Chapters Transcript Video Cardiomyopathy and Cardio-Oncology Symposium: Case Presentations and Panel Discussion - Part 2 Faculty review real-life cases from their practice and answer questions from course participants (session 2 of 2). Back to Symposium Page » So here is an interesting case. 28 year old male, no past medical history. His father was recently diagnosed with hereditary 80 tr cardiomyopathy complicated by heart failure. Um, he the patient does not have any cardiac symptoms. He has some non specific G I symptoms constipation and diarrhea at times, and a couple of episodes of what he described as maybe felt like incomplete bladder emptying. So his father symptoms included in a peripheral neuropathy spinal stenosis, and is now diagnosed. He came to the diagnosis of amyloid because of a cardiomyopathy, and he's currently on stabilizer for treatment for his cardiomyopathy. So the genetic testing for both his father and son show that they both carry a mutation, Um, s associate, you know, in the t t r Jean a 65 team. So here is the the R 28 year old. Here is his e k g. Andi. Here's his echo. He actually had already had an m r i prior to me seeing him. Um, but for all intents, purposes essentially normal eco normal looking strain in relatively normal looking memory as well. So his question to me and my question to you is sort of you know what's going to be our surveillance strategy for him, you know, as part of his G I issues. His primary did send him Thio g doc, who ended up doing an e g d in culo. We asked them to stay in for amalie, just thio if they were doing Bob sees anyway, where they did and those were neck. So you know questions to the panel. How should we? How should we surveil these patients that are asymptomatic carriers where we know that other family members have manifested over cardiac disease in the future? Serial echoes memories periodically, biomarkers. What do you guys think? So I I wouldn't start with a really good neurological exam by an experienced neurologist. He's probably based on the mutation in the history. Most likely the first present have a treatment indication from neurologic signs, and he obviously has some symptoms that could be concerning is pretty young, but we could be concerning for early neurologic involved. The moment you proved that he has a treatment indication with evidence of a either purple or autonomic neuropathy, you could start him on treatment. You're basically looking for a reason to start him on treatment. Um, you know, I would say, Thea, other thing is that I would personally start him upfront on off label Di Luna. Saul as something that chief. Probably pretty harmless. And though we don't know for sure, of course, likely Would would decrease the likelihood or at least delay development of of disease in terms of of screening the heart. Um, you know, I would say biomarkers are pretty easy. Straightforward thing to dio What's the I would certainly do screening if you didn't already have a treatment indication. And what's the right imaging? Interested to hear what wants, What want steak would be, um you know, I think probably Emery would be the most sensitive thing, How frequently you do it You have to debate, but that probably we might take a curator. Uh, on. I fully agree with what runs says. You know, this is basically an uncharted territory because we're not usually when we're thinking about this disease, we're not just, you know, screening for development off cardiomyopathy in a young person, you know? But certainly, you know, it will be indicated toe, you know, do CEO imaging, you know, because we know that you know, at some point we're going to get there, so on. I agree. Carry camera. I will be more sensitive and specific. Probably This is what we want in a patient that were kind of waiting for some sort off manifestation. Because the echo changes in my you know, I believe that they happened a little later, and and I think that, you know earlier probably will be better. And memory, I completely agree, will be a great option. Now, you know, whether it is, you know, yearly. Perhaps. You know, I mean, the good thing is that Emery, you don't have the concern of radiation. But you certainly with gallium agents, you know, there's a a concern about accumulation in certain part of the brain. So, you know, I I think that I will probably be a little more flexible, and I will use Gala linea. Um, the micros I click contrast and not the linear near are the older, and they tend to accumulate more. So I will do that. And probably every either every one or two years, something like that. I mean, I think we can be flexible in this scenario, but but I agree that we have to use the more sensitive and specific technique one. Is there a minimum threshold? Aceto before And what is the earliest sign on Marie, if there is any that before you have the full blown disease, you know, where do you cross that? So So again, You know, You know, I don't think in this disease process, you know, I don't think we're these are important questions. Because as we find more cases