so I'm after my cortisone say, excuse me, My voice mails eso management of patients. Echo is incredibly important for monitoring results off medical therapy on more invasive interventions for symptomatic patients that may have been prescribed. This is a patient with sure. I'm sure you've seen patients with with my post my activity and our course that the ablation this is slightly more niche. This is a patient with mean cavity. Obstructive hates cm so you can see the hypodermic function obliterative cavity on the left hand side, corresponding flax elevation in the mid cavity In a good going, Grady in, they have a job set Julia Chamber I CD. So we use that a prickly placed are right ventricular lead. Um, we can use it to pace the myocardium and introduce localized dyssynchrony in an effort to reduce. Get that at the my cardio T interrupt that hypercontractility, Let's say, Get the segments of the market, um, to contract ever such different times, hopefully reducing the the cavity obstructions. And this is the patient in science rhythm. Going great in a rest of 80 minutes to Mercury on with by enforcing R V pacing using a sense debuted a lot of 80 milliseconds. We've managed to chop that grating in half. Now the other incredibly important role or thing you must do and performing a pacemaker optimization for for obstruction is to look at that. I'll be feeling so. We measured the dust like feeling time in the A wave, a way of duration and take note of its morphology. So this is the you can see the cure us and like that, the way it's upright. So this is during during Sinus rhythm, and I hope you agree with me. There's very little difference when paste that since they were below 80 milliseconds in either that or follow gee or the duration of either of those those those intervals, because it would be by being too aggressive with pacing in order to with too aggressive with the atrioventricular delays. In order to get the maximal dyssynchrony, as it were in the in the ventricle, you could end up robbing Peter to pay Paul and leave your patient feeling symptoms as symptomatic, if not worse by by truncating that left ventricular feeling. And this is just a an example of how you're cute. Paste results continue to be to continue to see a decline in a, um, a cavity obstruction a zoo not only acutely, but then a one month in the patient. So not all that house and hit hints and tips of hard to perform a a cm echo. So it's all about knowing where to look on what four and use adapted adapted views wherever necessary, and the DNC guidelines underlying ms the importance of this sides interpretation of the doctor traces is absolutely key on provocation. For, you know, performing a good quality about salve and perhaps other methods with a sitter stand or exercises was also very useful when when resting values are not are not conclusive. So this slide really is just to say that unfortunately, despite the guidelines in there and your training, sometimes you just have very poor image quality. So sometimes of you may be completely unuseful, for example, and it's patient with with hay Seattle suspected hate salmon The short axis is really, really not useful here, but I'm confident from the apical three tender on the right hand side. In this patient, there is septal basal. Two minutes settle thickening. There is systolic anterior motion of the mitral valve. There's a septal contact lesion on. I'm sure the Doppler and Spectral traces will back up that diagnosis, so you know it's about the Nickel three team is such an important view for for after attract obstruction call. Often, you know, that's it's absolutely I can underline enough out. How much McKeever you that is for for resting and provocation images. You also got to make sure that you're color Doppler is in the right place for the for the phenotype that you're assessing. So on the left hand side, this is the patient. You'll have to believe me with cavity obstruction. So there is evidence in the outflow tract obstruction, but the initiation of that surveillance is up in the in the middle of the middle of middle off the cavity. So whilst the echocardia cardio for is already looked for Alfred Tract, obstruction of slightly missed the point at which it starts, which is depo within the cavity. Um, and this is it on the right hand side, a different patient with very distal obstruction. So apparent a comedian apical thickening on the color box really should extend through out a way to the apex. You see, this is April 4 chamber in the five range, but it may be that the alignment with the obstruction, if it is very distance, is actually seen better in the four chamber than five. But it's the importance of of having the color box extended to cover the entire apex is again absolutely central. Stop the profiles. You important, uh, parameters. You looking at it shape. It's timing and it's velocity. And this is figuring out can remember the reference so But it is the underlying what I was saying before about the difference between mitral regurgitation and out for tract obstruction jets. I don't find really everything slow sweeps be like this. Particularly useful, I'd recommend. I understand that you know, some people like it is to demonstrate the change of their sweeping between mitral regurgitation, toe outflow