Chapters Transcript Video Cardiomyopathy and Cardio-Oncology Symposium: Case Presentations and Panel Discussion - Part 1 Faculty review real-life cases from their practice and answer questions from course participants (session 1 of 2). Back to Symposium Page » Okay, so I'm gonna move on to cases, and hopefully you can have a big discussion. Make this interactive. Okay, So the first case is a 51 year old male? No, prior cardiac history. Average cyclist. A lot of mountain biking recently, you know, had a bike crash plan, chest trauma and that sort off, you know, let do. He came in with those symptoms, and because of the blunt chest trauma, shortness of breath, chest pain, he gets an echocardiogram. Um, which, surprisingly, to the surprise of the ordering physician, actually reveals an ejection fraction of 15 20%. No other associate id, valve abnormalities. And purely the echo was done actually more along the lines of looking for pericardial effusion. And, you know, he had some chest pain, most likely the trauma, But this was sort of through everyone by surprise. His e k g uh, shows, you know, pretty unremarkable. For the most part, you know, you have this incomplete sort of right bundle, um, on, but otherwise the voltages are not striking. Maybe borderline left ventricular hypertrophy. Um, but that was about it on this e k g. And here's what is Echo said so if it's 20% moderately dilated. Left ventricle, maybe some left ventricular hypertrophy increased ridiculous ation at the apex. No troubles. Left atrium is severely dilated. No significant valve issues. Uh, and so then he gets an ischemic work up purely unrelated. But they found the CF, and they're like, OK, we have to do something. And the ischemic work up was normal. Hey, then gets an arrhythmia monitor because he had some palpitations. Now this was it was just just from the er. See, the physician of an outpatient who puts them, you know, on heart failure medications for the low year lose out coronary artery disease. And then they want to figure out why. He's why the efforts, though. And he gets an arrhythmia month replaced, which shows x amount of PVCs and incidentally, picked up. It'll tribulation on DNA. Now he's placed on anti coagulant for strugglers prophylaxis for the A fit. And basically now we have a patient 50 year old, completely functional, healthy, no heart failure. Symptoms e f 20% on some PVCs. And given his age, the question is okay. Where do we go from here? A do nothing way ruled out ischemia be. Get more imaging. See, at this point, consider some genetic testing. History is mostly under grilling. No significant family history. He's never had any problems or issues prior. There is no right answer here or wrong answer. I'm curious what the panel doctor would tell a certain moment you would do at this point. Is this somebody you would go into further measures, like an M R I genetic testing or be like Okay, well, we just bought some PVC street and see how he does. Yeah, you know, I'll take the bait first. I mean, I would say eso from a causation standpoint, you know? Of course you wanna ask your standard questions? Does he have a history of drug use? Uh, which might be remote? The history of heavy alcohol use, etcetera. I want to make sure that again. He isn't somebody who got childhood chemo or something that he just forgot to mention because he was so long ago a cured of his leukemia, Assuming nothing like that, I would say this is absolutely somebody who would send for for genetic testing. You know, I would say I I would approach the variance of undetermined significance little differently than than than you did earlier. You know, they're so common. And I I am. I'm careful about reading much into into those at all. But if you see a clear path pathologic variants thing, that's gonna be your answer. Almost certainly, um, and and and could be used for predictive testing and families in terms of question about like, Are you gonna put a defibrillator in this person or not? I would say a couple things. One if his bike accident was mechanical, it would make me feel better than if he sink a pies. And that's what caused it. Um, assuming it was mechanical, I would say I wouldn't rush to put a defibrillator in because there's a reasonable chance will have a big response to medical therapy. Um, but there's a sort of patient you could debate whether to put a life vest on in the first few months for protection until you see whether the moves in the right direction or not, I wouldn't coagulate given the a fib, of course. Yeah. So we basically put on medical management thief. All history, which is crucial, was not a lot of consciousness mechanical. So we say we're intrigued with medications. Follow the arrhythmia burden and wait for your F to improve. And if you meet the criteria of 90 days, well, then obviously put a different later in. Did not prescribe a life list for him and related to the V. Us. You know, I mean, I I agree with you. I mean, it's a lot of the us is that we variant of unknown significance that we wouldn't act on. Sometimes it's challenging. Perhaps there's no right answer in the clinical functional phenotype how you view them. Uh, but in this case, eso question what next? We actually did an M r. I on him. Just to get a little bit more granular from the echocardiogram was show on E f or 20% Think gonna have a talk on this next by one in terms of how we approach memories And what sequences? The reason I did this echocardiogram was one. Okay, The echo. Should me and eff off 20%. I just want to get a little bit more detailed. Is there a massive area off scar or is there something the echo is missing that I would like to get here So the echo did reveal a fair amount of late gathering enhancement, which was the M R I, which is a bit surprising but perhaps would explain his PVCs. So we decided to medically managing, uh, when he continued to have recommend VT. His ear was holding up. But continue with this car in the info a pickle region shown here on the left image on bond involving a fair amount of the lateral wall in this patient. We