In this Heart Rhythm Webinar Series challenging case discussion, Mayo Clinic cardiology experts Samuel J. Asirvatham, M.D., Abhishek J. Deshmukh, M.B.B.S., Siva K. Mulpuru, M.D., M.P.H., Ammar M. Killu, M.B.B.S., and Christopher V. DeSimone, M.D., Ph.D., and guest panelists Melanie C. Bois, M.D., and Narayan (Guru) G. Kowlgi, M.B.B.S., discuss inflammation and rhythm disorders.
For more information or to refer a patient, visit Mayo Clinic Medical Professionals — Cardiovascular Diseases .
mm hmm, mm hmm mm. Well, welcome everyone to our heart rhythm. Clinical discussions we have in our series. Thank you for those of you who sent in some questions, please do uh feel free at any time to bring in questions or present anything that you feel would be relevant. Today's discussion topic is looking at arrhythmias in a specific set of patients those with inflammatory type causes for structural heart disease. We have doctors fish book dr DeSimone, dr Michelle peru and dr kilo here with us along with dr guru, khalsa ji hoo present an interesting case and today featured part of the session will be a part with dr Melanie voice of cardiac pathologists who will show us some of the sequel e of what we see in autopsy hearts in patients that we try to intervene on as electro physiologists. So to uh to get get started, you know, what what are we actually thinking of here? We've got we've got a set of diseases that have some unique features. We talked earlier in this webinar series about patients with structurally normal heart sweetie. And these were a unique set of patients locations like the outflow track the fast sickles and a common set of patients we deal with. Very important in understanding that group and complexity. It's just an enemy of the normal heart. Something that we discussed in some detail. We have some questions and discussion further on it and we'll bring up in cases that we present through the course of this series but we also have conditions where we have abnormalities. For example, when we have a patient mapping Pvcs but then when we look at the signals, the ventricular electra grams and sinus beats, ventricular electra grams during PVcs multiple areas in the heart. The signals aren't normal yet. Unlike the substrate VT discussion and approach we had where we haven't known in theology like ischemic heart disease or a familial inherited problem like lamb in apathy, we have a borderland type of conditions. It's mostly normal except there's an ongoing process. So this is a challenging group of patients because we need to have a thorough understanding of anatomy like with structurally normal heart VT and atrial arrhythmia. But we also need to understand dynamic pathological changes. It's challenging for treatment challenging to understand and to try to facilitate this process. We will go through a few cases and then we look at some actual pathology to try to understand a little bit more about this disease process. Now do we have Dr college E on with us here? Yes, I'm here. You want to introduce yourself and what you'd like to share. sure. Hello everyone. My name is group called the M1 of the Finally EP fellows here at Mayo Clinic. Happy to be here. I will be presenting a case which will base the discussion there after so I can share my slide if. Sure. Good. Okay, can you all see this? Yes. Okay, so this patient is a 50 year old male had comorbidities including hypertension has systolic heart failure with ejection fraction of 47%. Um was known to have frequent PVCS. He also wrote afraid to sleep apnea. He was on CPAP therapy, had morbid obesity and had undergone ruined by gastric bypass surgery a few years ago and he was as a non smoker and he had symptoms of Disney and palpitations and occasional chest pain. His chest pain was somewhat atypical, did not have any exertion all features and had this burning discomfort for many years. So he was evaluated in cardiology clinic for an abnormal stress test. This was his baseline BCG and as you can see he had sinus rhythm but had some evidence of conduction disease, has right bundle, branch block has a left axis deviation, likely left anterior vesicular block and china's credit cardio. And this was his holter monitor. He had about 19% PVCS and the PVCS were all uniformed. Will it looked like a right bundle branch morphology in B one transitioning in V. Five. We had one prior that looked like that as well. And then some of these had some runs or non sustained ventricular tachycardia where the initiating beat was similar to this BBC and then he had a different slightly different morphology of the beauty. And then he had a stress test done at one of our satellite facilities And on the left panel you can see arresting images in the right speak stress. This was an exercise stress test. He went about eight minutes had some maximum heart rate response. But you can see there was some wall motion abnormalities. And I played this one as well. So we had both had a pickle lateral wall and also some bass and lateral royal abnormalities here on wall motion. So, so that was his initial presentation. And then he was evaluated in cardio. So the evaluation was here. Patient with conduction system disease, not previously known. VT and PvCS. Pvcs one morphology. VT non sustained, maybe another morphology. A suspicion of coronary disease because of an abnormal stress test. And then I assume the patient had an angiogram. Right? He did. Yes. And if you could just tell us findings on that in general, did not have any obstructive coronary disease on his angiogram. So if I have to summarize this patient, your thought processes, why is there this seemingly new onset conduction disease ventricular arrhythmia more than one location. Right wall motion abnormalities, dressed and stress. But normal coronaries, correct. If patient presents like this to you, your thought process on what you'll be thinking about. Yeah. So this is, So when I see evidence of conduction disease and Ventricular hated me. As in a relatively young patient is 57 obvious like to think of certain conditions that one of them would be cardiac sarcoidosis. And then um in this patient there was no familiar history but some other rare conditions could be Lamont cardiomyopathy is where you can have conduction disease and have ventricular arrhythmias. So there's a couple of things I think of. Very nice. So really like what does clued you in towards? Okay we need to think about an inflammatory process. Is either the presentation of conduction disease or ventricular arrhythmia but not fitting a pattern. And you're thinking why are they kind of going together? Do you want to tell us what happened with this patient? Yes. So this patient unfortunately. So after this evaluation was completed a few months later was found unresponsive by his wife at home and E. M. S. Was called and by the time he was brought and he was declared dead on arrival and then had an autopsy senior. So this patient passed away without any further specific evaluation. And we're going to discuss some of the autopsy findings in this patient coming up in just a little bit. But if I if I now go to uh the individual aspects VT and the conduction disease maybe we can discuss a little bit, see what questions we get from the audience and and and then we can come back. Two looking at what what the autopsy findings are and what we learn when we do ablation on patients like this. So maybe do you want to try to talk to us about this E. K. G E. G. Sure. So I'm looking at the CCG just atrial rhythm it looks like it is bye physic and be one upright and the inferior leader looks like sinus rhythm. And then there doesn't seem to be any correlation with the ventricular with them. So appears to be sinus rhythm with complete heart block and the underlying and when trickle a rhythm. The curious is also slightly wide um negative and we