tract than that. Yeah, potentially. That's awesome. Awesome utility there. However, if if you really want to focus on the shape, the timing in the velocity of a Doppler trace and having a sweep speed of 12.5 millimeters per second is is not entirely useful. Use. Use 100 millimeters a second if you can, and also you talk to talk Teo to the patient when you're when you're scanning them, listen to when they when they experience their symptoms. This is a gentleman who um, described very, very clear post prandial symptoms where, you know, after after a large meal would have stand up Teo to the bathroom or whatever, and and that is when you would experience his his symptoms of dizziness. So this is Ah, did Valsalva raft about salve on a patient with with abnormal sub mitral apparatus? And I was determined to 10 straight outflow tract obstruction, but for love, nor money could only get 3 m per second. But then, after talking to him and understanding that he gets, it's when he stands up, I got him to do a sit to stand and completely unmasked his his outflow tract obstruction. So do you talk to the patients if it if they get it, it's only post brand new on. But they stand up booking afternoon echo, getting to have a large lunch and then getting to come in and on do sit to stand for you. I said of B S C. When it comes to reporting a C. Um, it has to be a C. Give some gifts and I knew you guidance gets, um, tips for for language recommendations. So where there's ambiguity in in a in a scan, they recommend using the text. It raises the possibility of hate cm rather than trying to, you know, definitely rule in rule out. And if the if the results are are very much consistent. And that's been without the red flags that for mentioned that the phenocopy he's been in the news language, that is, that is positive and for relatively for minutes in his diagnosis and say this is consistent with his skin. So the conclusion points to be S C really do do Trulicity that's that. That that's the conclusion point should be should be single line and then could include some key bit is not just about copying and pasting the LV section of the of your brain. Report. It's about putting you know four or five keep oint in bullet points in there. You should include theater action fraction beard, visually biplane assessed the maximum or things, of course, the left atrial diameter, the left ventricular outflow tract. Greedy and even if it's five, put it in the conclusion, because then whoever's doing the calculation confirm very quickly. Safe right matter. Walk Fitness 18 Left was on it for 45 up a track rate of five. That's just helps when reporting and taking it next steps with the management. You don't look any evidence of pulmonary hypertension on any evidence of a left ventricular apical aneurysm. So emerging risk factors for some kind of death that was a really ductions in ejection fraction below 50% on the left ventricular apical aneurysm is absolutely key elements during through in your in your report. If if I'm allowed, I'll quickly talk about some phenotype. It's a big variation that I'm particularly interested in my wrist. My research focuses on so on on the left and panel, you've got this sort of like matrix of a patient with multimodality imaging of mid cavity obstructive. Hey, see, um, you could see the LV aneurysm had glamorous, um, on the left and left upper panel on on on the owner CT on you can see the Associated Doctor flat abnormalities and the bottom left hand side on the right hand side, you can see they're similar sort of angiographic and CT, uh, matrix on down on the echo. You can see this sort of eighths of spades. Obliterative left ventricular a packs on. This is a new patient without obstruction. So able thinking I'm good without obstruction and the corresponding Doppler Stop it! And this is that patient again. So the patient with with apical eight cm So you've got the classic on a space for you. In April 4 chamber would see that on geographic imaging if they had an L B ground, a little thickening of short axis. And then in the on a Doppler tracing, you see a sort of early run into the early Smiths or Midsystolic Ah, bit of flow acceleration on special Doppler and then nothing, nothing After that, there's Doppler dropout in the context of one obliterated cavity. There is no more flow of the apex, and that's what you see in the space of a difficult age. This's Ah, a diagram from a recent review in a place. Yeah, well, they include patients with them without Levitra or aneurysms. This reminds me slightly of sort of smarts or something, or some cartoon. My my daughter would watch, but they highlight here that the patient can have a great season with the with the without an aneurysm, a mixed mixed my cardio phenotype where you've got. But I increased it up to the mid cavity and then also patients were predominantly med cavity. So they include these patients in this overarching bracket of a peak waves. Yeah, and these air some corresponding echo images from someone with a cavity obstruction or a big wave salmon and made cavity obstruction that say so again, the parasternal access Not much doing the apical four chamber and to chamber and on five chamber. Those, um, some abnormalities become apparent. You got what looks like distal intricate, a