Chapters Transcript Video Comparison of Chagas and Sarcoid Cardiomyopathy Dr. Marcus describes prevalence of chagas disease and diagnosis ramifications. So today I um I opted to, to give a talk called Chagas or sarcoid Cardiomyopathy, um. And the reason that I, I, I did this is because over the years that I've been thinking about Chaga's disease, I have been really stunned by some of the ways in which it's very, very similar to sarcoid cardiomyopathy. I mean, there are a lot of obvious differences which we'll go into between the two disease processes overall, but there's a way in which these diseases are really, really bizarrely similar. And one of the things that's come up over the years that I've been doing this is that folks have been Have really gone down the, the, the sarcoid cardiomyopathy rabbit hole when they're trying to make the diagnosis of a patient, only to realize sort of many weeks or months into it that the patient actually is at risk for Chagas disease. Um, so I hope that during the course of the talk, I'll, I'll be able to share, you know, share with you the similarities between. These two disease processes and then explain to you why it matters to meet the diagnosis really for both of them because both of these disease processes are underrecognized and actually have some special import to the patient by them. So I will also just stop to say that that was a very, very kind introduction. But as you heard from it, I am really an expert in Chagas disease, not so much sarcoic cardiomyopathy. I'm an echocardiographer by training, so I will be putting information in about sarcoid cardiomyopathy that I've learned over the years, but I will at the end leave some time to answer more questions about sargas, and I unfortunately will not be able to provide you with the same high level expertise about sarcoid, so I apologize for that in advance. In any event, I've talked a little bit already about why to compare these diseases. I think they're both underrecognized, although I do feel like in the United States that people are more likely to think about sarcoid than they are about Schaga's disease. There are many crazy similarities in the diagnosis really matters. OK, so what are they? So we'll start with Chagas disease. Chagas disease is an inflammatory cardiomyopathy or Schaga's cardiomyopathy caused by trapanosomicruzi infection. Uh, that leads to a long standing myocarditis and subsequent scarring, uh, and end organ disease. Um, I put at the lower left of the um. At the lower left of the screen, the trepanosomicrui life cycle, what happens is this insect here that you can see actually in the top right, the, the rejuved bug, which lives in the cracks and crevices of poorly constructed housing generally in rural Latin America. We can talk a little bit more about the demographics of it in a minute, will bite the human host. And inject the parasite into the host, and then it spreads through the bloodstream to end organs which include the heart, the GI tract, and autonomic nervous system termmini. What is sarcoidosis? It's actually a little bit less well understood, as you know. It is a granuloma it's in trait of cardiomyopathy of unclear ideology. It's thought that there's an immunologic response to an antigenic trigger. Um there is some suggestion that in some patients it has to do with the P acnes bacteria but not in all of them. So it's really not 100% clear why it is that certain people get this particular granuloinous reaction, and it can cause intermittent myocarditis and subsequent scarring as well. Obviously there are other organs that are affected by both the infection in Chagu's disease and in sarcoid, but we're really going to be thinking mostly about the disease in the heart. OK, uh, moving along, this is, I wish that I had a similar slide here for folks, um, for folks with, uh, with Sarcoid, but this is really, uh, I think a great slide that shows all of the end organ damage related to the Chaga's cardiomyopathy. What you see here is a very large heart with scarring at the apex, and the arrow is pointing to a thrombus at the at the apex here, and this is basically all of the main bad clinical manifestations of thy disease in one slide. So you see somebody who likely has a dilated cardiomyopathy, may or may not have had reenter ventricular tachycardia from this, from this scar here, and might also have had thromboembolic phenomenon from the apical disease here. This is a patient who likely came into the clinic with symptomatic cardiac disease, probably complaining of heart failure, maybe also because of syncope from VT or from a stroke, but the fact is you probably saw this patient with symptomatic disease. This with might have had no antecedent events before they had a sudden event related to their shot as cardiomyopathy. What you're seeing here is basically normal myocardium, but you're seeing an arrow pointing to the apex here where this patient likely has an apical aneurysm. These folks can have apical aneurysms in the absence of others. Cardiac manifestations. They don't necessarily have any symptoms related to it until they have their incident thromboembolic event. So this is a patient. The only way you'd know that they had Schaga's disease would be to screen them for Chaga as opposed to doing a diagnostic test after they presented for with, with their incident event. This is a similar patient perhaps where you see normal myocardium here, but you see skin thinning and scarring of the basal infrarolateral wall. This is thought to be due to my ischemia, watershed ischemia from a thrombo a thrombotic arterioar process that occurs with Chagas disease, and this scar in the basal infralateal wall is both very common and also intensely arrhythmogenic. These are folks who can be feeling fine until they're running around on the soccer. Field and then experience sudden cardiac death, and this is a slide of the hyperinji system that you see, um, that the patients are also at risk for brady arrhythmias as opposed to tachy arrhythmias. You can see intense scarring here. So