won sort of notched in one in A. V. L. Sort of a left Brundle junction and escape. Outstanding. So you know this is this is probably one presentation where electro physiologists have to get clued in. So we have conduction system disease. But although we don't have BT on this tracing, we have suggestion that this patient has gotten ventricular skull, You very nicely pointed out they change in way front the glitch or the notch in different parts of the Q. R. S on the QRS complexes here. So even though there isn't retail, we're thinking here, conduction block could be multiple reasons but there's also a scar in the ventricle. So that's like a surrogate for DT Plus conduction disease. Much like your patient thinking about this now you put together a very nice slide of like potential causes for a V. Block of relevance to electro physiologists. When we see. Do you want to give us a brief look at how you use this information. Sure. Absolutely. So whenever, so this is what you should was on a 50 year old professor actually was having exceptional shortness of breath when he was giving long lectures and going for walks. So whenever that happens he got a E. C. G. That showed complete heart block as we saw young patients complete heart block. You know it's almost like a class to a recommendation that we have to rule out certain inflammatory cardiomyopathy. But generally when we think of complete heart block in patients with younger patients, degenerative disease, limes disease can be seen. Even covid now is causing some A. B. Block and bradycardia. Apart from that, you know, prior history of radiation is important and beyond all this. You know, sarcoidosis really the main uh condition which we really need to be aware of to make sure that you know, we don't we are not missing. Now the key thing with sarcoidosis some of these inflammatory cardiomyopathy is that they can cause a V. Block and they can also cause VT. So both are extremely concerning. And as we picked up from the g the fact that the patient had fractionated QRS you know, we you know, the patient does have underlying substrate to have a ventricular arrhythmia going forward. Yeah. Go ahead. And now the thing which comes becomes challenging that when we see these patients, if the ejection fraction is normal, you know what kind of device to offer, we'll come back to that. Okay. But we just stay like let's say for our trainees new electro physiologists you know you sometimes get a big list like this and we have to have some method of how we're looking at it maybe I'll just share mine so advanced A. B block anybody can get it. But typically you're going to see it with the degenerative population. So you're going to know the age of the patient and you'll see other features if atrial scar, atrial enlargement, things that go with age and you have the age of the patient. Advanced davey block probably we don't think too much more things that would be classic scenarios which should make you think of a condition. one is rapidly evolving conduction disturbances, Bundle Branch Block one Day, Complete Heart Block Another Day. One condition there we have to think as giants are my courageous. So VT and heart block likes are coy with this condition but it's a rapid not a sub acute it's an acutely evolving syndrome to make us think about that. Now I appreciate you included you know Lyme disease as a cause of block. We have sugars. We also had ankle. Oh since pond light is all cases that we see with this type of presentation come to us because they also may get VT So some just clinical clues to this. Lyme disease tends to be higher level 80 blocks. So tends to be QRS is okay we don't see some bundle branch block and closing spondylitis very very similar to the type of block type of E. K. G. That you get with sarcoidosis. The difference is the, well you will you always have some aortic regurgitation when heart block is as a result of influencing spondylitis. So there is just combining some Oscar rotation or knowledge that they have some disease of the eye, arctic melt Becker's and dystrophy is the QRS is abnormal. Classic feature here is the appearance of right ventricular involvement because of posterior scar taller with In B. one sometimes very slow process, nothing evolving like with an inflammatory carding mill radiation as you pointed out, very similar to what we get in many of these. But difference is hard unusual to get sole myocardial or conduction system disease. You'll see multiple valves, involvements, maybe coronary involvement as well. Last point a mate is with sarcoidosis. You can start with a V. Block and then get bundle branch block in the escape. Whereas in Chavez disease you start with bundle branch block particular blocks and then can progress towards complete david block. So that's one angle. That's one place that we get this type of clue to try to avoid where we're getting to a patient that we missed the diagnosis and passes away. Suddenly the other is Bt and that's something we're accustomed to and many of your questions around. We'll come back to that. But I'd like here to just first of all thank Doctor Melanie boys who is going to talk us through and show us the autopsy heart uh to see what allegiance look like, how the diagnosis is made in the autopsy laboratory. but also to give us some clues when we're thinking about mapping or a bleeding VT. So I'll play here. So dr Boyce really want to thank you for giving us a chance for seeing the actual growth and enemy gross pathology in patients that we evaluate an oblate for sarcoidosis. So, so looking at what we have here, uh, do you mind pointing out to us what you see in this patient that looks at normal and we'll compare this with a similar section in someone with scheming heart disease. Absolutely. So when I start to think about what looks abnormal, I think the best way to start is to really identify normal. So here this coloration here is a normal color in the Maya cardio in this particular specimen. Now this specimen is fixed and it has been fixed for several years. So it will start to lose a little bit of its coloration. But I'm looking just at this side, what I start to see or some white areas here here, right in here that are representative of either scar or active inflammation. Um, within the myocardial itself. Now, just looking at this by itself, I would think that it could be an ischemic event that could cause this or it could be a multi vocal myocarditis that's really only manifesting at this mid ventricular level in this location. But the sub epic cardinal nature of it starts to clue me in that might have something going on in relation to an inflammatory disease. Rather than s chemical heart disease. Maybe I'll point out a few things for uh the electro physiologists here. So one thing that we noticed is we need to understand very accurately what the overlap areas of the right and left ventricular because it's this septal area where various amounts attempt that we see the lesions that cause for promote reentry with ventricular attack, guarding. Not for example, we intuitively understand this region as inter ventricular septum and think about the bleeding from the right or the left ventricle. However, look at this overlap area so called anterior crescent and posterior present IQ regions of the right ventricle. You really have to insinuate the catheter between the tribulations and if you do and get to this location mapping this region becomes quite straightforward. Trying to a blade that same region from the left ventricle. But present biotechnology is biophysical methods is very, very difficult. Note also that a catheter wedged in this location to pick up voltages from this maya. Cosmetic region with simultaneously record signals from the right ventricle giving us a false negative with normal electra grams. But looking at the quality of the