In Part 2 of our discussion on the risk of sudden cardiac death from arrhythmic mitral valve prolapse, a case study offers new insights into properly identifying, diagnosing and treating high-risk patients. Topics include:
Risk factors and risk stratification
What to look for in a patient’s Echo, MRI and PET imaging
Relevant EKG characteristics
Potential electrophysiologic and surgical approaches to treating Arrhythmic MVP
View Part 1
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Hi, I'm Doctor Randy Martin here at Mount Sinai. We had an interesting discussion about arrhythmic mitral valve prolapse in part one. But now in part two, we want to talk about a case and really look at the new knowledge that we've gained. You've, you've helped me out a lot to understand things. And now I want to know, I wanna know more. I wanna see a case and know how I should be working these people up and this is a brand new case. So it's perfect timing. This is a 55 year old female uh was seen for occasional palpitations by uh I think first an internist and then a cardiologist, they did an EKG or they did an exam, they noticed there were some extra beats. So they decided to do a holter monitor, perform the holter monitor. The PV C burden was about 15.9% which is moderate, which is less important to me than on the actual holter. She had evidence of couplets, nonsustained vet and at least two different morphology, complex ventricular activity. So the patient came to the doctor because of the palpitations just for the palpitations. Ok. That's important because there's got to be something that will get you there. That's usually what I mean early on. Had no doubt. Ok. So she gets an echo. Then they decided to do an echo because they saw the PV CS. She had Bileaflet prolapse. Mild Mr at best. And they did note Mitra annular disjunction about 13 millimeters. There was, is it more, it, it's getting into the range where you, you're more concerned about it? So it was like an advanced prolapse already. So if you have this junction, we know it's not an international state, it's been going on for a while despite the fact, there's no regurgitation yet, which is important to you. OK. So then this is just an example of the 12 lead EKG that was done. So this is a 12 lead EKG. We look at this EKG and the first thing we try to identify is what is the source of the PV CS? Is it in the left ventricle? Is it in the right ventricle? Does it involve typical structures? So, in this particular scenario, what we notice for it, besides the fact that there's couplets, which is always concerning is that one of the, one of the PV C morphologies is has a right bundle branch morphology with a superior access, which to us usually indicates that the papillary muscle is involved. But this is that this PV C is originating from the region of the papillary muscle. What's also interesting is that if you work further down the EKG, there's a second PV C morphology shown here. And you know, if you're an electrophysiologist, what you'll notice is and if there's any electrophysiologist watching there's a QR pattern, there, a QR pattern usually indicates the aorta mitral continuity. Again, another area that's exposed to stress that are causing these, these PV C, we have two areas that involve the mitra annul lists which are giving off PV C. So there's actually recent literature where they took a whole series of patients with mitra prolapse, they put them all on treadmills, they ran them in order to induce PV CS. And what they showed was that the majority of the PV CS were coming from the papillary muscle, but at least one third of the PV CS were related to the mitral, whether or not it's the ac region or other areas in that aorta mi continuity. Again, these are areas that are probably exposed to the forces of the mitral valve. And mark, do we know if this patient had normal coronary anatomy? Because presumed to be? Ok. So that, that's not the context that we need to point out that the presence of this ectopy in these patients are in the absence of other structural pathology. So the patients have normal coronaries and they don't have channelopathy or other abnormalities for the conduction system. This is worrisome is concerning and, and someone with prolapse, I mean, it would be concerning to me and someone without prolapse because why should you have, you know, couplets and that sort of two morphologies. So, because of the presence of what they identified as two risk factors, which is complex ectopy and significant micro ruction 13 millimeters. So it's not an overall uh they refer the patient for a cardiac MRI which makes sense. Again, if you look at our triangle, you kind of work your way up the risk factors. Ultimately, what you want to decide at the end of the day is do they have the substrate that would predispose them to cardiac arrest? Which in electrophysiology is fibrosis. So the MRI wasn't to confirm if there is a Mary, it was just for substrate because a lot of, a lot of these uh patients are sent for MRI S to, to confirm if there is a month. How much is it? OK. So you're, you're saying, so we had two risk factors which you would agree with. And now the next step is to see if you've got scar and where is the scar? Right? Yeah, I guess I would say three risk factors, female gender complex ectomy and as well as an med. OK. OK. So we need to change that to three. That's true. No, but, but, but we are creating and she's in the right age range for it. Um So she gets a cardiac MRI, they, they call that there's LGE in the basal and prose. Now, obviously, we don't, we don't do the MRI ourselves, but we would believe that although we'd more commonly see it in the basal inferolateral region, like the classic mitral valve prolapse scar pattern is basal inferolateral. What we've learned from our own imaging studies is that when patients have uni leaflet prolapse, they tend to have the classic mitral valve prolapse scar pattern, which is basal