that's sort of the main clinical manifestations of Chaga's cardiomyopathy in an individual who has this disease. So in terms of the histopathology comparing Chagas with sarcoidosis, it actually looks fairly similar, doesn't it? The one big difference here is that. You see a nest of amastogoats here in the heart, but this is lymphocytic infiltrate. You see, you can see giant cell granulominous reactions, and here a sarcoid, basically similar except that you don't see the parasite. And if you were to biopsy either of these diseases, which you might get lucky enough to see one or the other of these, but you might also miss the amastogoats. So on biopsy they can look very, very similar unless you actually see the amastogoats in the in the shaga's heart. So what about the uh the cytokine and and cellular immunity pathophysio? Allergy for the inhaler you can see very similar cascades of inhibitory cytokines, interleukin 10, TGF beta, interferon alpha, and what we know is that there's immuno regulation between both of these diseases that allow, um, particularly in Chagas disease to if you have a salutary immuno response, then you actually don't necessarily progress to that disease, but if you don't, then you can actually end up with very bad scarring cardiomyopathy. So there's something really very intriguing about the antigenic triggers for whatever these, you know, whatever it is that's going on in Sarcoid looks very similar to what there is in Chagas disease. Um, OK, moving along, are they similar in terms of their demographics? Well, the demographics of sarcoid is that interestingly enough, it affects the young. 70% of folks are between the ages of 25 and 60. Most commonages maybe 20 to 40 years old. Sarcoid is actually found everywhere in the world, but more in certain subgroups in African Americans, Northern Europeans, and Japanese. In terms of sarga's disease, it also affects the young adult in that folks generally tend to get infected in childhood. And then leave their acute infection, most people without actually significant damage, but about 15 to 30 years later, so about the same age as somebody with sarcoid or sarcoid act of sarcoid or sarcoid cardiomyopathy, start to develop the signs and symptoms of the cardiac disease. Chagas disease is traditionally thought of as a disease of Latin America, but because of immigration, it's now worldwide, and you can see on this map here. Places in the world where Chagas diseases can be found all over through the Americas, through Europe, Japan, and Australia are places where there seems to be a fair bit of Chagas disease. There and I put here now domestic disease in the United States. I'm not sure if you happen to see any of the press coverage of this recently, but there was a call for making Chagas disease endemic in the United States because we have both the Vector and the parasite in the US and in the southern half of the United States. I will say that my work is chiefly with folks who were born in countries where Chaga's disease is absolutely endemic and then have moved here with the disease. And in general, I'm not sure that we have a big problem in the United States of folks with domestically acquired disease, but one thing that is certainly true is that we don't actually know because we haven't been really looking robustly. We do think there's an estimated 8 billion cases worldwide. So moving along, prevalence of sarcoidosis is a different way of looking at actually how much disease there is. You know, in the United States there's about 5 to 10 cases per 100,000 in whites and 35 to 40 out of 100,000 in African Americans. In the US, it does not seem to be a particularly significant disease in Latinos, but it does not actually mean that it doesn't happen, maybe 1 to 3 in 100,000. Um, we know that in terms of cardiac sarcoid, the heart tends to be affected in about 20 to 30% of folks who have sarcoid, but only 5 to 10% will have clinically apparent disease. So if you go looking for it, you might find it, but it isn't necessarily that patients are presenting with a cardiac presentation. But up to 25% of deaths from sarcoid are due to cardiac illness. In terms of prevalence of Chagas disease, we did talk already about how it's a disease of the Americas and immigration they're from. What we do know is that the prevalence varies by country and that currently Bolivia has the highest prevalence, which is about 6%. I put an asterisk there because we really don't have great data. Most of the estimates currently in the world right now for Schaga's disease are based on blood banking data. Because Schaga's disease can be transmitted via blood transfusion, and so the countries in Latin America where Chaga's disease has been endemic and known to be endemic for generations have started to screen. And but the problem is that some of the folks who have Schaga's disease are so poor or live so far away from urban centers that might actually give blood donations that it's very difficult to really extrapolate well. So we say that Bolivia has the highest prevalence of 6%. There are areas in Bolivia where 70% of individuals will have Chagas disease. In the US, what do we know? Well, we, we have a sense that there are around 300,000 individuals who have Chagas disease, and I'll tell you a little bit more about some of the zero prevalence studies that have been done. The 300,000 number comes from extrapolations from the published country of origin. Zero prevalence as I just showed you, 6% in Bolivia and then looking at census data or American Community survey data of areas in the in the United States and what we know about the immigrant populations there and then multiplying those. It's been updated a little bit that that estimate's been around now for about 25 years, but it's been updated using some of the newer zero prevalence data that we'd rather. gathered over the years by working in these communities and it still seems to be around 300,000. Again, we don't really know how much domestically acquired disease there is, and these estimates use some flawed