electro grams, we can see the slow conduction around this region. That voice. Could we compare this with what we would see in ischemic heart. So in ischemic heart disease. Looking just for orientation purposes, we have the anterior left ventricle gear, lateral inferior posterior ventricular stepped of it, right. And in this particular instance, we have a scar that has formed within a vascular territory. In this case the left anterior descending coronary artery territory, there's also very little scar forming and other vascular territories, which helps us to understand that this is what we're seeing in the scheme of heart disease. Also note that advanced remodeling that has formed when there's dense fibrosis within this area um indicating a remote myocardial infarction. Non pathologist. When I just examine that scarred region, I see what looks like some normal tissue. There. Would that be what we'd expect to see when we look at this? This to logically as well, it depends on the degree of the infarction. So you can have sub into cardio and functions which would result in scar tissue in this location and then other normal maya cardi. Um um Further out in the mid mural and sub epic cardio regions. A trans mural infarction, on the other hand, would affect the entirety of the left ventricular wall in this case, um and would mostly be representative of scar tissue in this location, we will have some insinuating normal maya sites that have survived that ischemic event, but the majority of it will be scarred and remodeling along with some fatty. This is a critical image for electro physiologists to look at and compare first notice the sharp demarcation of visibly normal and abnormal sites. This is what we see with our capital. In other words, the voltage cut off that you used to record the electron atomic map is much more forgiving with scheming heart disease with its growth abnormal versus areas that are completely normal. So if we take, for example, a window between .05 million balls As low as that to say, one million board, we'll get a tremendous differentiation of the area that we need to concentrate for substrate modification. On the other hand, with the example that Dr Boyce showed us before, there's really no area that's trans muir really abnormal or perhaps even transmit really normal. So the gradation in voltages differences between affected and unaffected sites are much more gradual, much more nuanced. So to really tease this out, we have to rely on the quality of the local electro grants rather than hold it. You know, this is one of the limitations of substrate ablation entirely based on bipolar voltage. No dance. So there will be a tremendous difference between a catheter placed here looking at uni polar voltage in this region versus this region, but not that different in bipolar voltages. On the other hand, with the skinny card infarction patient, we'll have no real difference in either unit polar or bipolar signals here or here and a major difference between bipolar or unit polar signals here or here. Now. I notice Doctor Voice that as we move away from this obvious kind of impacted area. It just looks like there are streaks of scar, streaks of abnormality now for the genesis of arrhythmia. We really need viable tissue to be able to sustain the Iranians. I'm just curious what is it that keeps these living My cardio settles viable after and in fact has happened. The reason as this is one of the debates and electrophysiology is whether the remaining tissue that we see as pregnant as stars fragmented signals within scar is perkin gee that has a higher threshold for a screaming death compare. Or is it actually my accordion that survives any thoughts that we need to keep as E. P. S. When we see something that looks alive even though the rest is obviously scarred and death. A very interesting question. One that is clearly very nuanced with regard to the literature and evolving research as well. I think what we're seeing um can be variable again depending on the degree of the Islamic in front consist of it and whether or not the patient was reaper fused at the time of the event. So re profusion injury can happen along the borders of the ischemic event and can lead to paki Maya site. Um survival and patching Maya's death depending on the insult to that particular self. Um So I would I would think that honestly you have a mix of normal Maya sites as well as for changes that have managed to survive. Um Thank you. So so we'll uh come back to dr Boyce who has kindly looked at several cases from a pathology perspective and we'll come back to talk about that. But I want to look first before we go back at some of the other side. The other side of where we get a presentation that clues us in to a complimentary process is an evolving ventricular arrhythmia syndromes. So maybe uh this is a patient I know guru, you're familiar with this uh tracings and we'll get your help with this but very similar to the case you presented as well. But conduction abnormality, acute development conduction abnormality. So it's not going to think lives disease in a grace disease. Something's going on with all of this. And as right before your eyes as the days is the minutes to hours progress. You not only have evolving conduction abnormalities but you start developing ventricular irritability and then you get frank ventricular attack occurred. Mhm. So this specific type of rapidly evolving, rapidly evolving. So this is not something that is that going after days or months thought processes and your approach, maybe we'll get dr k lose perspective on this type of syndrome if you have this evolving your thoughts on how you approach this. Uh huh. Yeah. So sorry, I wasn't sure if you're asking guru still. So when I see this I do worry about an active myocarditis. Um So some severe inflammatory process going on within the hard whether it's a giant cell myocarditis um you know something like that. So it needs very prompt and aggressive management. Um Often a multidisciplinary approach I think. So that would be one of the main things that I would be worried about in this kind of situation. So am I when you say multidisciplinary approach, you're saying that like, you know, like with Giant Cell for example, this can go so bad, so quick. You want to have heart failure, advanced therapies, support transplant, all available and probably rheumatologist, pulmonologist who may be more expert than as an immunosuppressive therapy involved. Those would be the big too, I think, isn't it? Absolutely, because it can really be competent. You've shown the slides here, but before your eyes, as you said, it can happen. So it just needs to be all hands on deck, I think to really try and optimize the outcome for the patient. Um one thing I would as well, sorry, is I've seen something similar with checkpoint inhibitors and I think someone mentioned that in the in the texts with medications we're seeing for patients with melanoma for example. So just important, I think to be aware of that, Yeah. Do you want to expand a little bit on, on that to see like what times or time course of that syndrome and prognosis would be prognosis isn't great or much to say it's actually poor, but it can be reversible treatment requires immuno suppression as well, but it can evolve very quickly. I've had one patient in the last couple of years where they presented with Sync api and they had advanced A. V. Block but then rapidly started developing ventricular tachycardia um diagnosed quickly or at least the suspicion was there started treatment very early. But unfortunately the outcome wasn't good but it cannot also evolve quickly in a similar fashion to this, at least in the rare cases I've seen. So, you know, one of the one of the things and this is why I believe Doctor County had presented this to our group was early in the pandemic. There