Drs. Adams, Gibson and Uddin review real-life cases from their practice and answer questions from course participants.
it looks like there's a couple other questions. I'll go ahead and read. There's a question and a comment for me. So I'll go ahead and read that and then there's one for dr Adams. So one quick question for me. I believe on one of my slides when I was talking about papillary muscle fibrosis with regard to mitral valve prolapse and metro annual or disjunction. The question is what is L. G. E. And related to the in relation to the papillary muscle? That abbreviation is late gadolinium enhancement which is a feature on cardiac MRI which indicates scarring and fibrosis. So I apologize for not having that listed out there. So but that's that's basically an indication of scarring and fibrosis um that is associated with those higher risk features and the people with mitral annular disjunction. Another comment here which I appreciate when we were talking about the use of beta blockers during pregnancy with regard to S. V. T. And arrhythmias. Although I did say that beta blockers are considered safe in pregnancy. That things to uh keep in mind. There certainly are risks though and I'm not sure who made this comment but you're absolutely right concern for possible use beta blockers extensively throughout pregnancy. There can be a concern for intra uterine growth restriction. I know there is also a concern for fetal bradycardia at times, as I mentioned, it does pass a little in the breast milk as well. So postpartum can be an issue. And with regard to vagal maneuvers just to emphasize you want to be really careful about those in the third trimester, somebody really close to delivery, encouraging vagal maneuvers with S. V. T. Can certainly cause preterm labor. Um The other thing which I didn't mention but I just thought of now since this question came up there were a very few case reports about actually doing ablation in people with during pregnancy which sounds really scary. Of course it would be a last resort. There was one case report of a woman who had V. T. Storm unfortunately. Um And so of course you know you take all your precautions for proper shielding. You know radiation safety presumably. You know this is not going to be somebody who is going to be having multiple relations. So you certainly try to avoid it. But there have been a couple of circumstances where women have unfortunately had to undergo ablation during pregnancy. And I believe there's a little bit of a risk. Even though we said cardioversion is safe sometimes again towards the end of pregnancy there can be a risk of putting those women into preterm labor. So I appreciate that comment um from where that came. Um It appears that there was a question for dr Adams can you are you, yep, she's there with us as well. So, the question it was addressed a little bit, but maybe we can just clarify one question. I'll read you the whole question. I think it will be easier to address that way with regard to the atrium of atrial dilation. It says, how does neurotransmitter imbalance fit into a complete assessment for a rhythm control strategy? Where does atrial dilation fit in as well? And then the second part of that question was to get a little clarification on a fib and men during vigorous exercise, but then having increased vagal tone during sleep, um maybe I'll let you address that part. And then it seems like there's part two of that question is Yes, So, so clarifying again, you know, when we talk about a fib we need to I think, be a little bit more specific about the type of a fib we're talking about. So we do know that there is an increased risk and really kind of male athletes. Again, these are typically elite athletes, again averaging greater than 1500 hours of vigorous exercise per year. So when male athletes they tend to have compared to female athletes, they have more concentric ventricular as well as atrial remodeling. So the atrial remodeling is thought that that might cause some a fib. So when when athletes have the remodeling they actually get it can alter their diastolic function. They men also tend to have higher blood pressure during exercise um and higher sympathetic tone. So when the atrial remodels they think that's that the actual atrial modern self increases the risk of a fib in these athletes. Now this is not a fib during exercise. So in rat models. And again, most of these they actually make endurance rats, which I think is kind of funny to think about but they show that this vagal enhancement. So athletes get there a fib during vagal, high vagal tone and during the vagal this vagal enhancement that promotes reentry um causing this short shortening of atrial re fracturing this. So basically it's not that they're getting a fib, it's not exercised, induced a fib, it's endurance athletes who get a fib but they're paroxysm a fib most likely comes on during a vagal time, during high vagal tone rest, sleep, postprandial. So uh so when in the as opposed to young male athletes, the vagal triggers predominate in men and that's kind of the gender difference for both men and women. Again, chronic long standing hypertension tends to lead to atrial dilatation, diastolic dysfunction. And again, we think that's associated with triggers for a fib. I'm not so sure about the neurotransmitter question. Um Maybe they can make a