data. For instance, Bolivia has the highest rate right now with 6% that's published, but the Immigrants that come from Bolivia to the US come from areas where there's way more shyves disease, and I'll tell you about that a little bit more in a minute. So we have several studies that show serro prevalence rates in the community, between 1% and 4%. The one that we have been part of was a Johns Hopkins study that looked at the serum prevalence in the metropolitan Washington DC area. And because we have little Bolivia here in Northern Virginia. And because those Bolivian immigrants come from areas where there's very, very high rates of Chagas disease, we have the sheer prevalence in our Bolivian immigrant community was about 20%. So to know the exact rates in your community is a little bit tricky. Moving on, in terms of prevalence of Chagas cardiomyopathy, we are a cardiology grand rounds right now. What we do know is that 30% of infected individuals will develop cardiac disease about 10 to 30 years after infection. So looking at the prevalence in the communities that we have here in the US, in cardiac populations in Los Angeles County in the at the Olive View Medical Center, Dr. Shiba Maandi and her group looked at folks who had A bundle branch blocks on their EKGs if they came from an endemic country, Latin born Latin American born immigrants, they found 5% of them had Chagas disease as the cause of the bundle branch block. If they had bivesicular block, 19% of those folks had Chagas disease. If they looked at folks with pacemakers, not the fibrillators, and found that around 8% of them had Chagas disease as the cause of their conduction system disease. And then there are a variety of different papers from around the US looking at prevalence. In heart failure populations, so the first one with the 13% was done at Elmhurst Hospital in Queens. And looking at Latin American born immigrants with EFs less than 45%, 13% of them had Chagas disease. Then going back to California to the Olive View Medical Center looking at Latin American born individuals with EFs less than 40%, it was around 19%. The 25% actually comes from our study at Washington Hospital Center in downtown DC. We looked at individuals who were admitted to the hospital with a with a presenting complaint that might suggest Chaga's cardiomyopathy. And had really no no EF cut off whatsoever. Looking at any at all comers, we found 16% of folks had Chagas disease, but looking at EFs, less than 50%, 25% of them had Chagas disease. So wide varieties, but still a very significant portion. And you know, as you think about the patients that you're admitting to your hospital who are Spanish speakers with the kinds of presenting complaints, how many of those folks have you been thinking about Chavez's cardiomyopathy? OK, so we've talked a little bit about some of the clinical features, but we're going to do a deeper dive into them to make the comparison between Chagas and sarcoid. So both of these diseases affect the conduction system and are profoundly arrhythmogenic. So what we know is that there's right bundle branch block for these patients, heart block and BT and I think a lot. Folks really know that if they have a young person coming in with heart block or VT, that they're going to start thinking about sarcoid cardiomyopathy. I would also encourage you to think about Chavez. We know that these are scarifying cardiomyopathies with regional wall motion abnormalities, and the echo findings can range from normal to severe impairment of ventricular function. So we'll start with the EKG basic tool. One of the reasons that I like to start with the EKG for Chaga's disease is because the abnormal EKG is the main diagnostic criteria for chronic fates. So I told you that folks get infected in childhood. In general, after their acute illness, which tends to go unrecognized, they go into what we call the indeterminate phase of disease. That means that they have the parasite in them, but they are not damaged by that. About again about 15 to 30 years. Later they will present with cardiac complaints. The way that we say that someone actually has Chagas cardiomyopathy rather than indeterminate face Chagas disease is based in general on an abnormal EKG. What are we looking for? Conduction system disease, sinus node dysfunction, PR, PR prolongation, heart block. Bbivesicular block is actually the poster child EKG findings for folks with Schagu's disease and PVCs because a highly arithmogenic illness. To this point, this is a meta-analysis of EKG findings. This is about 49 studies aggregated with 32,000 patients. A third of them had Chaga's disease, and what you can see are left anterior vesicular block, PVCs, first block, afib, or flutter, bivesicular block, complete right button branch block, as being the things that really shook out as being the most highly associated. I do want to mention though that just because your patient doesn't have one of these things does not mean that they don't have Chaga's cardiomyopathy, and to that end I'll show you EKG differences in cardiomyopathy patients with Chagas disease. This is from a Brazilian heart failure clinic where they looked at folks who had Chaga's cardiomyopathy and folks who had other non-schemic cardiomyopathies. The Chagas folks, 50% of them had bifesicular block. Of the other non-naschemics, only 5% had bifesicular block. However, 10% of the Schaga's patients had left bundle branch blocks, so it's not that a left bundle tells you you don't have Chagas disease, it's just not the sort of hallmark presentation, and 50% of. Folks with other non-naschemics had left bundle. This is a different data set from the paradigm atmosphere Heart failure data data set, which included some sites in Latin America. So again, we have Chagas, other non-naschemic and ischemic. And while the numbers are slightly different, it's basically telling the same story. 