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EP on EP Episode 96: Malignant Mitral Valve Prolap ...
EP on EP Episode 96: Malignant Mitral Valve Prolap ...
EP on EP Episode 96: Malignant Mitral Valve Prolapse Syndrome (Part 2)
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Video Transcription
Hi, this is Eric Prostowski, and welcome to another session of EP on EP. We're here with Dr. Sam Asivatham, and he's been discussing the malignant mitral valve prolapse syndrome. Sam, welcome back again. Thank you. You did a marvelous job, as anticipated, in defining the syndrome for all of us and the patients you worry about and how to screen for them. What I'd like to do now is say, okay, you have that patient in front of you. You now have screened, and they're now under your care. What is the role, and I'm going to go through four things, and you can handle them anyway. We can go back and forth. The role of drug therapy, ablation, ICDs, and surgery, and how we approach these patients. We can take it any order you wish. Yeah. So maybe I'll start with surgery. So there's two questions that come up with surgery. Is surgery itself antiarrhythmic, and can surgery be proarrhythmic? And I think the answer to both is yes, but in our own studies, about a third of patients who have the phenotype of malignant mitral valve prolapse, that is complex ectopy, a lot of ectopy, have maybe floppy bileaflet prolapse, some mitral annular disjunction. About a third, there's a significant decrease in the amount of ectopy after mitral valve surgery is done. And the particular surgery was both repair, which was in the majority of patients, and a few who had the valve replaced. The opposite is also true. There are a few patients who've not had much ectopy, but after they have surgery for the mitral valve, for example, because they had severe regurgitation, do get ectopy, even to the point where treatment is needed. And this is maybe about 5% when we look at just single-center databases. And it seems to be related to the neocords. If neocords are placed on the papillary muscle, perhaps the tension, the tightening around it may give rise to this ectopy. And a handful of patients who've had ablation procedures, the ablation wound up being on the papillary muscle, not too far away from where the neocord was inserted. So occasionally can be pro-arrhythmic. In a good chunk of people, it helps surgery itself. A rare but definite indication for surgery is patients with malignant mitral valve prolapse syndrome who've had a sudden death event, have an ICD, have frequent shocks, have had ablations and antiarrhythmic drugs, and still are getting malignant arrhythmia. And even if the regurgitation is not severe, those are patients I do recommend surgery to reduce the redundancy, repair the valve, and often at the time of surgery, cryoablation underneath the mitral valve, often in that disjuncted area. So those would be just thoughts on surgery, where it kind of plays out in the syndrome. Before you go on to another therapy, I've sent a couple of patients to surgery who had very severe regurgitation. They actually came to me because with PVCs, that's what they referred to me. And during the workup, they had a pretty significant murmur, you go do your non-invasive testing and they have four plus mitral regurge and clearly need to go to surgery. I've always wrestled with, do I need to do anything more once the valve is repaired? In this one case, I'm thinking of the PVCs became fewer and I'm just following him, but do you have a metric you use after surgery to decide if you have to do anything else? So I kind of look at, are the PVCs themselves a major symptom for the patient? So if they're frequent PVCs and that's really bothering them, I usually will ablate before surgery. The only reason is, you know, however careful we are, ablating near the mitral valve apparatus is not easy. And if we make the regurgitation worse or we snap a couple of cords, if they're having surgery anyway, then that can be fixed. You'd hate to do that, like after they just had surgery and then you have some scallop that's prolapsing again. No one's happy with that result. So I'll usually do it upfront. There are a few patients though we have ablated post-surgery, either because it's problem started after surgery or they just had continued to have episodes, especially VT. So if the surgery though, if let's say they weren't very bothered, I mean, the PVCs, let's say they were 10% and, you know, you wouldn't have maybe just gone and done an ablation. If you do the surgery in someone like that, Sam, and post-op, they have a marked reduction in PVCs. Are you comfortable leaving that person for operation? Absolutely. Okay. So I'm sorry to interrupt you. So, okay. We've taken care of surgery. What's your next thing? So the next one may be like, you know, who are the patients who have an indication for an ICD and who do we kind of at least do some shared decision-making? So easy one is secondary prevention. I mean, symptomatic ventricular tachycardia already observed, regardless, I think, if the patient has surgery, ablation, anything else, that would still be a clear indication for a defibrillator. Now, in the patients who've had observed polymorphic VT, but never passed out, maybe they felt it, but continue to do what they're doing, but it's polymorphic and it's not, you know, one of those two beats, three beats, but, you know, 10 beats, 12 beats clearly looks malignant and