question about a follow up question on that. And there was one other question which you're welcome to answer. Any of us can answer is can you explain what's meant by left atrial appendage velocity? Uh Oh we love the left atrial appendage velocity. So um yes so those of us who do T. E. S. A lot that velocity we use as to uh you know just interrupt just for those who don't know T. E. S. Is trans esophageal echo which allows us to get a more close detailed look at the left atrial appendage. Sorry dr Adams. So the concern is that if a thrombosis forms and that left atrial appendage even after a successful ablation which we jokingly referred to as cosmetic, cosmetic, sinus rhythm. So the patient still in sinus rhythm. But if you image there left atrial appendage, the velocities within the left atrial appendage are still very low and the inflow from the mitral valve are also still very low. So we still worry about could that person still be at risk for forming a thrombosis in this kind of dilated unhealthy you know geographically changed atrium. And I think Doug should really comment more on this than I. But but yes L. A. We love you know actually that reminds me Doug you can talk about you know when it looks smokey and the appendage what you sometimes do in the E. P. Lab to that's the velocities pendants velocity. I'm surprised it hasn't made it onto the chads vast scoring system. To me that's one of the more reliable risk factors for stroke. You know we do something we do in some patients have to isolate the left atrial appendage. You have to a blade around the mouth of it. And so even in sinus rhythm, the appendage velocity can be low in those patients continue to be at increased risk for stroke. And it's pretty impressive. I mean, you have to really be careful with those patients. I tend to think that that's a good patient population for Appendage inclusion because everybody, nobody goes on a blood thinner for stage for 10 years and stays there uninterrupted. Um so anyway, that's what the appendage velocity and stand to that question. And we measure the velocity of blood flow in and out of the appendage. The lower it is, the worse. It is more likely to form smoke and thrombosis. And we do have uh we actually submitted that paper finally has been working on this for years and get a couple fellows interested. And so hopefully that will get accepted here soon. But about 20 to 30 patients where we gave ISIS paternal when we have smoke independent. You can tell there's a clot there or not. We gave ISIS paternal increase the cardiac output and oftentimes the smoke will clear showing no clot. We had one case where the smoke cleared and showed a clot down deeper. Very sick patient patient went to transplant four months later out in new york and they pulled a big ugly looking cloud of dependence. Show all those patients had. Yeah, I think all of them had low appendage philosophies in that in that study so that the paper will be coming out soon. Hey Christina had a question for you about the exercise and do stated that's that's one of the more interesting um aspects of research. Did you see anything about oxidative stress? So with those, those endurance rats, um they actually found atrial dilatation which we see in human athletes but associated with that. You know obviously they sacrificed rats after months, after months they took out their hearts. They saw atrial fibrosis there. So something's killing heart muscle cells and those those endurance rats. And there was some discussion about oxidative stress, but I haven't heard much about that lately. Yeah, I agree. In fact the in human studies um it's well known typically after marathons that people have elevated proponents um and so that there is some damage to the myocardial. Um And obviously there's going to be individual variability um Which which when we talk about oxidative stress. The question is you know, if you did I think more cardiac MRI is gonna be really helpful because if you stuck a whole bunch of athletes, you know, in the MRI scanner, that's where we can really see that enhancement in the atrium for fibrosis. But it looks like it's really affecting men more than women. And we talk about endurance sports, it's really those um long distance cyclers and um with your political behind you and with with cross countries here's so I think though for the general american population though everyone needs to be exercising more rarely. Are we going to get you know, have our patient population developing that you know that uh exercise induced a fib not a big definitely seeing that there was some guy who was working on a mitochondrial specific antioxidant. Um and I just never saw that go anywhere. But they thought the oxidative stress was generated in the mitochondria the powerhouses of the cells. And basically when you you basically rest the mitochondrial oxidative stress, the mitochondria dies, the cell dies gets replaced with scar tissue. And so antioxidants obviously have free radical scavengers in our bodies and you can take antioxidants. But the mitochondria has a very high negative charge density and whatever you take, Coenzyme Q 10, it kicks it out of the mitochondria. So I think he put a positive charge on. I think it was coenzyme Q-10 coupled with the positive charge molecule goes into the Mitochondria and stays there. But then I never I haven't seen