was this recognition that Some patients with COVID-19 did get cardiac involvement and that involvement was my cardio and conduction disease. Uh and uh this is in keeping with what we see in other types of my academic processes. On the one hand we may have re polarization abnormalities. This kind of mimic of an easier syndrome. Other hand, we have just heart block a V block coming in pretty early and that closes in to just like it would in other times into a possible inflammatory cause. And then the same thing of despairing with ventricular tachycardia, arrhythmia and conduction block. But with other features of inflammation. The instability the dynamic city of the course and some inflammatory changes that may involve the pericardium as well. Now generically we can think about inflammatory myocarditis as like viral versus auto immune but within vital also there may be some differences. For example, in patients with coxsackie virus, a very common cause of myocarditis worldwide. When a virus is identified, you tend to have simultaneous involvement of atria nodal tissue and ventricle so early in the course. Sino atrial block, sinus arrest along with bundle branch block. Not so common and very rare and circuit for example, very rare, which I answer my card itis to get prominent sinus node involvement that might clue you in onto this diagnosis. Another unique syndrome also tends to occur endemically sometimes is part of a viral myocarditis. Big thing here is n ot earlier involvement. So for Kinji is affected more. Other evidence of industrial dysfunction and one of the ones that's easier to biopsy because it is an immaterial dominant type of disorder. Couple of points we should remember. It's not always VT and heart block. Myocarditis tends to produce inflammatory types of straights in many places. Plus the patients may be very sick. They may have a RDS, they may have other, they're critical illnesses as part of their clinical course. So, multifocal atrial tachycardia. There's something that can complicate the course in these patients and the real risk in this dynamic city is you have these multiple sites that can each produce maybe non sustainability but they can all do it at the same time. So this is like an exponentially severe E. P. Study going on continuously in this patient population and exactly why dr kilo pointed out the minute you sniff the diagnosis, you're going to get ready to get supportive therapies, transplant Elbert. Because this is a tough one for us to try to manage with the catheter. I should also point out that in patients with inflammatory card itis they may have a pericardial syndrome that involves the ganglia. So these patients after they recover may be left with things like an appropriate sinus tachycardia, parts like syndrome or even neuro cardiogenic syncope. E that they never had before. What comes after this myocarditis critical illness type syndrome in both aspects of that are probably playing a playing a role now, one of the one of the questions that we had related to a kind of substrate mapping approaches uh in patients with inflammatory disease and may be our last doctor will follow to get us get us started in that thinking any things that we should be aware of or think specifically when we're thinking about electra graham interpretation substrate in patients with inflammatory that you use a compared to like how you do it in other syndromes. So as you showed him the autopsy specimen, you know, the scar is kind of patchy. It's good to get some sort of imaging either a cardiac MRI or pet scan. So we know where to focus our efforts in the ePA lab. Once we get to the E. P. Lab, it's good to use some sort of electro and atomic mapping system to tag areas of abnormal electrical signals that correspond to our imaging abnormalities. And it is those areas. We should try to if you're trying to take a biopsy those are the areas we should try to target for a biopsy. As far as ablation is concerned, these patients are quite sick um in an acute phase we really don't take them to the lab let acute heart failure to manage them after the scar um after the scar forms. But the situation has stabilized. That's the time to take them to the lab if they're having a recurrent arrhythmias. One exception is PVC induced ventricular fibrillation. The patient keeps on having multiple episodes of ventricular fibrillation refractory to other therapies. Um You know we may have to do a very focal targeted ablation just targeting that clinical T. V. C. And hoping that we at least reduce the burden of those BF episodes. Fantastic. Thank you. So uh maybe I'll go to this year And if you see other questions also let me know. But here's kind of a slide that dr fishman put together. So very nice kind of pictorial view of what this patchy kind of involvement can be just more fixed more scar than the heart of the patient that dr Boyce looked through. So can easily kind of get that idea that we're going to have some unique electro graham correlates in these patient populations that were not used to seeing in others this interest first thing of normal abnormal type tissue coming so close together. Now just in terms of distribution uh the sheikh, I know this is a slide that you put together with sarcoidosis in your mind. Do you have some key areas you look at which are very likely to be abnormal with sarcoidosis? Or is it like civil pointed out? We just need detailed imaging and mapping. I think certainly, you know, starting off with the S. E. G. What we saw. I think from there itself we can start to in our minds think about where the abnormal substrate is going to be looking at. The fractionated QRS is certainly imaging is going to be very, very helpful. But as you saw on the autopsy specimen, certain areas even if a map and accordingly we may certainly miss it because it is so patchy in present involvement and certain areas we may not be able to clearly map to know whether this is uh you know, whether we have any abnormal signals such as deep into the septum or epic ideal substrate. If we have not done a Picard l. You know mapping. So I started with imaging and then tried to horn on my horn on my mapping more in that direction to at least try to get get some insight into the clinical arrhythmia what is going on. And then finally, you know, the VT morphology is certainly helpful. The device interrogation and the tracings are going to be helpful and all those kind of will clue you into map which area of interest beyond mapping everything else. So, you know, one of the things like a victim in our field is in the right ventricle when we think does the patient have sarcoidosis? Or do they have right ventricular cardiomyopathy? The two diseases where we may have some familiar things like outflow tract cardiac, but it's not in a normal heart. One of the victims is sarcoidosis, acceptable disease. Right ventricular cardiomyopathy is a free world disease. But I think it's important from the picture you shared here that this is a it's not that sarcoidosis doesn't involve the freedom. It just has a predilection for the septum, especially the high inter ventricular septum, the region of the ventricular crest. And is the reason why we get bundle branch block and david blocks so commonly with sarkozy. And it's unusual to have soul free while involvement. So you won't get this without getting this unlike right ventricular cardiomyopathy, where it's extremely unusual to get septal involvement and pretty much unheard of to get septal environment before free wallet. So now uh guru, do you have a uh ice image of a patient from biggest relation to kind of show us this uh sexual involvement. Yes. I do not know whether I can share my scream. Yeah. Can you see this? Yes. So in this image you can see the crest of the inter ventricular septum here and. And in this patient mapping was