they're not going to get surgery, I give them the choice of like, you know, VT, we can do an EP study, ablate, see how much substrate abnormalities there are, assuming the MRI wasn't showing some, you know, rip-roaring gadolinium enhancement. And at that time, I specifically do ventricular stimulation from the mitral annular region to see, can we induce VT? And if despite that mapping ablation, we're still inducing VT, even if there's been no clinical event and inducing monomorphic VT, I do recommend a defibrillator. If they're still having substantial ectopy, let's say, I mean, you give them a defibrillator and you, and you know, one of the worst areas to ablate is the one you're talking about, right? It's hard to get a hundred percent. So how do you handle that? I mean, I know I don't want to get into totally drugs, but let's say you've decided to put a defibrillator. I get that. But now they're still bothered by their arrhythmia and you've done your ablation and they're still having some, do you just go keep at it or do at some point you give them a drug? I think it depends, like, you know, one is, if it's monomorphic ectopy and you might have just missed the right spot and I would ablate again. If it's polymorphic, kind of all over the place, I won't ablate again. Sometimes it's polymorphic, but it's still like a family. It seems mitral annular, it seems like one papillary muscle. I will try to ablate again in that case. But if it's truly all over the place, or we really don't think at the end of a previous unsuccessful ablation, there's nothing really fundamentally different we would do. Then I do think about antiarrhythmic drugs. There, a large part will be the age of the patient. If the patient has a defibrillator, had ablation, lots of ectopy. And if they're young, I'll usually try a drug like Sotolol. If they're older, like say late seventies or older, I'll usually do amiodarone. Okay. Anything else that we haven't covered in surgery as far as your general approach, do you ever use, you know, the old clinical saw was beta blockers and, you know, they still work in some patients who are not, I guess what I'm saying is not the malignant syndrome. There are patients who just have PBCs and in my experience, sometimes the beta blockers will work. Yeah. Yes. There are some patients beta blockers help, they feel better. And even if they're having PVCs, it doesn't bother them as well. There is a very specific cause of PVCs that I won't ablate, or at least won't go to ablation early. And that is mechanical contact PVCs. So that's just the floppy valve that's hitting it. Some of the clinical clues to that is patient will say the PVCs are positional. It happens more when they're dehydrated. And in the EP lab, when it's hard to figure out, we can, after transeptal, just physically lift the valve and the PVC stuck. Those are patients you really think, you know, maybe surgery is the best way to go. You just reduce the size of this prolapsing mitral valve. So Sam is, I know you have a strong genetics department there, you and Michael Ackerman and the folks. Anything new in genetics, family, I mean, you know, this can run in family. So I guess you do recommend at least test is evaluating family members, but anything in abnormal genetic things you've found? Yeah. I mean, of course, as you know, there's this many, many causes of syndromic mitral valve prolapse. You know, the Marfan's and mitral valve prolapse, Ehrlich's, Danloss, Pseudoxanthoma, elasticum. But in all of those patients, their prognosis probably is more from the fundamental syndrome. The mitral valve prolapse part would still think the same way. Is there complex ectopy? Is there disjunction? Is there late gadolinium enhancement? You know, have they had worrisome symptoms? Those kinds of things. But outside of syndromic mitral valve prolapse, there's also just familial with nothing else. No other phenotypic features of mitral valve prolapse running in families. Right now, you still look for the malignant features. It's not like this gene, as far as I know, this gene is malignant. This gene is not malignant. It will still be the phenotype and electrophysiology. Thank you, Sam. Well, Sam, thank you so much for educating all of us in this area. And thank you. I know you're just a lone electrophysiologist at the Mayo Clinic, some lowly-titled electrophysiologist. But of course, that's your personality of not being out there. You've done phenomenal work in the field, and I personally want to thank you for your hard work and how you've helped our patients. And thank you for being with us. Thank you very much, Eric. I appreciate the opportunity. Thank you.
Video Summary
In the video, Dr. Sam Asivatham discusses the malignant mitral valve prolapse syndrome, focusing on treatment options such as drug therapy, ablation, ICDs, and surgery. Surgery can reduce ectopy in about a third of patients, but can also sometimes lead to increased ectopy post-operation. The use of ICDs is recommended for patients with symptomatic ventricular tachycardia. Ablation may be considered for persistent arrhythmias post-surgery, with antiarrhythmic drugs like Sotolol or amiodarone being options based on patient age. The importance of genetics in evaluating family members for potential inherited conditions related to the syndrome is also highlighted.
Keywords
mitral valve prolapse syndrome
treatment options
ablation
ICDs
genetics
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