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EP on EP Episode 77: Cardiac Sarcoidosis with Wend ...
EP on EP Episode 77: Eric N. Prystowsky, MD, FHRS, ...
EP on EP Episode 77: Eric N. Prystowsky, MD, FHRS, sits down with Wendy S. Tzou, MD, FHRS, to discuss an update on cardiac sarcoidosis.
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Video Transcription
Hi, I'm Eric Pustowski. Welcome to another segment of EP on EP. It's just always fun to have my next guest with me, Dr. Wendy Zhao, who's the head of electrophysiology at University of Colorado. I know she's probably mad at me because I always ask her questions about sarcoid in the heart. Wendy, that's what we're going to talk about today, cardiac sarcoid. Are you okay with that? Of course. Okay. Let's start from kind of a broad overview. Tell us about what we should know as far as just even suspecting cardiac sarcoid and how to make a diagnosis. Yeah. Well, I'll tell you, you know, over the last decades, we really have heightened our awareness about this disease process because in large part of very famous people kind of going down and post hoc, you know, realizing that they actually had sarcoidosis, not only in a non-cardiac way, but that eventually invaded and, you know, involved the heart and led to sudden death in those people. So from that experience and just from increasing knowledge that sarcoid is really a masquerader of sorts. It really can mimic other disease processes. A person can carry a diagnosis of, say, for instance, ARVC, if there's primarily right ventricular involvement, and be treated as though they have that when in fact they have something that may require different treatments to manage it. So there's a whole spectrum of presentations from essentially relatively asymptomatic, and it's kind of an incidental finding, to, you know, people who clearly have non-cardiac systemic involvement. And, you know, those are the people that we really key in on the most in terms of monitoring for cardiac progression. And I would say that that latter population is probably the one that we see in our clinic most commonly because, you know, they have a known diagnosis, say, of pulmonary sarcoidosis, and there is worry that they could have cardiac involvement. And again, that can be very subtle. It may not manifest as anything. But certainly if they have EKG abnormalities, be it in the proximal or distal conduction system disease, that's a little bit worrisome. A young person presenting with heart block, for instance, young, you know, is variable depending on what age group you're in. Everybody seems young to me these days. That's right. But, you know, per the guidelines that David Birney and Will Sauer kind of spearheaded for HRS back, I think it was in 2014, the consensus for the group was anyone under the age of 60 presenting with unknown heart block, you know. Under 60? Under 60. Really? Okay. Should be considered for evaluation, you know, to look for it because, you know, now it's an older study by Candelin, a large, long-term epidemiologic study in Europe following a cohort of patients. When they intensified their screening efforts, they really found a much higher proportion of people presenting who had cardiac sarcoidosis and some of them actually had just cardiac involvement alone. Right. Now I want to, that article really bothered me because we were always led to believe that it was a small percentage of sarcoid had cardiac involvement. And now we're finding, I think it was a large percent if I remember that paper, that only cardiac. So let's say you suspect it. You know, they have, I mean, I've had a patient, let me give you a patient. So I have a patient who has a heart block, a younger guy in his 40s, no reason, right? And so I did an MRI. Let's say I find something on the MRI, but you know, the usual differential could be this, could be that, could be sarcoid. Right. What do I do next? That's a great question and it is increasingly coming up, you know, as people get imaging for other random reasons, you know, we find this. What I typically do is, you know, a more comprehensive scan of like the chest, even just looking for lymph nodes or something that might be abnormal that could potentially be biopsied because certainly a native heart biopsy, especially when we're talking about sarcoid, which can be so non-diffuse that you know, the risk to benefit ratio of doing a biopsy in that setting is just too high to be acceptable most times. Well, I'm going to stop you for a second. So if you're lucky and you find something good, but let's say now you found nothing and you still got my patient. No, I mean, these are real cases that you see, we all see. So what's my next step? Do I just assume sarcoid or do I need to do something else? It's incredibly uncomfortable and there are not right answers at this point in time because I would say that the one right thing to do would be to continue to monitor, not lose track of that patient. So continue to have them follow up. The question of repeat imaging is there, but I wouldn't say that unless something happens like some change on EKG or symptoms or something that would prompt this clinically, I wouldn't necessarily recommend doing follow-up cardiac MRI, for instance, at any set interval. But it is something that you just kind of have to continue to follow. It's a very uncomfortable area because if it's not sarcoid, you could subject that person to treatments that they really do not need and could overall on the balance be more harmful than beneficial. But if you miss it, then it's the other way. So let's say, let's get a little bit into the treatment part. For years I said if I'm putting hardware into a sarcoid patient, it's going to be a defibrillator. Now I know that's controversial. This person I'm talking about actually came to me after he had a pacemaker and he had pulmonary sarcoid. And the question was, did they do the wrong thing? He actually brought it up and came to me, an engineer, and he had been reading. So I did do a PET scan on him and it was clean. So I guess in my gut I think he probably has sarcoid of the heart, but I left him on a pacemaker. His EF was normal. Is that the right thing to do? It's not the