inferolateral. When they have bi leaf prolapse, they can actually have a patchy distribution of scar almost to the point that if you weren't paying attention to the prolapse, you would assume they have some other substrate like sarcoidosis. In fact, a lot of our hybrid pr I have a very typical pattern that you'd almost be misdiagnosed as cardiac sarcoidosis if you didn't realize that this also occurs in bilingual. So it's patchy in our own experience. It's been patchy in the Bileaflet prolapse patients very focal in the unal leaflet prolapse patients is the distribution for the patchy still same or is it all is it, it tends to be septal, some septal, some anterior, some basal and prolapse? What's interesting is that almost makes you wonder if that goes to the question of because people have always asked why is high leaflet prolapse, higher risk than you need leaflet prolapse? Like what would be the difference if you look at it from an electrophysiology perspective? And we know this from the non ischemic literature for many years. When you have a patchy distribution of scar in a non ischemic patient. They are much higher risk for sudden death than if they have just focal replacement fibrosis. So maybe that's one of the reasons their distribution of scar placed them a higher risk for future in. All right. So what happens here? OK. So the question comes up, of course, you know, do you do additional imaging at this point? Do you just observe, do you give her beta blockers and antiarrhythmics? I mean, she, she just came to see, see you because you being an internist or cardiologist because she has Ivy. Now you're, you're telling her that we need to look further because this could, could potentially be a risk factor to you for others. We know that she has a risk for sudden death. What we don't know is she has, she has all the, what is her risk like when they ask, what is my, I'd like to know if I, what's my risk of having sudden death, correct? What's the best? You could tell me? I could tell you that on the highest end, it's 2% per year, on the lowest end, it's 0.3% per year. So that's, that's a significant difference. Wouldn't get on a plane for a 1.8% risk of crash. You don't want to be that 1%. No, I got that right. So I think the question is, can we modify this? Can we modify the severity of the p, there's no amar to correct at this point. Well, I think what she's asking is, can we modify her risk for? I, I don't care about the MRI want, I don't want to die. So, what I'm saying is that we, we, there's nothing more to control but that the toy and how that can because most people would, what we, we said at the outset it's, you know, I've got Mr, so I need surgery on my valve. This does not apply here. It's mild, Mr, she has now done her reading. She's gone online. She recognizes that as soon as she types in the word mitral prolapse or any arrhythmia. The 1st 50 articles will be about the risk of cardiac arrest. So she is now comes to an electrophysiologist or a cardiologist and she says, what do I do if I know more information, can it be treated? And that would be outside of putting a, an I CD is a, is a big deal and we can, you know, obviously we cannot be putting and that's the treat when the injured. And until recently, there was no guideline recommendation for this. And the absence of what I'm asking is there are other things I can do to get to clarify her risk personally. At this point, I don't think additional imaging changes moves the needle study changes. So that's a good question. Is, is, does an EP study predict risk and the answer is, we don't know. And there's no clear consensus on whether or not an EP study is either sensitive or specific for the risk of future sudden death in patients with prolapse. We know that in other substrates, ep study generally is not predictive of risk. So you're telling me that that 12 and four not going to help as much that I've either got to choose from three or five. Is that right? Two observation is a reasonable strategy. I don't want to be observed if I've got a rescue, but for 20 years, that's what people were doing. No, no, I understand that. But I'm just saying, I think the real question is number three or number five. Do you give beta blockers again, we give beta blockers to people with ectopy. What impact it has? We do not know, but it is recommended to give them beta blockers or I CD and I CD is a big step. It is. So this, this was the consult note. I just want to read it at the outside hospital just to show you this field has, has progressed in the last few years. You would have never seen a consult note like this in 2019. Just an example. So I'm just gonna read it for you. Her MRI Findings echo and case were reviewed in the EP attending conference given her high risk features. Most everybody was comfortable including me in recommending a defibrillator So the patient actually took this opinion, got a second opinion. The second opinion said it sounds quite reasonable and, and then they opted to have the primary prevention ICD impact which for a young woman is a big lifestyle deal. I think it's a responsible choice because it has mileage ahead of her. Yes, but there's also, there's hundreds of thousands patients similar. So when we, when we put in, for example, I just give you a hypertrophic cardiomyopathy. The old thinking is to be everyone with hypertrophic cardiomyopathy should get an I CD because they're at risk. Nowadays, we only put in icds for patients with an annual risk more than 1%. And we have a risk score to achieve that you have to have a certain septal size or a certain or you have to have a aneurysm. So for most people, if I have it again, if I have VFIB or something, this will shock me out of it. So this is giving me a little bit of insurance or a lot of insurance, we have to be careful, we don't over implant. So this patient agreed and underwent a dual chamber I CD implant on February 15th, 2023 which is about three or four months