performed in this region. An ablation was performed substrate by stipulation on the septum. So I would guess if you're blade where you're showing you're going to get a B. Block? Was it was the reason you have plated there? The patient already had a B block from those lesions? Yes, patient already had a B block and I had a CRT device implanted. Yes. So this brings up an important point. one of the hardest vts to oblate is high ventricular crest crest of the inter ventricular septum beat is very difficult because you're going to get a B block. But the flip side of that is the most common scenario to get the key from there is sarcoidosis. And in those patients, the reason they're getting the VT from there is the implementing the lesions. So good bet they have either really bad conduction to begin with or actually having the block. So we're able to plate in that region. So maybe I'll kind of show the uh equivalent image for that region that you you've shown us from pathology. So this is this is kind of what you showed us. Let me go ahead. So it's really this so inter ventricular septum and you show beautiful image from your ablation case of like uh huh at this region. So in this ventricular septum when you go to its highest point, you're above the center. A little bit of the trigger stick that so this is actually approached from the atrium and when you approach it from the atrium, you're above the valve, you're on the septum that's conduction to shoot territory. So the atrial ventricular septal region, high point crest of the inter ventricular septum, almost pattern in the morning of sarcoidosis. You should expect to see things here and you should expect to see VT here. The problem is we may fail to recognize it for several reasons. one is the patchy involvement could result in an exit of the VT fairly far away from where the circuit is housed. So we're focused on these regions. The second is we forget this is winter where we keep the track hospital. Well, we go up above that. We're feeling that we're on the atrial side. But this actually this region is approached from the side. Now um seva I see there's a question from one of the, from the audience about uh the role of biopsy uh in in sarcoidosis. What are we using to target the area for the biopsy? And maybe a I'll have you comment a little bit on that. So, and then let's see if we can get this slide to come up here. Great. So, you know, biopsy, it has to be a star koi is one disease state where you have to do targeted biopsy. Unlike other conditions like amyloid giant cell where there is more of a global environment of the myocardial with sarcoidosis, you just go and take pieces just from the RV ethical septum humane miss the actual area in world areas. So one way to pick the abnormal myocardial regions is to use the electron atomic mapping tools here in the picture you can see there is an ablation catheter. You create an electron atomic map, looking at the quality of the signals and also the voltage amplitude of the signals. And usually if you have Apache scar and in the border zone where there is fractionated signals, you know where the voltage is transitioning from scar to normal but still not completely normal. Those are the areas we should target here. I believe the catheter is coming from the internal jugular vein. And sometimes we use an alligator clip. I think you have it on the other slide to see also try to map from your bio dome. So you can use that as a marker and you display the electra grams using that biopsy domes. So talk us through the setup for this. Uh how would how would you do this? Okay, so here we could see that you are to be black cliff of this is almost like a pacing cable that we are using. We are connecting that black alligator clip to the to the metal spring there which is in continuous uh connection to the distal tip. It's almost like a uni polar electro graham if you record this and you use either Wilson central terminus or if you connect the red alligator to clip to a patient like a needle on the patient's body or a forceps that is in contact with a patient issue. This is almost like you know using it to obtain uni polar electro grams here. Okay, so you connect this up like this and then you'll have this maybe touching something metal in the patient and then this is what we're using to biopsy. So what will maybe we'll ask. Maybe she could put this together for us. What would you look at the shape to kind of say I'm going to do a biopsy here. So first of all, when we are mapping, we need to identify how the normal myocardial signals look like. And then if and then as we are mapping along, say on the septum the clues what I look for is if we start to see any fragmented signal or if we have a his bundle catheter then if we are I'm seeing that the delay between the near field and the far field ventricular signal, what you can get from the his bundle is quite further apart. So that will tell me septal delay. Then as you're going down the septum if you start to see these abnormal signals then at least I attack them on the voltage. Attack them on the mapping system as you have seen on the left, the pink dots and then on the green dots. And then if I find a safe location predominantly on the septum then you know, we biopsy that mind. Well that's our coil as we talk, likes to hang out more in the septum. So overall specificity and sensitivity of getting the diagnosis is a little bit better of its archives compared to other inflammatory cardiomyopathy. But this is generally the approach I would use and then really depending on the clinical situation, if we are really compelled to biopsy the left ventricle or RV free wall at least I would shy away from RV free wall but certainly we have done even LB biopsies for these patients. Fantastic. Thank you. So if we go to another question that had to deal with we'll try to get as many of these questions as possible. But about inter cardiac electro graham maneuvers specific in these populations. Maybe I'll use this patient as an example. This is a patient with recurrent VT and a known implemented very myocardial process. So the diagnosis was already known question is how are you going to deal with the VT? So maybe guru. Maybe I'll ask you to comment and chris feel free to chime in as well. Looking at this. What's your thought process for planning the ablation procedure? Yeah. So just looking at the CG white complex tachycardia. I'm looking at. We won first and and very wide notched fragmented QRS and then um sort of transitions and we three that's where he slurred on set tells me it is it's taking time to engage the conduction system and then sort of left bundle early transition, inferior access and right word access. So I would think of something on the septum. Yes. Tallinn field it's a positive but not very tall, suggesting high in the outflow tract. So maybe basal septum so very nice. So few things very important. Things that guru has pointed to us. one is we're used to saying that when we have a delayed upstroke in the VT we're used to thinking that's epic article and that could be true. But what guru said is probably more accurate. It's not so much a cardio but you have trouble engaging the conduction system. Heh, Picardy um is away from the conduction system. So we see this in a pericardial betes. But if we're in a region where we don't have conduction tissue like near the annual lists where we have a positive of working G tissue. Yeah. Metro Manulis, lateral bicuspid analyst, lateral or the conduction tissue itself is severely diseased. And this is exactly the subset of inflammatory micro status. We have VT and we have really bad conduction system. So we expect to see VTS which have this epic cardio look because conduction system is bad. So even if you engage it, you're not going to get much from you. He also pointed out that you have