and we find, you know, earlier, you know, situations especially, you know, in in genetic, you know, more related toe genetic problems, you know? So I think that the one you know, t one values they tend tohave. You know, you can detect earlier. There are a little more sensitive, and they don't require the use of contrast. But again, this will be completely uncharted territory. So, you know, it will be more like a, you know, expert opinion. Take, you know, not really enough data to say what will be the best way off, you know, really following a patient like this, but I will think that you could even consider doing a, you know, non contrast, uh, memory. Looking at Taiwan values And if there's a significant jump, you know, or what I am normality, you know, I think that that will be You will have to then the full study. But I think you know and again is the problem with the Taiwan values is that they're not completely standardized, meaning that the, you know, the vendor that has the most robust value on consistent measurements will be seaman's. So if I if I were and not that I receive anything from Siemens, but they really have this, you know, technique, pretty consistent. So So I think that it will have to and again, whatever I say, it's not gonna be right, you know, like someone else can have. Ah, different opinion. But But I think that certainly, you know, to me, it will be ah, lot of sense toe use m r I, you know, toe to really identify any progressing situation. But but yeah, I mean, this is some completely uncharted territory for sure. The other key is that you're really looking for when, if and when you're gonna biopsy. Because, you know, a subtle sign on memory is not gonna be enough to be able to start treatment you know, So I wouldn't biopsy now based on the imaging that we saw on. But it's really if there is not another treatment indication, when does it across that threshold we're gonna wanna biopsy, which is probably the most sensitive, any any sort of long term registry type data or centers looking at these type of patients and including them in anything like this year or well, I mean, I think you know the problem here is, uh, teach your aunt. It's teacher Emily, Though it's been known now for a small handful of decades, it's really only been in the last 10 20 years that large numbers of patients that followed. So I think probably most centers have certain patients, you know, have patients like this. I mean, there was the house registry with CRM land registry, but in terms of how maney, how many's that enriched with these sorts of patients? It's small, but I think we're going to know in another 10, 20 years, as we're following patients like this right now that we're looking form or and we're finding it more e. I think some of this problem of like even for the powerful space Can you agree? I mean, in terms off course. Now we're only imagine image in the symptomatic and really symptomatic. But as we sort off change our thresholds, right? Yeah. I mean, you know, I just had a patient very similar to this and who's, you know, the white scan was really quite negative, But there were not suspicious things that we biopsied her and she was, and she positive, you know? So I just think he wipes. Can is eyes really not made for these cases of really trying to pick up super early involvement? So taken point tissue diagnosis. It's ultimately so if in a case like this is what, uh, all right, so we'll move on to the second case. So this is a 55 year old male with no past medical history who's seen for shortness of breath. Hey, first noticed this about 3 to 4 months before his presentation. He is typically a long distance cyclists. He's done even triathlons in the past in the recent past, but for him it's getting progressively harder and harder to do his cycling. Um, he's developed some shortness of breath and even some chest discomfort in heartburn with exertion. So initially seen by his primary doc ordered a treadmill test in a G I concert. Ended up getting an e g d. And that was negative. Um, here's his e k g. Here, and, ah, some emerging from his baseline study echo study. So his treadmill was negative for ischemia. He was in seen by general cardiology. And given some abnormality seen on that echo with the L B H, he ended up getting a cardiac memory. So here are some selected images. Not the best images I know. I'm sure if I send it to you one, you probably would have picked some other images to show, but these were the ones that I kind of pick just based on looking at sort of the degree of L V H on. Been trying to see if you know what that kind of scar pattern. Have a quick question. There are. So I mean, the guy is a cyclist is young. You can expect some of the age right. Prompted getting an m r. I in this patient. What? Didn't look so sick or was there with me or something? Or was it a degree? Yeah, sure. No, of course this was done by somebody else. But from from what I could gather, it was done because of the fact that there was just the L V h itself on the on the echo. Yeah. You know, this is this