thickening and the color Doppler. In the bottom left hand screen, you can see blue, blue, blue, blue, blue, blue with each with each heart attack cycle. There's two pulses with blue and blew away towards right. So you got blue, a movement of blood in early systole, away from away from the probe. So from Apex to base. And then you got a second power dot school movement of blood later on a cardiac cycle. And this is what it looks like on continuous weight Doppler so you can see that there's a very early systolic. Let's call it a systolic peak. Increased velocity not particularly fast, almost 3 m per second. And then there's a zoning Doppler Doppler dropout. So a black drop out after that. Until then, you have a second peak. This is a paradoxical diastolic chat. You can see that the mitral inflow eyes detected on the on the top side of the line so that where the the wave on exactly the same time you've got blood moving, apparently within the cavity away from the L B A packs towards the mitral valve. So this is obviously abnormal. So something something's going on here. I want to draw your attention and highlight how this is different to the trace I showed earlier. And it's to do with the timing on day shape of the trace. So this is the lobster claw that's absolutely classic for our contract obstruction. Like I said, the point of just distal to the point of sexual sexual contact on this is a systolic event said that the jets that trace is contained within ventricular systole That started by the Blue Arrow is the diastolic events or the diastolic timing. So you've got isovolumetric relax a shin period and those who have metric it a feeling possible. Trigger that feeling soon by the Red Arrow and you can see the difference when compared to a patient with mid cavity obstructive hate cm in this classics Jewell Peak as they were. And this is the early systolic, the systolic, then the paradoxical diastolic movement of blood from apex toe base occurs well into diastole. It's beyond the point of volumetric relaxation. The mitral valve is opened with passive feeling taking place above. But you've got a jet going moving the other way to meet it. It's in that same patient. When you do about salve a minute, it really this trace looks more to me like a car taken of a water or something where you got this long, you know, early systolic peak, and then a long diastolic tale that really is. It goes away until almost to the next cure acid after the P wave. So you've got the velocity measured in that paradoxical diastolic jet out of 5 m per second grade ian from Apex to base of 100 millimeters of mercury, and it lasted, and this is an example of a catheter trace. So this is where we got invasive hemodynamic assessment off that patient with a cavity, not the same patient, a patient with makeup and the two traders. You can see one that read is in a pigtail entire picture. Catherine the l E Apex. The greater pressure met directly measured of around about 180 millimeters of mercury. The blue corresponding traces is arterial pressure measured. A side on the of a seven French femoral sheath on that measure is measuring an aortic pressure of around about 90. So we know we've got a big a big pressure Grady in that is often underappreciated by echocardiography. We did some some work on this and published in a couple of years ago where we did simultaneous Doppler echo Doppler. Imagine mint compared with direct invasive measurements of off obstructive your patient suspected to have obstructive obstructive eight cm on What we found is that the patients with outflow tract obstruction The agreement was very good between Doppler on D invasive. Imagine Amick measurement, um but in mid cavity obstructive a cm due to the obliterative cavity and the inability to measure increasing pressure when the cavity is obliterated. So incredibly, that movement of blood if you can't measure the movement of blood you're using using Doppler and you could no longer measure measure graded. So we found that the mid cavity Grady it's would consistently dot lower with Doppler compared to the invasive measurement. So you really don't get a naprion. A shin of the environment that the L B Apex is is is under that It were using Doppler, so you can you can. You can define the timing's that that alert you to make cavity obstruction and possible LVH difficult aneurysm. But the pressures and no, not well defined using this is just, ah, schematic showing to demonstrate the points of obstruction and the classic, uh, acceleration of blood in the outflow tracks for patients with with outflow tracts, Sam related outfit, right obstruction and the corresponding pasta often posterior a secondary mitral regurgitation. And these these are arrows around it, just to show that in the early stages of sisterly, you get blood moving in the cavity Obstruction. Not that issue is not the mitral valve, but in the Macavity blood moving from from apex to base, but also a portion of blood moving moving out of the mid cavity into the apex, which is then under incredibly high pressure. Then there's then during during it asked me that when the mid cavity relaxes, you get paradoxical movement of blood from the apex into the base exactly the same time as the as the much of our weapons and on del be attempts to fill