ago, correct? And then on March 7th and this is an electrogram from the I CD. The patient had a PV C that initiated her ventricular correct, first documented BF and she goes into ventricular fibrillation as you can see here on the RV channel and she appropriately gets defibrillated out of it. Now she's had an episode, correct. So the right choice was made in this particular patient. So after this, in addition to the beta blocker, she was already on Fleck aide who was an anti arhythmic was started hopefully to suppress the PV CS. How effective that is. I don't know. And then two weeks later, she had another shock for the same thing PV C induced V TV. F. So twice. Now, in a short period of time, she was saved by the defibrillator. Again, this is just an extreme example of someone who got lucky. Yeah, but she's now really anxious over this, right? So now the question becomes ok. So you saved her life. She's happy about that. She absolutely hates the idea of being defibrillated, right? She can avoid it. So now the question comes up. Do you just add a new anti arrhythmic? She's too, she's always too young for amiodarone. Do you offer her again? If you remember that triangle on the outside catheter ablation of PV CS, hopefully that if you remove the trigger, then you remove her recurrent I CD shocks. It doesn't necessarily remove all her risk, but it can reduce the risk of future events. Do you offer her mier or micro surgery? Plus PV C? She still got mild Mr Don't you? Correct? And I think if this patient had moderate to severe or severe Mr then three and four would be the right answer, right? But with mild Mr two is the right answer. Two would be the correct answer. So that's what we offered her cat ablation and PV C. And what I wanna show you here is just an example of, you know, how much more we're learning as the process goes on. So this is what's called a scar map of the left ventricle. What you see here is this is well, purple is healthy tissue. This is the endocardium. Ok. And then this is the endocardium, what's called a unipolar map. This looks at the epicardial surface of the heart. Here is the mitral valve, here is the apex of the heart. And so you can imagine that the papillary muscle is right about here. All of this green tissue here is cardio fibrosis. So even though the MRI doesn't identify replacement fibrosis, she clearly has interstitial fibrosis in a location that would be expected for mitral valve disease, mitra valve prolapse showing again that her substrate, even though her Mr is mild, her substrate is worse than just mild. And this would explain why she is obviously able to perpetuate ventricular. What do we do now? Well, so she's been treated for catheter ablation of PV CS. And so now it's just basically a waiting game. Eventually we'll wean off her medications and we're hoping that by removing the trigger, we've removed her future I CD shots it could be another 20 years before she needs microvalve surgery. If she had moderate or worse, Mr, then she might have had surgery early on with when the thinking was if you take, get rid of that. But in the presence of Mr, yes, that would be a modifying factor. The question is because of the complexity of the ectopy, whether, uh, the correction of the Mr would actually have a benefit to the ectopy, I'm going back to her. So if she had moderate or more, Mr, then surgical correction of the Mr module, well, repair with abortion at the time of that would also be an option. Is that correct? Correct? OK. Even if you have in this case, more than one f, right? Because you, well, here she has at least two foci but at least one of them involves the papillary muscles. You can do an ablation there. And the hope is that the other one might be in the aortic. Exactly. So that, that can be addressed by the Mike. So where do we stand? Because I mean, this is fabulous. It's really good. And I've learned a lot of stuff. Where do we stand with our knowledge? Do you have a summary? Now of this, I know you've got MRI, you have MRI with inflammation and I want to see that because that's important. So I have a, I have a different case which I think gets to the question of, you know, how do you manage these patients with ablation versus surgery and or both of them at the same time, if you think about uh mitral valve disease and and ventricular ectopy as two different mechanisms, then your therapy has to be focused on that. So as an example, you asked about, do you just do mitra valve surgery? Just do ablation? So if you, for example, here in this scheme, right here, if a patient has prolapse and mechanical traction and they've already developed their fibrosis, so they've already developed their scar. Then if you just fix the mitral valve, then all you've done is really affected this pathway here to get to your ventricular arrhythmias. This pathway here is still present. So in our particular experience here, if we think if we can affect both this pathway with ablation and this pathway by fixing the mitral valve, then we should be able to block all the future ventricular rhythms. But again, you need to have, you need to have enough Mr to do surgical intervention. Is that correct? Correct? But also I think it's important to add that correcting the matra regurgitation is not removing just the mechanical traction forces. It also improves the intraventricular hemodynamics. This ventricle has been chronically loaded and it has by way of the prolapse itself, abnormal vorticity of the way that the blood flows into the ventricle and moves out of it by correcting it. We actually alleviate those hemodynamic forces, those shear stresses that affect and trigger inflammatory pathways that are contributory into the genesis or sometimes she is a little of perpetuation of arrhythmia you showed in an MRI and pet scan. I mean, those are elegant. Now you're really adding another level is that needed in patients. I think for wrist stratification, an MRI is needed. I don't think you