is the type of case where you know, I know, you know, for hypertrophic cardiomyopathy in the past, you will, you know. And still, I think some people still recommend toe the train, you know, to see if there's resolution of the hypertrophy and all that. But, you know, with Marie, definitely You don't definitely need it to do that. I mean, if you look at the, you know, image in the middle patient has L v h. But there's significant enhancement there, and that's completely abnormal. And an athlete hard. It's not gonna look like this, but this is, you know, certainly, you know, a positive stuff, you know, because you can see that the trans near transmitter enhancement. So, uh, but, you know, in in other scenarios, if you rely on echo, you know you're gonna have toe, you know, maybe say, hey, why don't you stop doing exercise? Let's see what happens. You know, Yeah, yeah, Or you're gonna be more aggressive and do like, you know, go on, do biopsy. But in this case, I think it's clearly, you know, positive for like, yeah, and I mean, you know, a case like this where he has new symptoms, you know, pretty pretty progressive Newhart player symptoms and L b H that demands a further work up. And in terms of things that can cause that, you know, he's not obstructive. So this isn't obstructive hypertrophic cardiomyopathy on echo, I mean, and eso amyloid quickly jumped to the top of differential. So on alternative, would it be first has to send off like change group? That's what you would have been most concerned about. Certainly. Start with memories Another reasonable way. And you see this pattern? It's like, Okay, that something else is going on the panel. How useful do you find the tissue? A. You, uh you know, several of the last year, especially in terms off Realogy. Or you don't rely on that, given that, you know, with age and high blood pressure, everything confound because oftentimes you know the patient, if they actually center is easy to order. But it's different to other way and you have somebody sending three hours. There's something I need to send for them or I or something, you know. Don't find it particularly useful if from nothing. Well, I mean, I think it depends what's in your differential again. You know, if you say here if you believe that Hey, this guy is heart failure and if you're not sure that that's the symptoms, you could also do some quick bio markers that will give you a sense. But if you believe that that this still in the scheme of things fairly young guy has has new heart failure in a thick heart, it's a pretty small differential of what it could be, right? And that what you're really concerned about in this case or should be example Yeah, you know, for tissue Doppler I will say that I will look at the tissue Doppler because you know and an athlete, right? I mean, and this is, you know, assuming that the symptoms are not that clear like classic heart failure. But, you know, if you if you look a reduction in the tissue operator in an athlete that really is abnormal, that's something that should jump right ahead and say, Hey, you know, he should operates abnormal. There's something else going on, you know, this is not LV eight because of athlete. Yeah, you're an athlete in your mind. What? Rio on interestingly, you know, he is clinical presentation was such that he had already backed off quite a bit for a number of months already. By the time he was getting some of this work, I've done so he was already he already scaled back quite a bit. You know, the other important point I just want to make is that you know, l amyloidosis, which is the likely diagnosis here, should be viewed as a near emergency s. So this is not the sort of thing that when you see him clinic, it's okay to say, Well, order an m r I. And maybe it'll take six weeks to get done, and then we'll follow up on that, etcetera. You know, if you get a memory, uh, the next day or in a couple of days, that's fine. But you need to quickly exclude ale amyloidosis because that's the thing that could rapidly progressive killed. Exactly. Yeah. What? Sure. Um, so you know again. This was outside imaging that was done. So he was. He was diagnosed. His HTM started on beta blockers. Progressive symptoms, basically now unable to cycle at all. And given these progressive symptoms, he was sent to us for further evaluation. Um, this is just more of his echo from when he came again. So that's exactly what we did was the first test. And unfortunately, this these are the results that we had. So his biomarkers there on the left and his his life changed there on the right. So very much consistent with the Lambda predominance here. And high enough, that sort of fits the criteria, actually, for what looks like multiple myeloma. So he was seeing urgently by Hugh Mythology. Underwent a bone marrow biopsy. And turns out he has 30% plasma cell clone. Uh, no amyloid in the bone marrow itself. But again, like land a light chain predominant, he's got one Q 21 duplication. He did have a fat pad aspirin, which was also negative for any amyloid deposition. So