24% of the folks with Chagas had right funtal branch block, only only. And only 12% of the shots patients had left bundle, whereas these numbers are reversed for other non-nachemic and ischemic. All right, well, what about sarcoid? So we have to give it its due. clinical manifestations of cardiac sarcoidosis, what you see here is arrhythmias AV block, 26% to 62%. Of course this is a very broad range, but certainly it's, it's, these are high numbers. bone or branch block between 12 and 61, SVT VT between 2 and 42, sudden cardiac death and some serious extremely high. Uh congestive heart failure and pericardial disease. So if we look at an EKG set also with sarcoid and other dilated cardiomyopathies, we see a very similar thing to what we saw with the Chagas patients right bundle branch block. We see 57 here for the, for the sarcoid and only 17 for the IDC left bundle, really only in the IDC group. Here we have 3rd degree heart block and and interestingly less atrial fibrillation, not really sure why, and more sustained VT. So similar kinds of things here with the sarcoid EKG. As a matter of fact, I remember when I first presented a case of Schaga's disease when I was in Othello and I presented the biovesicular block and asked one of the senior cardiologists what they thought. The first thought was sarcoid. OK, um, sorry, I should have given you this bigger panel here to look at. OK, so what is the role of EKG and sarcoid? It's not diagnostic of cardiac sarcoids since right heart string can cause EKG abnormalities, right? So we know that people can have pulmonary sarcoid, that can be obviously extremely common in terms of sarcoid presentations can cause. Pulmonary hypertension can cause right heart strain. So it's not necessarily that you have sarcoid infiltration of the myocardium causing it. EKG abnormalities, however, are associated with cardiac involvement in that 13% of folks with abnormal EKGs had cardiac sarcoid in one series and only 0.1% of those with a normal EKG. So what might we do next? Echo and Chagas is really important. We actually consider it to be something that should be done with every patient when they're first diagnosed because a normal EKG does not necessarily exclude cardiac disease. Um, interestingly enough, sometimes that's because you can have a normal EKG one day and an abnormal EKG the next, and I see that particularly with rate related findings and sometimes actually I'll figure out that the patient is brayrhythmic when I move from the EKG to the echo, which I do myself in my clinic, and I find that Patient with a heart rate of 55, which we might consider to be normal, will all of a sudden have a heart rate of 42 while I'm doing the echo imaging in my nice dark room. So, uh, and then they're rate related bundle branch blocks and things like that. So, um, so we do generally ask folks to do an echo as part of their initial evaluation. In general, it's going to be normal in somebody with a normal a normal EKG, but not always. Sometimes there will be regional wall motion abnormalities that can show up with normal EKGs. What we do see them with their regional wall motion abnormalities is they tend to like to be in the postero basal region, the basal inferior wall, and then the apical aneurysm, which I already showed you on the histopathology, and that can sometimes have thrombus in it. End stage disease is a dilated cardiomyopathy. And this is really where your imager can be helpful to you because often folks, if they see a big heart, they, you know, they might just say, you know, they might just say it's there's, it's globally decreased and in fact it may be globally decreased, but sometimes they're scar, and knowing that their scar actually really can be helpful in terms of diagnosis. I mean, first of all, you're thinking about coronary artery disease which you always have to think of with scar. But then you're thinking about the scarifying cardiomyopathies, of which there are several, but the ones that we're talking about today are Chagas and sarcoid. So I would encourage the imagers actually to pay attention to those regional scars and to mention them even in. Patient with a big baggy heart, uh, because they can be helpful in terms of diagnosis and also to think about the diagnosis, you know, sometimes I even write in my Apple reports in the absence of coronary disease, sarcoid or or chais would be, um, in the diag in the differential. Um, OK, in terms of the echo findings of sarcoid, so you can have normal, and then we have major criteria which is basal septal thinning. I did mention to you that there are a lot of wall motion motion focal scars in Chaga's disease. The basal septal thinning is not one of them. So one really main way in which you could Potentially differentiate between these two diseases. Let's say you have a patient with a right bundle branch block and and and scarring, is that that basal septal thinning from sarcoid is really pretty, pretty specific for sarcoid unless you know somebody has had like an alcohol septal ablation or they've had coronary artery disease and a very focal. A basal septal infarct, that's pretty uncommon in terms of minor criteria though, it starts to sound pretty similar. We have intralateral akinesis and aneurysm. We have a kinesis and aneurysm, all this, this is less common and dilated cardiomyopathy. I did put a little arrow here as a shout out for the hypertrophic cardiomyopathy presentation for sarcoid. That's really not something that you would see in Chagas disease, but I think it's always worth keeping in mind for folks who are in the imaging seat in terms of how Um, how sarcoid might, might, uh, present, but these are really pretty similar in a lot of ways. Um, does a normal echo mean that you don't have, uh, either cardiac sarcoid or cardiac shaga's disease? No, uh, it doesn't mean that because you still could have inflammation or scarring that is sort of, um, subclinical enough that it doesn't actually cause overt manifestations on the echocardiogram. We do use strain to look for this sort of subclinical myocardial processes, and then we'll talk about advanced imaging after we go through strain, but I did just want to show you this really nice set of