then wanted to dig in mawr. Um, would you agree with this? That would tell us in terms off your thresholds in memory and someone like this? Well, you know, I think I'm sure more so than our next speaker tend to be a little bit of a nihilist in terms of with them. Or I like Thio be asking, you know, is there a specific thing that I'm looking for with it? I think you know, if there was a thought about non compaction, for example, I think there was a comment about tribulations, and you want to get a better assessment. Be very reasonable. Um, if you wanted to exclude a disease like sarcoidosis, you saw no delayed enhancement That'd be pretty good. Um, you know, be awfully weird presentation of SAARC oId frankly, but s. So I think I think one could make a reasonable case here either way, on the Emory, Um, I'm not, You know, you see a little bit of delayed enhancements on lots of people with non ischemic cardiomyopathy. These and it's more helpful if it's not there to exclude again things like Sark oId. If you see these sort of random patterns, it doesn't really tell you much other than yeah, I mean, there is some scar there. E feel the same about memory. Oftentimes people reach out and get memory to clinch a diagnosis. But usually m r. I almost never clinches the diagnosis. I'm sure one would love to argue basis with me, but I think it helps close in. And it has a good sort of negative predictive value. And I think especially it's so relevant now in these times of coed, where you know that paper that just came out showing viral myocarditis? There's Carolinian. What does it mean? Three only reason we did this Emery is not my routine practice. If somebody had PVC, I'm gonna get Marai right away. But this patient had runs off VT and very significant. You know, what is some looking EKGs which prompted three m r i s o. Then, um, here's the Marie report. So consistent with the echocardiogram, there was some areas, you know, as you pointed out, you know, tribulations. But it really wasn't consistent with non compaction. But he did have, you know, this really striking transmitter Carolinian enhancement. So do you take into account sort of the degree? I completely agree, of course, when you know, wait for med, you know, appropriate guideline directed medical therapy for a couple of months and reassess, um, to see if there is some recovery ability. But perhaps the degree of scar if there is scar the degree of scar maybe somewhat predictive of whether or not there's going to be recovery a point. I mean, So there are these big right? I mean, if you have more than X amount of 15% or something like scar involvement, it would see a much higher risk. It's not absolute absolute. And other Doctor Bill mentioned, you know, it can be a little dynamic to depending 5% scar plus minus. Could go away. If this was unknown, viral proto almost syndrome that he had. But the extent along with V. D suggested you know, something more. Um, and could it be that the just dropped because of subclinical arrhythmia, PVCs, that is happening and control the heart failure? Arrhythmia function better. Um, so this then, you know, and I think one will talk about this, but in terms off, you know, it's not just adding a cardiac came, all right. And then you see these words, mostly that looks like latin to me. I don't know what they mean, but there's a lot of sequences and protocols involved. All I understood what they did this, uh, enhancement images. I'd like to get more from him. So this fish, we actually then, you know, went ahead. And this we're to proceed with a sock protocol completely. There was nothing in the history. There was nothing in the other organ systems to suggest it. But when he came in on that er presentation, he had the c t scan looking for trauma, and there was some limits nodes involved. So it was like, Okay, well, we reaching too much. But he had this Galilean here We had limbs, notes, and he had this VT. So I'm related to this. Were like, Okay, let's look for star Coy. And I just wanted to show this case not so much that this is the right way to approach. I mean, Sarko is one of those, you know, maybe you'd wait a few more months. If you have more bt, then get it. But sort of just a least go through the thought process at times, you know, like really the suspicion for Sarkozy should be high and usually driven by the history or extra cardiac findings. And what users kids just to illustrate the image in protocol involved in circle. So he had a rubidium f d g pet protocol for star coin. And as most would know, the key, unlike a regular pet protocol, is the diet, diet, diet, diet. If that diet is wrong, not done right, the study becomes absolutely useless. Um, so curious to hear your thoughts that you were jealous of the scan. Yeah, well, I mean, I think the key with with S o F D g pet scans are simultaneously the best and the worst thing that has ever happened to the field of cardiac sarcoidosis. It's It's definitely allowed for a lot more diagnoses, but it's also definitely facilitated a ton more over diagnosis. And it's in. You know, it's because of the issue you were just getting at, which is the dietary preparation. But it's it's even if a patient meticulously follows a no carbohydrate diets. That's basically just a way of trying to push the metabolic Omnivore that the heart is towards fatty acid metabolism away from from glucose except ferries, inflammation. And everybody is going to be different, and some people you just don't quite achieve that. So what you're really looking for on the F D G pats is, I'd say two things. Number one. Do you see signs of extra cardiac uptake? Because if I don't see any signs of extra cardiac uptake, it's not that you can't have isolated cardiac sock when you certainly can. But it makes me much more suspicious of the diagnosis in the setting of a quote unquote positive pets, Um, that that it might be a false positive, and then number two is you just see sort of diffuse uptake, or do you see focal hotspots and the Mauritz focal hotspot or spots, the better you're gonna feel about it, you know, here