diagnosed with multiple myeloma currently on Darryl and Ellen amid index A meth, his own, uh, started on that and has also received Cortez amid on DNA. Now he's having more and more heart failure symptoms. So increases in doses of Lasix a furious might to keep his volume under control. He had one hospitalization for an episode of heart failure. In fact, in the last month or so and these air sort of his most recent light chain quantification so improving. Certainly, Theune. Of course, when we first saw him. So questions here do we need I mean, this is essentially, you know, we know it's multiple myeloma, the light chain abnormality significant. We have the memory. Um, you know, we felt that there wasn't any necessarily any change in our management strategy with the tissue diagnosis of the heart for for amyloid deposition. So we opted to not do it. But any indication there for AnAnd Amar Cardio biopsy in this case in particular? No, I don't think so. I think I would have done the same view because he has a treatment indication anyway, for myeloma. You know, I'll just say that Yeah, depending on how experienced hematologist is in caring for this sort of treatment targets with myeloma. An alien with doses are different. They're gonna mawr aggressive in trying to get light teams really under control with a lot amyloidosis. And so you just want to make sure that this patient, when they're when they're given the chemotherapy and deciding winces which the maintenance and all that they're doing that from the context of a Lemley doses patient with bad cardiac involvement, not just myeloma. Um, it would have been reasonable to biopsy, but the biggest thing is, you don't wanna wait to start therapy on Ben. What are going to be, you know, therapeutic options for him for advanced heart failure. So his GF has been note dropping now. So 57 to 53 to 43. Now, we considered an implantable cardiac monitor to help manage his fluid status a little bit better. Um, but is he potentially, you know, depending on what the treatment course for his multiple myeloma is like and whether or not he gets into a state where the light change or fully controlled, will he be a heart transplant candidate in the future? If that if that comes up. Yeah. I mean, when I was seeing this case, the first thing given his age and the severity of this project and he has a lot of bad prognostic factors, including just how high the proponent Waas on s o. I would say the real key is excluding other significant organ involvement. Andi, if he does not have other significant organ involvement, I would say, based on the severity of his cardiac involvement heart failure, he should be, uh, considered for heart transplantation. E I asked again another case where I asked the pathologist to go back to his e g D. Uh, samples sustain and those were negative again. You didn't really have G I symptoms. So it's always hard to know what organ you know. You're gonna find it in. Yes. I mean, you want you want to scream for parental involvement, which you could do with Taliban area. And, of course, the GF are you want to look for a liver involvement without boss? You wanna scream for neurologic involvement mainly by history exam and then g I. It's a combination of symptoms when we're gonna do you think about heart transplant. We do pursue being cola on everybody with random biopsies because it can be patchy in the track and give some degree of the some sense of the degree of involved. But, you know, I'd say this is potentially a very good patient for heart train consideration. Describe. He's actually I think he's done a virtual visit or some sort of consultation with you already on DSO. Certainly somebody that we're gonna be following together. I think going forward run so good. Um, that was all the questions that I had for that. Are you from the audience? Um, are you noticing an increase in heart failure? Patients who have recovered from corporate say it again and increase? I couldn't hear. He said, Are you noticing an increase in quest heart failure Patients You have recovered from cobalt. Oh, from covered. I don't I don't know. You've seen that yet? I don't. You know the recovery phase. We're still in the recovery phase now. I think on dso Maybe those some of those patients that got it back in March and April maybe they'll start presenting now. I I don't think you know you and I have Certainly I don't think j ask you as well. I don't think we've seen that yet. Agreed. I mean, some of it is on the phones, right? I mean, there are the known sort of diagnosed cases and the mildly symptomatic ones who actually didn't get tested. We don't know if they have on, you know, any newly diagnosed heart failure. We're not going back and checking or we're not checking for Cupid antibodies if they did have Corbett. But broadly speaking, no, we have not seen any. So it's not video concepts or anything different around your thoughts and one, Yeah, I think this is a really good question. And it's actually a really important point now that we're, you know, however many 67 months into