this is seen really clearly on color and mode, where you've got a systolic ejection of blood blew away on. Then you've got to competing jets. Is it worth doing doing diet Diastolic? Go to ask me what you got Red coming in through the mitral valve and blue and paradoxically objecting from the high pressure apex where it's been but for your blood has been has been trumped. This is some work that one of our master students, Tim did for his his scientist training program and it will really get taking it. Teo Seekonk conference where we looked at identification compared a multimodality imaging Hey cm. So patients with with transthoracic, echo and CM bars within six months of each other, I found the only 30% of left ventricle apical aneurysms were identified on on a transthoracic accurate compared with those with with dementia admittedly small but demonstrably aneurysm. By see a mom. However, the there were Doppler signs, subtle Doppler signs, not a Z. Obvious is the examples I've shown you so far, but much smaller lower velocities with little discrete, paradoxical jets. To these these doctors Signs of President 77 3 quarters of patients. Um, interestingly, Apical on all my cavity obliteration was clearly reported, but that this's a really a clue that should really prompt further thorough investigation. And you said contrast echo cardio cardio fear, if necessary, rattled through. And I hope I hope I haven't haven't rushed. But this in conclusion Echo is an absolutely key tool for the diagnosis Restriction of on management of patients will actually the cardiomyopathy there's really there are wide finger typic variations and then maybe overlaps between the phenotype. So don't approach eight cm echo with trying, attempting to pigeonhole. I'm thinking that you need to pick one off to phenotype so there may be, But there may be a very wide variation. Yes, it look and you will find you really need Teo. Use a Z Pak for the B s. C. Guidelines adapted. Viewed for based on the on phenotype that you're assessing on. Yeah, I can't. I I'm bias, but obviously I and recommend you closely at the apex with color Doppler and on dyspepsia Doctor as well. Just think it's, um, recommended. Reading is this is a mean Hey, Sam is a very, very well studied, um, study disease. Echocardiography is a is a is a key tool. As I say, there's lots of lots of literature out there. Very importantly, I included the SC card lines and then recently released America, uh, College of Cardiology guidelines and and, of course, the transthoracic transthoracic HCM guidelines from the British Society of Echocardiography, which I draw upon this tool. Thank you very much. So I stopped sharing. Good. Yeah. Great. Thank you very much for a talk. Jobs. Um, I've just popped into a lot of cute a section so of anyone. Doors have any questions for gym tonight? Please pop them in there on. I will send him a direct in tourist gyms. Um, and whilst we're just waiting for some of those questions, really, Teo filler fruit on. Ultimately, if you what you can do is if you're not able, Teo, you miss some of tonight's talk. You can catch up later. Later time. I'll be publishing this event on metal on That's really important for me in terms of mechanistic Cecil, because that means you can catch up on these episodes at a time that really works. See your work, your lifestyle commitments and your family commitments, which is very important. And that means you can read Watch this episode on necks, Um, perhaps notes and pause on some of those key points that James was touching upon tonight, while so just waiting for some questions. One thing that I guess I really wanted to. I ask you in a pretty I appreciate you didn't really. It wasn't a main focus of today's talk, but it's something that always, I guess, interests me from my previous research would be a lot of our definition, a lot of addiction definition. So I 400 for Carmelo with they are based on, for instance, 15 millimeters and MAWR. That's where we call that concern or if it is difficult with the 13 millimeters and more what do you Of course, we all know that we need to index and we need the index just some, like body surface area, whatever. We also appreciate that index in from a two day measurement of really emergent is quite difficult. Onders limitations With that, we know that that's, um truly remove the impact of body surface area. And that's what we look at stuff like all metric Skilling. Where do you see the future? I guess in this. Do you see it's continuing to use these crude upper limit measurements. Or do you see things changing? Changing our guests in the future? Yeah, that's a good question. Uh, Governor, I think I guess the answer to that will come with further research in ongoing studies that are investigating things like prefinished ipic um, a pre obvious hypertrophy phenotype. So in patients, you've got, um, confirmed genetic mutations. You know, maybe the first three relatives and harbor carriers of the mutation and they're being studied to see what you know, a visually echocardiographically normal wall thickness. But using more advanced techniques, such a CEO, other other multitude mentality imaging, Let's say, are there actually you know, abnormalities within the myocardium such confusion or strain imaging or something, Something