anything come out of that. Yeah I agree. I think again the problem with that Coenzyme Q 10 is that there's there in the limited studies that they used there were too many different variants of which supplement they actually use. So there wasn't there isn't a generalist recommended manufacturing for coenzyme Q 10 or for example reserve a troll or some of the other kind of anti oxidative stress you know. But I think that's a that's a wonderful area of research. I'd love to see more on that. That's great. Thank you that. I actually love that discussion. I think we probably end up talking about some of these things all day if we're not careful. So, so thank you both. Um I have a couple quick cases I was going to present related to the topics we've discussed so far and then we all get a little bit of a break um for for whatever you like, we'll take a break from about 1015 and what will resume it? 10:45. So I'll quickly go through these cases again. Happy to take questions during these cases. Everybody else's welcome to chime in. So let's see if I can advanced the, here we go. Okay, so this is the first case I mentioned briefly when we were discussing the concept of mitral valve prolapse and metro annular disjunction. And you know, this is one of those bizarre things that I think happens to a lot of us where you learn about something and then all of a sudden you see it like everywhere, even though you weren't noticing it before. And so I I wasn't really paying too much attention to mitral valve prolapse in this Dangerous, a rhythmic way. Um and this is a patient that I actually saw in 2017. She was in her thirties at that time, young healthy woman, largely asymptomatic, and she was actually referred to me for a symptomatic bradycardia. Someone just I think she noticed it on her Apple watch, you know something and this is somebody who exercises, She was running several miles a day. She was going to Orange Theory, which is some of you may know that that exercise class where you actually monitor your heart rate and everything, It's up on a big screen while you run on the treadmill. So so it was brought up then and you know, so I saw her she had a diagnosis of mild mitral valve prolapse with no regurgitation completely asymptomatic. Um and so you know, we we repeated an echo or we didn't echo at that point and we put a heart monitor and she was largely asymptomatic, a couple of PVcs on her monitor, but nothing concerning. But I do remember at that time um she was there with her mother who was a nurse and her mother Also had a diagnosis of Mitral valve prolapse always asymptomatic but actually had sudden cardiac arrest in her 60s out of the blue. Not symptomatic from her mitral valve regurgitation or prolapse or any of that. She was very fortunate her mother because her mother happened to be married to a physician who was able to resuscitate her during this cardiac arrest, all of which was very, very lucky. Ultimately, her mother ended up getting her mitral valve repaired and she ended up getting an icd. So that was the reason why I was paying a little closer attention to this woman just because of the family history, that's really what caught my attention. So at the time we did a cardiac MRI and I'll be honest with you, it didn't really catch my eye so much at that time. But then she just came to me and follow up this month and what I wanted to show you here. Hopefully you can appreciate this is her. Hopefully you can see these images. Her initial Echo in 2017. You can see a little bit of prolapse of the leaflet here, but not significant regurgitation. And again, same thing here. You can see these lethal still come together reasonably well. So to be honest, most some may have even called this normal It's not a very significant mitral issue at that time. We did do a cardiac MRI, which I have shown. Let's see here and we didn't really pay attention to this mitral annular disjunction actually, recently when she reads, when she came back to my office, had our imaging experts take a look at this and they were actually able to measure if you can see this 4.9 centimeter mitral annular disjunction here, from the leaflet of the mitral valve to the ventricles. So, as we were talking about before, it's just a truly displaced mitral valve leaflet. So the repeat echo that we did just about a month or so ago, um you can see here is that we ended up doing it hears her regular echo all of a sudden. Hopefully you can appreciate that she's got a significant more regurgitation there. You can see that backflow in that four chamber view, all of this blue coming back. So so this was actually interpreted as severe mitral regurgitation over the course of you know, almost 3.5 years. Um And again you can see here on the trans esophageal echo um the for those who aren't as familiar with looking at trans esophageal echo, this is the left ventricle here. These two are the mitral leaflets and this is the atrium and hopefully you can appreciate that the leaflets are thickened and curling and a little bit below we hear this. This is kind of what we classically called Barlas mitral valve where it's thickened, it's something usually congenital and you can see quite a bit of flow here. So based on these, the question for this and this is actually a decision we're making right now. So the question is, is she going to go the way of her mother