this. Um, So there's been all this controversy about cove in myocarditis and actually interested in what thoughts are on the memory studies. You know, my own belief on I think the preponderance of the evidence is that this is wildly overblown about the degree of true myocarditis and little areas of lead enhancement. You know, scant areas that does not equal myocarditis. Um, the fact that we haven't known and this is true and, you know, places like New York City as well that have really high Koven rates. Um, that there hasn't been this explosion of heart failure patients, I think argues pretty strongly because you're talking about a virus that has obviously had a huge prevalence on because so much suffering. Um, the fact that it hasn't really speaks Thio, that the rate of true myocarditis with this is really low. There are some. There are clearly occasional true cases of myocarditis, but that is the exception. Yeah, I completely agree with you around, you know, back before called it. If if I see some LG in someone that had chronic hypertension, I will say this is hypertensive heart disease because you can see lg. And now you know, of course, these patients at a higher risk toe have severe disease. And if you do an Emory without control, which there was a study that they didn't use controls, I mean, and that, unfortunately, gets overblown by the media. I mean, I mean certainly were not blowing off that there could be some cases like you said that you know all these Aled, these viruses can cause my car like this. I mean way have seen clinical cases of myocarditis, but that we have ah ravaging high incidence off all this. That that's really not perceptible and I think we're if there's, uh, something with teeth, uh, you know, toe this, we will see. We will have seen him already because we have so much search cases in New York and we haven't seen that. So So I think it's interesting. I think people should look into it, right. But I don't think that we should, you know, right now, be worried that we're gonna have ah, you know, a search of Margaritis because of call it because because it's not really happen. But just talk to my friends. I was just talking to one of my colleagues in New York N y u, and just at the wrong point. I mean, given the volume of cases and timing viewpoint, they start way ahead of most of us. It did not see any searched as far it's sort of points more thio, maybe, uh, at least as far it seems similar to other viruses in terms of cardiac involvement. Yeah, Dr. Christopher, you could join us here. One of our colleagues had a question about whether you guys have seen any increase in just arrhythmias. So not necessary heart failure. But, you know, increased incidence of PVCs, uh, heart, block E nothing. You know, at M d Anderson, you know that 30% of my counsels are safe, and that has still the same thing, you know, because if it is very prevalent in cancer patients, it's in, You know, it is extremely brilliant, but, you know, we haven't seen anything different from from the court page a question for our own. And Raj, Um, if it's the g I involvement, it's patchy for amyloid. Will you? Can you miss it when you're doing G biopsies? Yeah, so that's a really important point. So? So, first off, I want to be clear. Not everybody with a lamb would needs endoscopy in polo and random biopsy. This is only in the context of working somebody up for transplant generally and amyloidosis. If you biopsy the clinically involved organ, you have a really high rate of finding. So, like you, probably the heart and somebody would suspect project involvement. You will find it if it's there. We're talking here, Maura, about screening somebody where it's not so utterly overt. And the key is yes, you can absolutely miss it, which is why you need to do a bunch of biopsy somebody at a scope and got one biopsy taken. You could have just missed it. And so are our standard. And suffocation would be to do endoscopy and colonoscopy. Could random biopsies from up and down with the I track Thio really get a better sample? And when we're sort of in the diagnostic phase that, you know, we sort of you have used it as a zoo. A You know, if they've already had a biopsy, they've already had these studies done. We might as well look at whatever tissue is available to us and just saying it. Not that we're doing that proactively to have them get scope for that without symptoms. Of course. Well, e think that's a wrap. Uh, what time it is and no more questions. Wait, that's great. Yeah, well, thank you both for for joining us. That was That was, you know, a Z best as could be expected. Doing a virtual. I think that worked out really well. Thank you for all of our attendees. For for logging in. We really appreciate that. Yeah. Thank you, everyone. Thanks, Ron. Thank you for taking the time and thanks everyone for on a Saturday morning. You know, just joining in. Hopefully you got something out of this and hopefully we'll do this in person next year. Thanks for a great conference. Thanks, everyone. Published February 2, 2021 Created by