multiple late fragmentation and this is a clue to us that we're going to see more than one week because late in the meeting. Somewhere away from the exit? We have slow conduction and we have scar. So this is not going to be the pathology section. Like what DR boy showed us of a discrete scar. Like the patient with the in fire. It's going to be more like this patchy substrate that we have. The inter cardiac signals can be pretty unique as well. Maybe I'll ask you guru as well. Uh Maybe talk us through what you see in the sofa orientation. We have the C. G. Gotta his fund recording site catheter. We've got an ablation catheter somewhere in this case in the right ventricle. And then we have the coronary sinus seated nicely in the CS atrial paste. Yeah. So we can see uh to pacing spikes. The first pacing spike is followed by um atrial signal from the coronary sinus. So I think that is a natural spike. You see p waves on the surface and then a second pacing spike with a positive QRS. And we won. So could be left ventricular pacing. And then on our intra cardiac, on our his catheter we see two sets of signals. I think that first one is too late to be an atrial signal. Probably both are ventricular signals and could be suggesting delay across the septum. And then we actually see a change in our QRS vector from the 3rd and the 4th beat. So with that there's more delay. Especially noted in the approximate his. Sorry if I ask you guru if I understand right, what are you interpreting this signal to be? Because this becomes the game when we're mapping either during the or during substrate is dynamic city. Of the signals will have to figure out which signal are we going to use, whether that's entrainment, whether that substrate, whether that's activation mapping. So what do you think this signals? I still feel it is also when trickle a signal, it could be the other side of the system. Not at all tagging with the so we would have to say this has been tricky but if this is ventricular, why aren't we seeing it in this beat here? I feel there are two components in in that beat as well. They're just closer together. Um as these are exactly what we need to keep our eyes open for Just spontaneously. Even with that late we can have this amount of change as a result of conduction. So instead of being together here, they're going to come apart here and notice the QRS changes as you pointed out as well. So this pump here is hiding inside the cube. Arras just like the signal is hiding in the ventricular electra graphs. So these are signs of not just slow conduction but dynamic city. And what do we mean by that? Maybe I'll use the board here to kind of come back to explain that. But we have When we see two signals like this separate, that could be a fixed block. So we have a catheter that somewhere around here and that's picking up the wave front that comes towards this needs to skirt around and come to this location. So it's a fixed block. On the other hand, if sometimes it looks like that. Other times it's together, it's not a fixed block. It's so close to being a block means it's very slow, easily detrimental conduction. So could go through or could block. So just from these type of signals, we know we're dealing with substrate for heating. It's slow conduction. So close, so slow it can block and it can do it with very little change in break, meaning we don't even need a close couple PVC to potentially cause this change in wavefront. And could get us started with a P. T. Type VT type process. So this becomes also important when we're doing entrainment. So, I think one of the questions dealt with entrainment issues when you have uh are you all able to see the screen here? Mhm. Yes. So this is an example also of how you can have these changes happen in real time change with ablation. So here you have eyeballing this, you've got pacing during the tea, Stop pacing. VT resumes, facing. Speeded everything up and has a morphology very similar to the HVT that we haven't slept but few things become issues when you have this type of substrate. Like we see this interspersing of normal and abnormal tissue so close together that you see this type of phenomenon. If you have these late signals here, your Q. R. S. Is going to look different for when you have those late signals to when you don't. So even though you may have concealed fusion during the tracing, the Q. R. S. Could look different because these bystanders sites may have detrimental tissue. So you're really in the circuit But downstream you may block two chunks of my accordion or not. So the Q. R. S. Could keep looking different even though it really is one circuit and you really are in that circuit. So how to get around it is you'll have to fix your reference on parts of the Q. R. S. That doesn't change. Are an electrode placed at a site that doesn't change and use that for your interpretation of entrainment. So I hope that kind of answers the question from our colleague who brought that up. Let's just look through some of the other questions here and maybe we can do one more and then we'll do the rest while we. So there's one question here that says about uh sarcoidosis related video ventricular rhythm. So this is a very nice question. And this is a very practical question, patients with this is true. Also with the checkpoint inhibitor therapy patients, it's also true with covid recovered patients and for sarcoidosis. You treat and you get this slow VT and you don't know whether you slow the VT, whether it's a drug you're using. But you also know because it's a conduction system inflammation. You can get this inflammatory related issue, ventricular arrhythmias. Maybe chris I can ask you to kind of get started on that and then we can maybe answer this and the other questions when we do our recorded session at the end of this session. Yeah, sure. I think it's tough because it can be I think any of the things you mentioned. So it could be kind of this cool down or or or warm up phase of that inflammatory process. It could be your drugs. It could be your treatment. So I'm not sure I would kind of watch and monitor to see. I wouldn't make any rush to judgment of interventions yet. Yeah, I definitely agree. So just to say like, here's the quandary. So normally we would say that if we have an idea ventricular rhythm, well, don't worry about it. Uh it's slow. We can override pace it. We can leave it alone. But in this subset of patients that part of the inflammation is the conduction system and the D a ventricular rhythms come from these bundle branches. So I think the doctor probably has had a quest patient like this and we've certainly had patients like this where it's tough because on the one hand, we have to think this is a manifestation of ongoing inflammation. So we have to look for other correlates other things with inflammation and see does this mean we have to give better immunosuppressive therapy And that's often the clue and the treatment that helps in these patients. But 100% agree with you Chris that we don't want to a plate early in this course because immunosuppressive therapy and time might help. And also what do you target if there's inflammation at multiple sites in the conduction system? Having said that though one of those patients you remember for years had exactly the scenario this doctor describes and we had on a fairly aggressive immunosuppressive biopsy proven sarcoidosis um me Orgeron previous ablation. And this was the rhythm and fast enough that it bothered the patient you couldn't really pace faster and it was depressing ventricular function. And patient actually was being taken for the transplant as an option as the likely option. And we wound up having to a blade and it was exactly the problem. Your blade one site and there is an escape for the media ventricular rhythm. So you almost have to trace out