strain images here. This is a patient with sarcoid. You can see that they're pointing directly towards the basal. Basal improlateral wall and you can see the myocardial uptake here on PET, and then you can see the strain abnormality here. Here for Schaga's disease. It's also this we don't have the PET imaging along with it, but you do see that the basal scarring here and a nice little divot here on the posterior wall. So we do think that strain can be helpful in terms of identifying a sort of subclinical disease. Um, I'm just gonna, because I'm an imager, I'm gonna show you some pros because they're always in the picture is worth 1000 words. Um, oh, I hope I'm going to. OK, so we, we're going to just show some of the regional wall motion abnormalities from sarcoid, um, to start off. And what you can see here, this is a this is a heart that's pretty badly impacted. You can see sort of basal infralateral disease. And then this is that sarcoid septum. That I was talking about again, not something you see in Chagas, and it'd be pretty tricky to come up with a coronary artery distribution that would match this unless you just happen to have an isolated for septal perforator in FARC. So when you see this, this is kind of shouting circling. Sarcoid apex, I've had people say to me that sarcoid does not affect the apex. This is a patient I know to have sarcoid. It was a patient of mine, and you can see that the apex is bulging in the wrong direction. You can also see their nice defibrillator lead. Now this is something that we don't see as much from Chaga's disease, but clearly another cardiac manifestation of sarcoid, as you can see, this patient has a giant right heart. This is the moderator band. The septum is being squished into the LV, so this is a patient who's got bad pulmonary hypertension, so bad that they have pericardial effusion associated with it. And a similar patient with consequent bad tricuspi regurgitation and pericardial fluid. Looking at Chagas, this is a patient that came into the hospital after experiencing having really no antecedent symptoms, lifted up a bag of potatoes, and had suffered cardiac arrest. Fortunately, he was defibrillated in the field, brought into the hospital, and this was his first echo in the hospital after his VF arrest. Um and about 9 days later he had this follow-up echo, which I'm really sorry for the quality, but you can see that his ejection fraction really seems to have improved modestly, but there's still abnormality here in the basal infraral lateral wall. His EF on MRI was about 50%, and this is a patient who had Schai's disease as the cause of his of his cardiac arrest, got a defibrillator, and went home. This is a patient who was admitted to the hospital with heart failure and lo and behold was found to have this giant thrombus at her apex. You can actually see that the apex is dyskinetic beyond the thrombus, so she does in fact have a little bit of an apical aneurysm. Obviously her LV ejection fraction is not normal. Um, and she, you can see also all sorts of ventricularectomy down here, and she had shot is the cause of her cardiomyopathy, but you are seeing that regional wall motion abnormality. You might be tempted to call this globally down, which it is, but also paying special attention to the apex. Sorry about my dogs barking in the background, um, and this is a really sad and intriguing case for me. This is a young man, um, who came, presented to medical care, uh, because he had palpitations. Um, he was about 26 at the time of this echo. He had. A vesicular black one is EKG and some PVCs. So we underwent this echo, and what I will tell you is that we all looked at this and thought it which really looks pretty good. But you know, now with my years of scrutinizing, looking for apexes of Chagas disease in retrospect, I would say this is actually mildly foreshortened. So because of his EKG abnormalities, he also went on to have a cardiac MRI at that point, which showed that he had a very abnormal finding and then was lost to follow up and unfortunately 2 years later presented with a massive right middle cerebral artery stroke because of the abnormal MRI finding, he had again had an echocardiogram this time with contrast, and you can see that he has this focal apical aneurysm here. It was present at the time of the prior echo, and we missed it because we didn't recognize the foreshortening. We didn't realize the importance of the foreshortening. And because of this now, I generally recommend that people if they know that their patient has Schaga's disease, even if it looks like their LV function looks good, to give diffinity contrast to be able to clarify what's going on with the apex. Um, I view this as being fairly similar to folks who have mid-cal or apical HCM where we know that we will miss the aneurysm if we don't look for it extremely carefully and in general, we use contrast to try and clarify that particular situation. So I will also say that along the way I've become very friendly with alternative. Imaging modalities to look at the apex. So if I see a patient and I'm not sure that we've seen the apex well even on echo, knowing that we've tried to look very hard for it, I will go to look at CT scans, and I found over the years that an abdominal pelvic CT often gets the apex of the heart, and sometimes I can see the aneurysm there or I can see thrombus there that was not recognized previously. But at the end of the day, if you care about whether or not there's an aneurysm, that will be a management decision for you, then it's worth actually going the extra step and getting additional imaging if you don't think your echo has given you the answer, particularly in a patient who presents with stroke and you're wondering whether or not they have a cardio andbolic phenomenon. If that patient has Chagas disease, you really do need to exclude the apex as a possible location. OK, ischemic MR, this is a patient who you can see that it's not a great heart, but the septum's moving much better than the basal infrarolateral wall, and as a