can risk stratify without an MRI. I think uh hybrid pet MRI is useful is adjunctive for wrist stratification but not absolutely necessary. Obviously, although it is, it is useful for uh peri surgical planning, I think because it can actually help us identify the location of the ventricular ectopy. And just to give you this example here, since you brought it up is this is a patient who had a hybrid pet MRI whose PV CS were not papillary muscle. So doing a papillary muscle ablation during mitra valve surgery would not be appropriate. Their inflammation localized as you see here just to the A MC region and the PV CS on the 12 VDKG were classic A MC appearing PV CS. So in a patient like this, if you are going to perform micro valve surgery and you have the opportunity to perform ablation, focal ablation, that location, you can probably affect the PV CS more than just fixing the valves. So, so I mean, so the take home messages are money but, but you told me a couple of facts that are really important, you have a you have a risk profile of patients that we go through the triangle in there and you have, have those uh that the degree of Mr is not the deciding factor. It's important if you're going to think about a surgical approach, that's reassuring, I would say correct. Ok, that's good that knowing where the PV CS are coming from and looking at the extent of scar and the ventricle is really important. So, so in this woman, knowing what, you know, now, after she's had to, would you have treated her differently? And in other words, we all, we all agree that the, the I CD was the right thing to go. But knowing what you know, from her, now, would you have gone in if you'd done, looked at the burden of scar, would you have done something differently? It's hard to know with the retros cope what you know, I know? Ok. But, but treating her, but, but treating her her w eye, I mean, with abortion was, was absolutely the right thing to do because now what we're doing is treating symptoms, her symptoms are her I CD therapies, she's not going to have death because she has a defibrillator. But we still have to actually treat the I CD shot. You modify the risk factor, definite quantify the risk factor like this. So, is this data now available to the common cardiologist to know? I mean, you've, you've taken us through a whole bunch of stuff because there still is a lot of, I mean, you know, the number of papers on mitra an disjunction are just, there is a lot more information available and there's not the American Heart Association and American College of Cardiology, both here in the States and the European societies have uh uh uh started an immense uh effort to inform uh institutions and, and physicians about the risk and both with uh uh you know, workshops and other media. I mean, I, I think mostly to educate patients. Yeah. No, I think it's, it's, and, and physicians though, I mean, you know, I think it's really important. You all again, going back to the concept that you've got a, a team approach to arrhythmias and again to uh Doctor Adams and his team's compliment, they've got you integrated into this as the EP expert, but you've got a team approach which is really good for patients because it allows really more, more knowledge of, of uh to their problem. Yeah. And I think to that point is actually very important. We used to have a slide where we showed at least here at Mount Sinai, which is, you have the cardiac surgeon, you have the cardiac imager, you have the electrophysiologist and they all kind of work as a team together. And the important part is that who's ever on that team sees a lot of these patients over the course of many years because over time you start to identify, you can kind of almost uh sniff out. This was the higher risk patients. This reminds me of that patient I saw and all that. Yeah. Yeah, you have to have a systematic approach to, to addressing these patients because although they're obviously quite different in their risk factors, the patterns are relatively similar, the patients who will have complications, their prolapse and even period operatively, we've effectively modified the way that we induce anesthesia. The way we take care of this patient during surgery, not per se, the management, of course. So from the induction point, all of the way that we can reduce the surge of surge will modify the industrial, very deep narcotic anesthesia. Very diligent control of your potassium magnesium, both during and afterwards. Eia and these are all steps, very small steps that are very crucial to not only to take the patient through the operating uh uh period, but exactly after the uh the because we, we do have Mark who immediately get to see the patient, the moment it goes into the uh intensive care unit. But that period, the post acute post operative is very important of patients who've had events during during induction. I think in the future, you'll see actual arrhythmic michael valve prolapse centers like pa places. That's what I was gonna my final thing. So you are, you, you've in essence developed that we hope so. Yeah. So I think that's important. That's an important thing. That's an important thing for the for the audience to know this. This is, is been really, really good and very informative of it. It's, you know, it's a, it's a lengthy discussion but a timely discussion I think really important. So, um you know, a compliment, you know, the colleagues at Mount Sinai who developed this and the knowledge is really, really important, some pretty dramatic knowledge. So, thanks, I've learned something. I hope that you've enjoyed this and learned as much as I have. And certainly, uh you know, now where you could refer patients if you need to, I do not making a pitch for a referral but, but a lot of knowledge and we it's really important because these patients uh I'm sure are extremely concerned about their risk. So, thanks. Thank you for you both are doing that. Thank you for joining us.