because she does have this mitral annular disjunction. And the one question I have for kind of a group which I don't think we have good data on is we'll fix. So she's still asymptomatic, she's still running several miles a day on the weekend. So well, fixing her mitral valve, that's one issue and that may help with the regurgitation, but she's really asymptomatic with no left ventricular dysfunction. So there's no urgency for that. Is there any benefit in fixing the mitral valve? Will that reduce her risk of arrhythmia? And the next part of the question is is this someone who should prophylactically have a defibrillator because of what happened with her mother and what's the best way to monitor her. So I have some ideas. But I wanted to see if anybody um from the group has any anything they'd like to add or chime in on at this point. So dr Adams is sitting here behind me and she is recommending possibly an E. P. Study. And I think Doug is smiling because didn't you do an ep study on somebody like this recently? Yeah similar similar patients story without the this lady had non sustained ventricular tachycardia. Same exact story except no family history of cardiac arrest. And she did have non sustained V. T. On a monitor. And this is a good case worthy of discussion because obviously there's some concern here about sudden death but there's a very significant lack of information about how to handle this. You know E. P. Study is a reasonable tool but it's not perfect. It has false positive and false negative rates. So you can produce a study that's positive. Give a patient a defibrillator and they may never use it. Or even worse perhaps is uh you know tell somebody they're safe and they go in and have a cardiac arrest. And you know what I failed to mention on here. She does have that mitral annular disjunction. But there wasn't any obvious scarring fibrosis or any of that gadolinium enhancement on her on her MRI. So the other thought to is will she develop it over time? Do we repeat to repeat MRI or do we just say hey you have this one feature. Not all of the high risk features. And the short answer is we don't know. But yeah I think the repeat MRI would be good because obviously the more scarring to have at least one of the papers were published, the higher the rate of cardiac arrest was wasn't it? And so when they start to get fibrosis that involves the myocardial and the papillary muscle heads. That that's the more ominous signs. Following with an MRI would be reasonable. You don't have any non sustained V. T. And her, right. And you know just a couple of Pvcs on her monitor. So my plan so she just had this t done actually last week and as a matter of fact I told her I'd call her after this conference so we can decide what to do. But are tentative plan was actually to put a loop recorder in her to monitor her more closely to see if her PVC burden goes up over time. Or if she has any little brief episodes of non sustained ventricular tachycardia. Because those are certainly higher risk features which, you know, the bigger question is really if and when she should have a defibrillator, I'm actually less concerned about the weakness of the mitral valve because that's something we can monitor with symptoms and and with eco over time. So, so although I did refer her for, you know, a surgical consultation just to look at the valve, you know, we discussed that that's really the less urgent of her problems. It's more the arrhythmia that I was concerned about. So, so that's our tentative plan for now, is to put a loop recorder in and really monitored closely for arrhythmias unless anybody else has any any brighter ideas. So, and doctor came over here if you heard mentioned genetic testing and I think that's a reasonable um option as well. You know, for those of us who have done genetic testing, it's tricky because sometimes you don't know what to check off on the boxes as far as genetic testing goes. Um It's certainly something I'll discuss with her because this particular entity doesn't fall into a classic cardiomyopathy. I'll have to look at the form and even see, I mean I suppose we could test for arrhythmias and channel open these and things like that perhaps. But I think that's a great point. Okay great well thank you for that. I'll move onto the next case. These two will be very quick. Um This is, oh by the way this is for cardiac monitor which I meant to show you which really didn't have anything significant. Um And again this is what Dr Gibson just summarized for us again that it's a combination of the substrate, the fibrosis, the stretching and pulling the scar that fortunately she doesn't have, she just has that mitral annular disjunction. So again as we talked about planning to put in a loop recorder with her and I'm strongly considering sub Q. I. C. D. If we see a lot of pvcs or arrhythmia for her. Um So the next one I wanted to mention is actually a case that I got from my colleague dr Rogers will be talking this after actually after the break. Um So this is interesting. So this is another very healthy 46 year old peri menopausal woman. Um This goes back to our discussion about hormones who began to have palpitations in 2019. Um You know sort of around her perimenopausal time. But you know this is a healthy woman who competes in these triathlons and these iron Man. You know she's exercising, she's