wrong thing to do. I mean, it's... That's good. I didn't make a mistake. Okay. I don't think it's useful for showing that he doesn't have active disease that involves the heart. It doesn't mean that he doesn't have involvement though, you know? And so it is something that just needs to be followed. You have the luxury of having the device actually, because then you can kind of real-time assess for concerning arrhythmias. And we subsequently did do an MRI. Initially there was a whole bunch, because the MRIs, you know, it was in the era where MRIs weren't, you weren't supposed to do them and complete heart block, but we sucked it up and did it. And his heart looks good on MRI. So I guess he's just a guy who has pulmonary sarcoid and heart block, and they were just unrelated. I mean, in that circumstance, it's actually, that's a little bit easier of a thing to deal with because he's already probably requiring treatment for the extracardiac sarcoidosis. So it's not like he has only cardiac involvement suspected. And then you're talking about like immune modulation and things like that. Right. Well, let's get into that a little bit, because then I want to get a bit into risk stratification in the future. So let's say a person comes in acutely and they have conduction problems and it looks like sarcoid. Your first step is in putting in a device, you would suggest medications, right? Yeah. I mean, I, there are cases in which the acute changes, especially with respect to heart block can be potentially reversible. For instance, with high dose steroids at kind of the outset, that's obviously not a desirable treatment for longterm if it can be avoided. So other immune therapies, including methotrexate and more recently, this is not so much for treatment of heart block, but being studied increasingly for treatment of cardiac irritability. So VT storm in these patients, Jordana Crone, Tom Crawford, and others with the cardiac sarcoidosis consortium are sort of coming together and studying the concept of using a drug such as anakinra, you know, for immune modulation, for acute management of VT storm in these patients. Because VT ablation in these patients, Well, I was going to get, that's your favorite topic, right? It is my favorite topic. You may not have wanted it to be your favorite topic, but unfortunately it's come to that. I love the topic. I do not love it in this patient population though, not in this specific patient population. So how do you approach these patients? You said it's a spotty disease. I mean, what do you do if you have to go in and ablate someone? I mean, do you, what's your technique for that? You know, so when someone comes in with storm, you know, the first thing to make sure of is that all of the potentially uncontrolled elements are controlled. That's true for any complex situation, but certainly true for them because if you bring them to the lab and they are highly inflammatory, you do run the theoretic risk of potentially making that worse. And so I will often try to quell them immediately with a steroid treatment, antiarrhythmic drug treatment. If they're still breaking through that and we don't have a choice, then I take them to the lab. Many times if they quiet down and they've had monomorphic VT, quiet down with the initial therapies, the fact that they often will also have monomorphic VT implies that there's some substrate there, some scar that eventually will probably get around to targeting. The acute treatment is really just stabilization and control of the arrhythmia with non-invasive means and then moving to the invasive step. So the question of the day is always risk stratification. Let's not take the one, that patient you just mentioned. We all get these patients that don't have horrible EFs, you know, they come to you with, you think are cardiac sarcoid, I mean, they have extracardiac, you've got biopsy proven, you know, the heart's involved. I guess if you need a pacemaker, I think, personally, I think the smart move would be a defibrillator, but what about if you don't? What about if you're just some doc, am I at risk for dying suddenly? What's your current way of approaching that person? That's a great question and actually our group has done a fair amount of work in that space with respect, and it's kind of an extension of Dr. Maeda's work with respect to using EP study to risk stratify these patients with sarcoid. It's usually just a highly suspected sarcoid based on imaging as well as an extracardiac kind of biopsy diagnosis. In our population of patients who had a normal EF, when we took them for an EP study, the EP study being negative had a good negative predictive value for absence of future effects. If they were positive, it was, you know, still a little bit uncertain, you know, the outcome. But I think in that population of patients, there may be some additional gains in trying to progress to that if you're really strapped for an answer. Voltage mapping of the right ventricle has also been performed because sometimes there can be some abnormalities within the right ventricle that are not detected on imaging. The right ventricle notoriously is harder to see abnormalities within, even on cardiac MRI. And so voltage mapping in that setting has sometimes provided some clues about whether or not we need to be more or less worried about these patients. Wendy, as always, what a great discussion. I can't thank you enough for educating our listeners. Stay well. Be good. You too. And thank you very much. Bye.
Video Summary
In this segment of "EP on EP," Dr. Wendy Zhao discusses cardiac sarcoid with Eric Pustowski. Cardiac sarcoid is a disease that can mimic other conditions and can range from asymptomatic to those with systemic involvement. Diagnosis can be difficult, but imaging such as MRI and PET scans can help. Treatment options include immune therapies and medications to control arrhythmias. Risk stratification is important, and EP studies may be used to determine the risk of sudden death. Overall, monitoring and follow-up are crucial in managing cardiac sarcoid patients.
Keywords
cardiac sarcoid
disease
diagnosis
treatment options
risk stratification
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