and oblate the conduction system. A very extensive type of ablation. Now you've done some work chris about the research into conduction system regulation for not inflammatory do ventricular rhythm but V. F. Do you want to give us a two minute update on that and then we'll take the rest of the discussion offline. Yeah. Sure. Uh huh. Can you hear me? Yes, I clicked off this thing. So uh it's really tough I think you know, we see all these illustrations and these pictures and these drawings that make everything so simple. What it's definitely dynamic. And that's one of the themes we've been talking about tonight about trying to map any of these things, especially information. But when we're using the Birkin gee, there's kind of that you always allude to this fourth or fifth dimension. So there's no cavett terry and there's parking delaying over these end of cometary structures like papillary muscles. And what we're trying to do now is see if we could map ventricular fibrillation and doing the mapping the ventricular fibrillation. There's dynamite dynamite simplicity of this. In terms of how much of the tissue is ischemic, how much of the tissue continue to go on and what perpetuates and maintains us. It seems like this is involving lots of the frickin gee? The distal break in ji uh more distal than proximal conduction system. And so the main thing there is to say, well what's the driver, what's the maintainer of something like if this VT goes wrong or if this meeting goes bad and to do that we're trying to do multi polar electrodes map out the creek indian map these in relation to the ventricular carried gram. So hopefully things to come. Of course this is all experimental based and we have to I think forward how to do this. And maybe patients on ECMO or advanced support where we could map out some of these areas and then a blade and hopefully treat these areas. But I think that's a good message for new entrants to Ep and also for trainees and people looking forward to the field. Some of the biggest changes that happen in our field is we take on tougher problems but it's our skill in understanding mapping, doing things in simpler problems that just have to come in there. So we have any do ventricular arrhythmia and how to manage a particular trigger. How to manage how to interpret signals in an inflammatory myocarditis. That's changing. But then those skills were translating to a huge problem like ventricular fibrillation to see if we can manage that maybe one day with a catheter. Thanks for that update and thanks everyone. Thanks doctor Additional for putting together some of those very nice slides and doctor called for presenting the case and especially dr Boyce. Taking time and going through the actual autopsy to tell us what we try to avoid and what to keep in our minds eye while we try to oblate these patients. So we'll start up here and then we'll discuss some of the questions and post it in our youtube as well. Thanks everyone. We're now going to try to go over some of the questions that you had sent to us that we didn't get to during the live part of the webinar some of your questions will just write to you independently as well. And please remember in the future when you're asking questions you're welcome to come up to the panel life and discuss this for us. We'll try our best to remind you and ask you but please feel free to just request to come up and present your case or question. So maybe this first question I'll ask dr will prove to get us started. It's really a set of questions on workflow civil. Do you use general anesthesia? Do you do substrate ablation homogenization? Uh in this like we do for say ischemia. S comic structural heart disease thoughts. So first we'll tackle the general anesthesia apart. If we are dealing with an acute myocarditis these patients are tenuous and may not tolerate general anesthesia. And second the second point is when you put patients to general anesthesia your target T. V. C. S. Or the triggers may go away. At least I would start off with propofol sedation cap. You have some trigger beats. If we're going after tiger PVC induced. Yeah so we record some templates. Now once you have the baseline template you can consider going general anesthesia it helps minimize any shifts of the map and as far as homogenization um you know I would at least target the clinical VT in this patient's when I come out of the procedure at least I want to get the clinical VT. And if the procedural time allows and the patient is stable, we can target other fractional fractionated signals areas of interest. But many of these patients may have extensive involvement and it may not be feasible to a plate everything in these patients. Yeah, I think you bring up a very good point. This is also a slide from a big shake. You know, you just think about how are you going to homogenize scar here? So you know, if we think about this is a scar and this is intervening my accordion, that's a huge chunk. Oh my a card, um that's gonna be dead. So this is I think an important difference in inflammatory disease versus esque emmick. Now with ischemia, you have these discrete areas normal from abnormal you can target them. You can and it's not really participating in cardiac sisterly. So we can emulate those areas. But with this patchy type involvement, I think substrate or homogenization is not at least in the traditional sense, it's not an option. And I think for that reason, what are we going to target? And really there's two approaches historically that are really relevant for these BTS. One that you pointed out. So that is you want to induce the wheat please as many as you can. We won't have as much clinical documentation of the Vts, as many as we can to give us an idea of this patchy scar. Where is the key areas? We want to focus the second is this is the place for the Nakagawa type approach for channel ho marginalization. So to remind those of those of us in the audience where uh what's the difference when we say scar? Homogenization is in a scarred area. We've got some kind of surviving Maya sites. And as a result a circuit could not only go around the scar but through the surrounding or even within the scar. So we target that, Get rid of that problem by homogenizing the scar and perhaps connecting it to some kind of an atomic obstacle. On the other hand, when we have this patchy type scar. Yeah, it's very difficult to homogeneous. This whole area may be impossible and may not even be the right approach because we're going to kill too much my cardio. But if we take another approach of fine mapping and we actually find this car and we have approximate tissue where we can connect scar do this. So called isthmus ablation or channel ablation. Famous saying from Hiroshi Nakagawa 1st introduced discount and in congenitally another question that came up on this was uh tuberculosis versus star coin. Uh huh. So interesting question and I'm guessing this is based on a case that may be one of the physicians had um do you want to take a shot at that question publish it? Sure. I mean I've not had a lot of experience dealing with T. V. And V. T. But T. B. In the heart can also affect the septum as we have seen in patients with sarcoidosis. The key thing with TB and how it is going to be different from sarcoidosis is that for sarkozy and active inflammation we are going to treat with immuno suppression with T. B. I'm just not sure whether we are going to be that aggressive with immuno suppression. In a traditionally we would treat meningitis and everything else with steroids and TB meningitis or miliary TB. But I'm not sure about TB and when trickle arrhythmias but certainly in those patients anti tuberculosis therapy along with again, mapping and ablation is going to be helpful. T. B may also be affecting september predominantly but can certainly have patches