result, there's an ischemic physiology for mitral regurgitation and a lot of MR we've had patients actually at Washington Hospital Center who've gotten microclips to treat ischemic MR or an MR of unknown etiology, only to find out years later that they had shot's disease as the cause. And this is also something that you'll see in sarcoid, right? We've acknowledged that sarcoid has this basal infralateral finding. Anything can cause, anything that's going to cause the posterior leaflet not to move well is something that can set you up for bad mitral regurgitation. And then this finally is that bad heart where you do see that the septum's doing better than the inferolateral wall. Again, we have that restricted, uh, posterior leaflet. OK. So those are lots of echo findings which I think are a fun way of showing some of the pathology, but I did want to move along to talk a little bit about cardiac MRI because we know that this is incredibly important, particularly we know very well about sarcoid in terms of of locating the scar and talking a little bit about wrist stratification. But the same is also true for Schaga's disease, and the findings can be really similar. Now I will say we have to acknowledge that the septum is a fairly classic finding for for cardiac sarcoid and is not true for chaga. So I think if you're trying to differentiate between these two disease processes, a thumb on the scale for sarcoid is going to be septal involvement in the way that we. From that first echo, but otherwise we have basilateral disease, basal inrolateral, lateral inferior. Apical disease is more common in Chagas, but it's not unheard of in sarcoid. We do see subepicardial disease in sarcoid. That is also true in Chaga. We more RV pathology in sarcoid. We know that ECV and T1 mapping can be actually very helpful. And we know that there's a risk ratification for ventricular tachycardia, which is pretty well established in sarcoid and has less robust data behind it for shagas, but certainly there. And then this is a paper that shows the distribution and localization of myocardial fibrosis and different cardiomyopathies if you want to review, and I think this is really a very, it's really comprehensive, and I don't want to go through the whole thing, but it also talks about the location of the of the the. Of the initial injury and the progression of myocardial fibrosis, you'll see sarcoid and Chagas disease are right here in the same panel. Um, so just to to talk a little bit about CMR or stratification of sarcoid, um, this is one of many papers, uh, but I think it's uh just to talk a little bit about the fact that in, in sarcoid you have pathology, frequent LGE, which definitely includes the, it includes the location of both in terms of the walls that are affected. But also where um um where in the heart of versus like subeddeocardial versus epicardial, there's pathology, rare LGE, no LGE abnormal EF, no LGE normal EF and what we see is the pathology frequent LGE is a really good risk stratifier for a rhythmic endpoint. Um, and that a lot, it's also a good stratifier for heart failure end point, but having, having pathology rare LGE and an abnormal EF are also bad. So you can find lots of these kinds of things, but it really does suggest a good role for CMRI in terms of breast stratification. In terms of Chagas disease, this is one of a couple of a couple of papers, and what we do know is that the magnitude of scar in LGE definitely is predicted both of heart failure and arrhythmigenic risk. Um, so to turn away from that a little bit towards PET, um, the role of PET and sarcoid, I think, as everybody knows, is incredibly important, important because it shows the active disease. We know that this is an inflammatory process and you. See this is a really nice example of one of these cases, and this, but as it happens, the same thing happens to be true for Schaga's disease, although probably not quite as robustly, but we do see patients who are undergoing, as I mentioned earlier, the workup for sarcoid where they actually have inflammation on their PET scan, and then at some point somebody realizes that the patient is speaking to them in Spanish and that they ought to test. Schaga's disease. So a positive PET scan does not tell you that you have sarcoid. You can have Schaga's disease as well. Do you have to have a positive PET scan for both of these diseases? No, because as we know that as the disease sort of burns out or goes through its sort of becomes more fibrotic and less inflammatory, you can see scar in the absence of pet uptake. The magnitude of data that we have about Schare's disease and. That is really quite small, and some of that is because these, it's just really, as you know, it's really expensive to get these studies, and Chagas disease is a neglected tropical disease and there's not a lot of money in it to be able to do these kinds of assessments. But I think this is really a fascinating thing to think about. We do see active disease in folks who have very bad ejection fractions, as was the case for this particular patient. Um, so moving along here, I just wanted to put this, just to try to continue to hammer the point home. The diagnostic criteria for sarcoid, uh, we have from the, from Japan, um, they have a clinical a histologic diagnosis group, which obviously if you buy up somebody and you find evidence of of non of sarcoid outside of the heart or or cardiac sarcoid. You're going to know you have that, but the clinical diagnosis group, we have major criteria, and I put little red Cs next to things that you would also see in Chagas disease. So high degree AD block or fatal VTVF, basal thinning of the ventricular septum. We know we don't have that so much with Chagas, but abnormal ventricular wall abnormality for sure, LV contractile dysfunction, CMR reveals LGE of the myocardium. And the final thing is that for sarcoid, coronary artery disease and other inflammatory myocardial diseases are ruled out. Now I don't think that that people are thinking very much that one of the other