it looks like I think if if I'm looking to this correctly, we have the profusion marker up top in the f d. G on on the bottom and, you know, it looks like from the profusion marker it looks, It looks pretty, you know, reasonably normal. There's a little bit at the kind of a pickle septal region that maybe, maybe less so, um and you can see on the head, you know, it's kind of you can see it on either the short axis or the kind of four chamber projection at the bottom. You do see this somewhat local hot spots on the lateral wall, kind of more the basal lateral wall. And, you know, I'd say this is the other useful thing is to correlate it with your other imaging. And so if you see that at the exact same place that you saw the Gatlin iam uptake on Emery, you're going to feel a little more confident about the diagnosis. And so that's you know, again, both of them have pretty good negative predictive value. If they're completely normal. Um, both of them are prone toe false positives in the sense that they could light up for something other than starkwood. When they both fight up in the same spot, then I Then I worry more that it's real. Yeah. So those are great points, Doctor. Tell us. I think I mean, starting with the diet itself. No matter how much I tell patients or how much they try, You know, the 24 hours prior Ah, high fat diet, not low carb, but no car. And then even fast for 16 hours and then really try to switch the metabolism from glucose space to fatty acids base. It's not perfect. Increases the sensitivity, but not perfect. Now, in this case, you know, the first sort of rubidium flow tracer Looks like I agree. My quite a profusion overall is normal. That does look like that. Is that f d g hot spot? Uh, which such as cell membrane, sort of disruption and perhaps information on the lateral wall which was somewhat consistent with the Marie showed. So it's sort of off, you know, increased our, uh, sort of sensitivity that this is likely to be, uh, something off pathologic significance. This just shows the rubidium on the left on the F d. G on the right and clearly showing that there is some f d g object. And then exactly as you mentioned now we have the other extra cardiac findings that went along with sarcoidosis meeting the lymph nodes lit up. Um, you know, and then when they get a pet ct contrasts of that fit. So all in all, we were able to put between the echo the Holter monitor, the pet scan and the m R I to say, Hey, listen, you have cardiac sock. You have active cardiac star boy, uh, both dropping. They'll be functioning arrhythmia. That would warrant, uh, treatment. So that's basically confirmed on. Then he gonna skip this for the sake of time, but this is what will be a little earlier. It's not one diagnosis, Emery Negative. And Marie positive. You have to put all these points together to come up with a diagnosis and keep fit. That criteria. Andi, This is where Stark gets really challenging. Is the extent off involvement you could have circled. One little granuloma you might be asymptomatic, was is it involves different areas off the myocardial and it can make you a lot more symptomatic and also convinced that block was Yeah, and this is a protocol from, you know, a paper that came out. I'm sorry. The reference is missing here, but but from Jack And do you agree with Dr with others? And in concept, if somebody has evidenced by arrhythmias or will be function, you start with some protocol along these lines. E. I actually don't agree with this, but I'll say that that you know there's enough equipoise in this area because there's not good dated to guide it, that reasonable people could choose different. My own protocol is that anybody who I'm starting treatment on I start up front on a steroid sparing agents. Plus, if they have active disease, of course, steroids, with the goal of ultimately trying to wean them off the steroids and leave them indefinitely on the steroid sparing agent usually met the trucks. Eight, um uh, indefinitely. And if we can get them to a point of a negative pet scan on Onley method truck safe, that's a win in my book, and you have to do the basic laboratory monitoring. But methotrexate has a very good safety record. Uh, the other thing I'll say is that if methotrexate is not enough or the steroids aren't being very effective, TNF inhibitors are really quite quite good for cardiac. Sarcoidosis would typically be our next go to Yeah, so that's a great point. I mean, this is how actually my practice started with early on using more steroids were working with the rheumatologist on dermatology colleagues is something that I've learned is more upfront use of methotrexate. And, you know, usually I used to start prednisone, wait for a couple of months and then think about the Brexit because of tolerance and side effects. But more and mawr rheumatology call the police are doing exactly the same from the get go. Adding prednisone plus Mr Brexit than reading the president's own off and continue to Brexit if they tolerated. And which is exactly what this patient actually ended up. Um, doing and in terms of different later, is usually driven by the arrhythmia history. If you can't suppress the arrhythmia, they usually end up getting and i cdr. Every function doesn't improve. They end up getting dicey. So this patient, you know, God on prednisone. Methotrexate. He's doing better now. There is a burden has decreased significantly. He did end up needing an E P study on Bt ablation in the next car focused. So, along with the e P colleagues, rheumatology, heart failure and some of the images expertise, he's doing a lot better. I mean, still young 50 something. Goal is not transplant at this point, but to preserve and conserve how he does. So he's coming along nicely. And I just want to illustrate this case to show you how damaging he plays the white key role and how a Africa specialist can work with a rheumatologist in taking care of these patients. Thank you. I think we will take a quick break and then come back for the next session of talks. Thank you. Published February 2, 2021 Created by