cars. But I think maybe the classroom you can shed more light on it. Yeah, I think it's a syndrome that's really kind of was brought to light by dr Nardi semen from Hyderabad and it may be under recognized especially in areas of the world where tuberculosis is common. But you know, the SARC I tuberculosis are tough diseases to distinguish from each other and to begin with in any part of the heart but clear documented examples from this group and others that your biopsy and rest of the testing showed tuberculosis when you actually would have been thinking sarcoidosis septum VT in a particular patient doesn't appear to involve so much the conduction system as we see in classics archive. Maybe that's a distinguish er doesn't end Uh sarcoidosis rarely is a prominent pericardial disease as we know tuberculosis can be. I have a patient that I unfortunately have etched into my memory because not only did I miss the diagnosis once I missed it twice. It was a patient referred from another country for outflow tract VT of tumor pathologies. In retrospect. That should have been a clue that we should think a little more. But our own mayo clinic M. R. We didn't do an MRI but echocardiogram showed normal heart. So I approached the case as a normal heartbeat. E we induced sweetie. We have waited. There was some pvcs very similar to the VT they targeted in a plate Patient went patient had traveled in 8 to 10,000 miles came and then I was disappointed they had a recurrence different VT but also appropriate. That should have been a clue. But the clue to me was we should think sarcoidosis and the patient came back. We met we a bladed. We biopsied. It was not an electra graham guided biopsy but we have biopsied and found just some lymphocytic type. Myocarditis. Non specific suspicion was archive. It was not convincing. There were no other features. There was no every block. So again we a bladed sent the patient home fortunately didn't have et again but had a lot of Pvcs and then thought what it could be. Then at their home facility had biopsy and cultures turned out to be tuberculosis. So one of those who never forget. Unfortunately not one that I'm proud of missing the diagnosis twice but it is a mimic and it does become a problem because treatment for one can interfere with treatment for the other but important question. And I I'm happy that someone had brought that up to us as well. Any role of immuno suppression in these patients. So I think it will be extremely brave and maybe we can ask one of the astrakhan assignment to come and but it will be very brave in the face of tuberculosis I think to do only immuno suppression but with other so called tuberculosis sarcoidosis syndromes like in the lymph nodes in the lung when both exist. Is you treatable? So if you're going to use the minister oppression, it will be when you are having like tuberculosis idle for drug type therapy along with the immunosuppressive therapy. So I think one of maybe we'll look at two of the questions that we also did not get to while looking through this? You know, one is the ablation approach not so much like do you target uh is it homogenization but is it easy or hard to a blade these sites? So radio frequency cryo assumed that's where they were going with that question. Maybe I'll ask um are dr Kalu to get us started and then her doctor this morning comment as well. Anything in this patient population and maybe a model also ask you to see as we haven't really talked about just dilated cardiomyopathy today. Where does that fall in the spectrum of our approach with ischemic? Pretty versus myocarditis? Sweetie. Where does just the idiopathic dilated cardiomyopathy play and any thoughts for us on energy source or causes for difficulty with the operation which is to our population. Yeah, so it's a it's a good question. Um in terms of SKM it versus non ischemic there's a higher pre election with non ischemic or dilated cardiomyopathy to have made mild cardio or epic cardio substrate. Whereas the scheme it tends to be sub earned or cardio. Obviously there are exceptions. For example, inferior wall myocardial function. You may have more epic cardio regions but in those patients where we're suspecting mid my cardio or epic cardio have a low threshold to perform pericardial puncture mapping and potentially ablation. So that's one thing that I would consider um as you showed in a very nice figure, the muscle thickness obviously can be preserved whereas with ischemic it may be thinner such that even if there is something deeper you can usually reach it in terms of energy source. I predominantly use radio frequency just for the flexibility that it provides. But I think cryo has an important role in certain situations, especially if you're a bleating on an endo cava terry structure. If you need some stability if you're worried about proximity to a certain structure that is important that you may injure where there's conduction tissue, coronary artery, something like that. I think there's a role for injunctive approach is also, I think may be beyond the scope of this talk, but whether you can kind of use the advantage point of the coronary arterial or venous system for difficult to reach areas as well. Mm If I understood you right there um are using the coronary arteries and veins for ablation. So, uh, so existing cases, existing reports, it's kind of maybe I'll use a figure here. We're thinking about a pericardial approaches. Something catchy something in the epic cardio space using the coronary vasculature. So one way is to actually find of venus branch or an arterial blanche and we use that to target for ablation. We've spoken at some prior sessions about venus system, alcohol Hotwire's to try to a plate. But arterial approaches, do you want to briefly review for us and then maybe I'll get some extra comments from chris Sure, the archaeo very early on, you know, for VT mapping people have put down like uh, oh and four wires to map the earlier signals, you know, very early papers. And similarly you can use. So when you have an area of scar, you can make the scar more homogeneous by using um an arterial route. So either you can inject alcohol through it some papers describing gel injection or completely occlude in that vessel. Um you know, hoping that you will completely and far and you homogenize the scar area. I haven't done that for that many patients. Only a couple of patients. We have used some alcohol. Perceptible perforate range action. Thanks chris do you have any additional comments about using the bacterial system And maybe you can briefly tell us about pulsed electrical fields specifically with relation to the arteries and whether or not it's possible to target only pathological tissue and spare normal. My according holy Grail of biophysics regulation. Sure. So some interim work suggests you can use some pulse electric field and the whole meaning to all this is that there's different tissue selectivity. It's kind of like the Holy Grail tissue selectivity to where you want to oblate, for example, my cardio tissue and not denude any of the epithelium of the coronary arteries. But also using that leverage to kill just that amount of tissue that you want to be. So, I think maybe an animal studies early studies there's some data that suggests you can do that. And the reason being is the elect electrical membranes that have different amounts of protein and the lipid bi layer and then different pulse electric fields will give you different responses to a certain dose or frequency of electro operation that you're going to give. So I think possible but not there yet. And test so kind of will probably talk more about evolving energy sources and please do keep us posted. But thanks everyone. I think we got the most questions. If there are some that we missed with right back to you separately. Thanks. Right.