inflammatory myocardial diseases that should be ruled out is Chaga's disease. So I'd like to encourage you to include that in. In your in your pantheon because Chagas disease really can do a lot of these kinds of things. Why does it matter? Well, we'll talk a little bit about treatment for both of these. So one huge thing is that treatment for cardiac sarcoid, as you know, can include significant immunosuppressive medications. And that can be a problem in Schaga's disease because Schaga's disease is an infection, and we know that some, to some degree this level of immunosuppression can lead to reactivation of acute disease. Why else would you want to know that your patient has sarcoid rather than not than some other, some other myocardial disease process, because we know about the intense arithmogenicity of these particular particular diseases. So we know that if a patient Um, has heart block and they need a pacer, we'd better make sure that they don't also need a defibrillator lead at the same time. If the patient's having BT, we know that these patients with sarcoid have epicardial BT circuits, so we need to be ready to do that. We also know about things like BT storm. And finally, we need to be prepared for the advanced heart failure also knowing that if somebody is steroid or immunosuppressive and responsive, that these are particularly bad diseases. So I wanted to just to quickly show you the verbiage in the AHHRS 2017 to 2017 guidelines about arrhythmia in sarcoid. So we know that there's a two-way recommendation for folks with cardiac sarcoid and LVEF greater than 35 who have syncope, myocardial scar, and MRI, or FDG PET, an indication for permanent pacing or inducible sustained VT EP study to a recommendation for a defibrillator. So this is really important because we're looking at this group of patients who have EF less than 35. Why is that important? Because if they're, if, I mean greater than 35. It's less than 35, we kind of already know what we're going to do. But if it's greater than 35, we have to figure out who we're going to risk stratify. So syncope or myocardial scar and MRI or PET or an indication for permanent patient or inducible sustained BT. So they're really suggesting here that we need to work up these patients really, really aggressively. So we're going to turn now to sarcoid. So the, I mean, turn down to Chagas. So the first question, just checking my time here, the first question that people ask me when they diagnose somebody with Chaga's cardiomyopathy is, is there a role for antiparasitic therapy? And it's a really good question. We know that folks who have recently been infected, who are children with the disease, we can, we believe that we can cure them with anti-parasitic therapy. We do know that if we have a woman of childbearing age, that if we give them anti-parasitic therapy, we're going to reduce the risk of maternal fetal transmission. We have a huge evidence-free zone about whether or not we reduce cardiac endpoints in folks who have indeterminant phase disease who are adults. But in folks who have chagas. Myopathy, we have a randomized controlled trial and it's called the Benefit trial and it was published in 2015 in the New England Journal. It's about 3000 patients who were randomized to benzinazole versus placebo. These folks to get into the trial had to have at least two EKG abnormalities that were consistent with Schaga's disease, that being Schaga's cardiomyopathy. That being said, most of them had normal EFs. The mean EF for the entire trial was 54%, but in an Eco sub study it was actually higher. Um and these folks generally had class 1 disease, so they were really, they did havechaga cardiomyopathy, but they were not particularly sick. There was a small group of folks with stage 3 disease, meaning they actually had clinical heart failure, but really not a study of folks with advanced stage D. Heart failure. What we see over time is that there was no benefit in terms of hard cardiac endpoints, and they actually had robust 5 and 7 year follow up. And these were folks whose average age was 55, which is a little bit problematic because we've been talking about how these folks can present considerably younger than that. What we did see was that at the same time these folks had really robust PCR negativization for infection. So what it's led us to say is that we don't have data. We have a negative trial saying that if you have a middle aged patient with Chaga's cardiomyopathy, they do not seem to benefit in terms of cardiac endpoints with fairly mild cardiac disease. So if I see a young person, say a 30-year-old with an abnormal EKG, I'm going to struggle with whether or not they're adequately represented by this trial, and I often will treat them just to see nothing ventured, nothing gained. Why wouldn't we do it in everybody anyway? Well, because these medications, benzidazole and nifurtamox have really bad side effects. They're very complicated to use, so there is reason not to use it unless we know that there's a clinical benefit. All right, so let's say we've decided we're not going to use antiparasitics in our patient, then we're going to use GDMT, understanding that these medications have not been robustly tested in patients with Chaga's disease, with now the exception of Entresto, which in the recent parachute heart failure trial was presented at ESC. And it's quite controversial, I would say, because the benefit that was shown in Parachute was a reduction in BNP as opposed to any clinical endpoint, and I will leave it to Scott Solomon and the rest of the heart failure world to weigh in on whether or not you think that's important enough of an endpoint to decide to use Entresto in these patients who generally can't afford much in the way of medications. Um, in terms of the arithmogenic issues, we have the same kinds of things with sarcoid. We have folks who need these pacers, defibrillators, and EP studies. The red star there is that I want to remind you, I don't know that I did this well enough early on, that Chaga's folks are at