asymptomatic with exercise but she really started to be bothered by palpitations. And so she ended up wearing a zio patch, a cardiac monitor and she actually had a couple episodes of non sustained ventricular tachycardia, which really doesn't, that's not normal, you know, an otherwise healthy exercising woman, we let pvcs go and we let P A. CS go. Um so she ended up having a stress echo. All of that actually came back normal as expected, given that she she's been athletic, but what was interesting and this is again, a really quick cases, these were really progressive and quite bothers them to her. Um and we weren't quite sure what to do exactly about that. VT interestingly she was also because of being paramount apostle undergoing some work up by her primary and she was noted to have on outside lab testing, particularly low progesterone levels and this was sort of put on the back burner because of course when we see something like VT we get, you know, we sort of get more worked up over that. But over time once we sort of, you know, she wasn't having any single p she was just very symptomatic with the PVcs overtime. Her primary actually thought to put her on progesterone replacement therapy and all of her symptoms went away, The palpitations, you know, the PVcs, the she was monitored again, everything kind of went back to normal. So this is kind of an extreme example. I mean, it's hard to prove, you know, is it just that, you know, was it a coincidence? You know, I don't want to say that progesterone saves the day all the time, but it was really interesting because really all the other work up was unrevealing and she was perimenopausal and she was the one who brought it up saying, hey, now that I'm on this kind of hormone replacement, I feel much much better. So I thought that was an interesting example. Um Oh sorry yeah. Are you guys checking progesterone levels now regularly? Because I based on what dr Adams presented Right? Yeah. So I'm not because I'm afraid of opening that can of worms however because I'm afraid but and we're all afraid. But but fortunately I actually found on this one of our colleagues dr del home, I don't know if anyone knows her and I don't mean to call her out here but she works over at the integrated center. Um And she's actually become quite good at managing she just primary care but she's been managing a lot of these hormones. Actually had a conversation with her. She was the one who told me that she's been seeing, she's been observing in her clinic that it seems to be the unopposed estrogen that tends to be a little bit more pro arrhythmic or at least symptomatic. So my new plan is to send these patients to her for help when I suspect that their hormones are at play. But I actually was hoping to collaborate a little bit more with her over time because she seems really knowledgeable in this field. But yeah, that's a great point. And I think I think I would over time like to get a little bit more knowledgeable about these hormones, but I think we need to know what we're doing first is the problem I'm afraid we'll just check them. I'm a little bit concerned about people just then, maybe inappropriately going on hormone replacement therapy. What's the downside? Well, there's a there is a slight increased risk if the dose is high again of being a little pro robotic saying, you know, over time, if I think that the problem that I've noticed with a lot of people on hormone replacement therapy is it's really only considered safe for up to five years. And sometimes you'll see these like 70 or 80 year olds who've been on it forever because no one's really monitoring or managing and people are afraid to stop it because they will become symptomatic. So my concern is not so much starting. It is that it doesn't seem to be managed closely. And I think the reason for that is a lot of people aren't comfortable managing it truthfully. Um At least on my end, I don't think I personally would say that I would feel comfortable managing somebody's hormones. I'd probably have to take a course on that. I think Dr kim took a course on hormones. Maybe she can tell us about it, but even after taking a course we get scared. Uh And this last is a quick case that I'll just show you again. This is another one of dr Rogers patients. Um This is um this is a woman who actually has unexplained sudden cardiac arrest in her thirties and unfortunately happened so long ago that she you know she she fortunately got an I. C. D. But we don't really know the cause it was before everybody was getting MRI. So she has this idiopathic VT. Um And she's always had a high PVC burden. And what I just circled below here that you can appreciate is that her Pvcs. So you know on most defibrillators pacemakers you can keep track of somebody's PVC burden and you can see that based on her hormone replacement and what goes on she actually decreases her PVC burden when she's on more progesterone. Um And we've been actually able to kind of go back and take a look at that. So that's been interesting as well. So I see them one minute overtime and I want to respect our break before I go. If there's any, um I don't know on our side if there's any questions that anyone has, we can certainly take otherwise. We'll we'll give everyone a little break. Great. Okay, thank you everybody for hanging in with us on a saturday. Thank you.