like that, Or, you know, evidence of fibrosis that precedes the actual over over characterization of that on echocardiography also so also looking at patients who never I didn't I didn't drill too, too much down into it for the purpose of time. But patients with theology any packs should in a normal half, you know, taper on become off there. So although you may have an allergy apex of of less than the 15 minute me to cut off, say, you know in L. B, A packs of 14 millimeters is is abnormal. So you cast for those is relative and they'll be associated abnormalities on on oh, could be associated abnormalities on perfusion imaging and with fibrosis to get, So I think it's it's recognize it. You know, taking each patient individually may contribute to incredibly thorough assessment off the whole of the myocardium, as I said and I think in the talk on Greeley, highlighting if there's if there's lack of a pickle tapering or remote areas remote to where you initially thought, um, yeah, I'm partially honest Question. Now let's get um, and I very much so because I think it touched on a part in that aspect. Supply we ultimate don't just look at while fit Mister's other aspects that we'll be looking at as well, and you touch the bonds that'll increase that sensitivity. I'm one of those things I waas thinking about is, at which point we and you touched upon those Molly Molly, image middle. These and one of those things with weight. Think about use that in that class example in terms of getting a passion. Teo, stand up after having not lunch. At which point do you think it's appropriate to recommend stress, exercise, echocardiography, um, in such passions? Yeah, well, I guess after you fart, you completed your I I appreciate you know, all these. All these maneuvers take time and the pressures on certain clinical services a great at the moment on it, because of post. Especially great, because the postcoital recovery. Now we have a good old days. Do you go? You gotta go to call it quits some some point. Yeah. So I guess it's about giving you your best. Um, your best effort. Excuse me, sir Teo to unmask obstruction if it's there on then feeling confident when you know, understand limitations of the test. Understand? A limited that the obstruction in particular is often labile on Berries on a day to day basis. Based on you know, the patient's hydration status of stress levels was they just rushed from the rush to be on the cob or bus. So, yeah, just but feeling confident to say that, you know, today we didn't demonstrate it. However suspicious off substrate for Septra, you recommend further, further further testing with stress Echo exercise for a second cooked know scrape on. Okay, so I'll wrap things up, I guess. Thank you very much for your time tonight games, especially after giving you enough for one more protein like this on for still staying on the course. I do appreciate that. Thank you very much on think you terrible. And he's joined in tonight. Hopefully you've all enjoyed it. And if you have, um, I would really appreciate if, um, you could share. This has been with anyone else that might be of interest interest in future episodes on. Of course, if you're are interested in presenting yourself, please do get in touch a Gavin dot mclean chest dot net on. I'd be delighted to hear about your interest in what you might be interested in talking about it. Or you can find me on Twitter at Gavin Underscore McLean, but just a sweet wrapped up there. I did. There was one question that came in their teams. If I could quickly address that to you on, didn't see it on, and that's my fault. On that is really a septal. Be useful to Mid Russia, helpful for differential diagnosis at all. So I miss that settle. Do you find septal beers? Ultimate Russia Helpful for differential diagnosis that adult settle basil to mid ratio. Um, accepted several basal separate. Possibly. I'm settled, Basil. I mean, if it's if it's because the hypertrophy of it's that if it's the base, it's it's you define. It is basically if it's a media to find, you describe it in the mid, so I don't and I don't I don't have to be honest. I don't I don't use ratios too often. It's more about like I said, having a really para appraisal of the left ventricular cavity in the short axis or left ventricular wall things in the short axis on make and making sure you can try and see is many segments is possible on describing just being descriptive on said it, you know it is the hyper literally started the basins onto a basal intercepted Was it in spiral down to the mid in for a septum, you know, Where does it go? Where does it start? When it wears, Where does it go? Other areas remote to the septum as well that I purchase feed on down. And then in your conclusion of the report, you know, try and given overview of the of the phenotype Be a reverse septal curvature, asymmetrical central part B A C M and then comma maximal thickness. Thank you, Jesus. Cool. Laura on. Thank you very much for that Jim's, um And against that is it for tonight. So again, Thank you very much for when he joined in. And thank you, James, for agreeing to do this test on tonight. Thank you. All right, everyone enjoyed a rest of your night on. Uh, please do shared a word about this product. General top. Thank you.