risk for very bad arrhythmia at EFs that we really wouldn't worry about, sort of same as sarcoid. So we need to, we need to be aggressive about trying to risk stratify them. And the other thing for EP is that they also have these epicardial circuits like sarcoid, and so they're, you know, folks who do a lot of ablation in the Chavis world will often actually start with epicardial VT ablations because the circuits tend to be there. The thing about Chagas disease that seems to be quite different from sarcoid is the issue of anticoagulation, and I already showed you a patient who's had Masses to patients actually with apical disease processes that would that would cause them to have strokes. So how do we decide who might be primary prevention anticoagulation in Chagas? And this is unfortunately virtually an evidence-free zone. We have one large trial from Brazil that was observational where they came up with a scoring system kind of like Chad's vast. They call it IPEC. It's never been externally. Validated, so it's really difficult to hang your hat on it, but it really is all we have. What we know is that folks who are over the age of 48, who have reduced LV function, who have apical aneurysms, and who have abnormal EKGs, repolarization abnormalities, they called it, are at if you have a point score of 4 or more, you're at high enough risk for a thrombolymolic event that it probably that the primary prevention probably outweighs the risk of an coagulation. So that is why I really care a lot about the apex and why I'm pretty aggressive at looking for it, because if somebody has an abnormal EF if their age is over 48 and they have an apical aneurysm, I'm going to try to have a shared decision making conversation with them about primary prevention and a coagulation. And then finally moving into the advanced heart failure realm, the one thing that is really very different from sarcoid is that we have to think about transplant reactivation, and patients do very well actually with Chagas disease transplants. They tend to be young, they tend to have less comorbid disease, but they do also tend to reactivate in the CDC series in the United States, 60% of the patients, I think that the CDC followed, and they really followed all of them. I had evidence of reactivation. Why would I say evidence of reactivation? Because in the United States we do not wait for clinical reactivation to decide that somebody has recurrent paraitenia. That's important. We do PCR starting in week one after transplant, and now patients can have abnormal PCRs with chronic Schaga's disease, so we don't say one PCR being positive is reactivation. What we look is from one week to the next to see the cycle threshold of the PCR to go down, which means that it takes less cycles to show evidence of parasite DNA, at which point we will institute antiparasitic therapy, and patients tend to do very well. OK, so this feels like it's stepping backwards, but I did want to show you that the American Heart Association HRS 2017, in terms of Chaga's recommendations, there is a big nothing. So this is really a problem for me because we know these Chaga patients have very similar risks to the sarcoid patients. Sarcoid has very specific language about who you really need to be worried about with EX above 35. Chaga does not, which means that I spend a lot of time trying to compare the diseases when I'm talking to my EP folks. About when they need to be more aggressive with the patient with Chagas disease who has an EF over 35, and it would be great for us to fill this blank in, I think at some point. OK, so I'm getting to the end here, and I thought I might actually answer this question for you. Do we even have any Chagas in Virginia Beach anyway? And so what I wanted to show you was a map here of your area and the percent of population that is Hispanic or Latino, and what you see, and you, I'm sure know this way better than I do, that you have areas in your catchment area, I'm assuming where there are a lot of folks who are of immigrants from Latin America. And this is a list by locality, but more importantly, we have the country of origin for these folks, and Chagas disease is found in any Latin American country that is not an island. So we start off with the Mexican population and we see there's a lot of Mexicans living in your area. Puerto Rico, you don't need to worry about. Central America, you sure do. Other Hispanic or Latino for sure, South American for. Sure, not Cuban, not Spanish, not Dominican Republic, but basically we know that you have a very significant group of people living in your area who are at risk for Chagas disease. When should you suspect the diagnosis? When a patient is known to have the disease, Chaga's disease is sent for ECO to evaluate for cardiac involvement. If you see right bubble branch block on EKG, heart block, or VT, and when you're reading an echo of a patient who the. Diagnosis has not been made and noticed findings suggestive of the disease, and that actually is the way that it sort of panned out at Washington Hospital Center is over the years that I worked there for, you know, the better part of a decade, sitting in the echo lab, I would yakk at everybody about it and finally the echo folks and the MRI folks would actually start to incorporate it into their reads so that it would signal to the team that they ought to actually look for the disease. This is specific for Ashagas. In terms of take home points, Chagas and sarcoid have many overlapping manifestations. EKG abnormalities, Brady and tachy arrhythmias, and wall motion abnormalities, both are more common than we think, and making the diagnosis matters in terms of the aggressiveness for replacement of AICDs, treatment ramifications, and prognosis. And with that, I will stop sharing and hopefully entertain some questions and thank you for letting me yakk at you for so long. Yes. Thank you so. Published October 13, 2025 Created by Related Presenters Rachel Marcus, MD National Institute of Health
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