Mayo Clinic medical experts in hypertrophic cardiomyopathy, Jeffrey B. Geske , M.D., Steve R. Ommen , M.D., and Hartzell V. Schaff , M.D., discuss the recent publication of the "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy." They review the top changes to the guideline, including the introduction of a cardiac myosin inhibitor, mavacamten, for support of people with symptomatic obstructive hypertrophic cardiomyopathy (HCM); the evolved guidelines for healthy levels of exercise; the formal split in sudden cardiac death risk assessment between children and adults living with HCM; and new advice on more frequent screening for individuals who have risk factors for atrial fibrillation. For more information, visit Mayo Clinic Medical Professionals – Cardiovascular Diseases .
Hello, I'm Jeffrey Geske, professor of medicine here at Mayo Clinic, joined by my colleagues, Dr Steve Almond, chair of the guidelines at 2024 update for hypertrophic cardy and Dr Hartzel Schaff cardiac surgeon with expertise in myectomy amongst other things. And we're gonna be discussing today uh the 2020 4h CM guidelines and management of patients with hypertrophic cardy. Thanks for joining me. Thanks, Jeff. Good to have you here. Thank you. Uh Steve, I I might start with you as, as kind of chairperson of the 2024 guideline update. Uh What kind of if you had to pick a top three or top four take home points, changes within the 2024 guidelines? What's what's the cliff notes that the, the high yield things that have changed with this guideline? Yeah, I mean, the the number one obviously is what prompted the rewrite of the guidelines was inter deduction of cardiac Mycin inhibitors, specifically Mava Campton. So that'd be the number one is where Mava Campton fits in therapy for patients with symptomatic obstructive HCM. We also evolved the guidelines we give to patients around healthy levels of exercise that are now encouraged in patients with HCM. Uh Next thing is we formally split sudden cardiac death, risk assessment of Children away from adults before they were in prior iterations were kind of in one big ball. And there's enough differences in the way you approach a someone under the age of 16 versus someone older than that, that we separated those in the guidelines. And then lastly, there's some new advice around maybe screening more frequently for atrial fibrillation, subclinical atrial fibrillation and patients who have risk factors for that. So those would be the four biggest things that I think with the changes knowing that that kind of the introduction of Mycin inhibitors has been the the trigger for this update. I'd like, I'd like to get both of your thoughts as to how uh NVA Campton and now another coming Ec Campton, how is this impacting care of patients with HCM? It makes the conversation longer. Um So, so it does impact care. It doesn't impact the beginning of care. I still think that patients deserve an opportunity to respond to beta blockers or the nondihydropyridine class calcium channel blockers because so many patients do respond to those medications and they're simple to use. But for patients that don't respond to those who can't take them for some reason, we now have another option, Maac Campton to include in what I put into an advanced therapies bucket to. It includes Sturm, Maac camp septal, myectomy and alcohol, sept ablation. So going through the pros and cons and logistics of all of those is part of the discussion and logistics. Tell me, dive into that a little bit. What have you, what have you seen? Yeah. So, so with me, then it came with what's called a REMS program or a risk evaluation and mitigation strategy. Uh because there is a small percent, 5 to 10% of patients who drop their ef when they're on this agent. And so the FDA required that we do frequent monitoring when we initiate mavi camp and we have to do it every month, an echo every month for the first three months. If they're on it, anytime you do a dose change and every four months or every three months during uh maintenance phase. So that's 4 to 7 echoes per year that these patients uh need to be willing to undergo and hearts is, does everyone need to try Mavic Campton before they head to myectomy? I don't know that everyone has to try it but everyone needs to be aware of it. And when you balance the, the option of septal reduction therapy with surgery or alcohol, septal ablation versus continued medical treatment, people respond very differently to that uh presentation. Some patients object to taking a medicine the rest of their life. And if there is an easier or at least a, a more certain solution for their symptoms, we'll choose surgery. Um Other patients have tried mac Campton and have not had the relief that they expected. Um, and then other patients are put off by the idea of continued close surveillance over the years. Yeah, I think that's right. I mean, we haven't really seen much of a drop off in our surgical volumes for Myectomy and all of the patients that are going to myectomy for all intents and purposes of the past two years have been offered mamin as part of their treatment plan and still are choosing to have surgery either because they failed therapy or for the reasons. Hartzel mentioned that they, they just prefer a mechanical solution to a mechanical problem and say you have someone who's tried NAVI camp and they're on therapy but maybe not getting the symptom relief desired. Do how do you manage that medication preceding operative intervention? Well, that's a discussion to have with the cardiologist and it depends on which of the drugs they're taking. But as I understand it, uh the Mamma Campton um should be discontinued for at least four weeks before operation. Uh, Steve. Do you have any? Yeah. So, so the concern would be in someone who's taking such a potent cellular level, negative inotrope in giving them carried. So we have recommended 3 to 4 weeks at least of wash out in the uh trials of Maac Campton, they actually had a longer wash up period than that. But in talking to the other high volume surgical centers that 3 to 4 a week is a minimum of time to have patients stop taking Mac Campton before they go to the, or, you know, I have to say when this first came about, I was worried it was gonna be so complex discussing, you know, which option to take. But I found a lot of times in office people kind of have an opinion. They either want less medications and want to opt for the procedure, as you said, kind of a structural fix for a structural problem. Others say, listen, if there's a way I can try a medication that avoids a procedure, I definitely want to do that. And the number of patients that are really torn between the two. It's fairly small. It has that been your experience. Yeah, I think that's true. I've got a, I've got a few that are wringing their hands over the choice between the two and often in those cases. We'll say, well, why don't we try the medication first and see if you get relief of your symptoms and if you do, we know you're gonna get that same relief with surgery, but you'll have more confidence going into the surgery if you, if you want to, I've seen cases go both ways. I have a particular patient who's had an amazing response to Maac Campton. I've also had several patients who have tried it and realized I'm not sure that I feel as good as I want. And then underwent operative intervention and felt substantially better. So, it's certainly a shifting landscape. Yeah, it definitely is. And patients are certainly more aware of new options. Now, there's advertisements on national television shows and those kind of things. So patients are coming in and informed about their options. But you're, you're right. Many of them come in with, uh, a preconceived notion of which one they would prefer if all else is equal. Maybe I could ask you to a question about the use of Mavica because as you mentioned, it's now being widely advertised. Is it something that should be used earlier in the conversation with patients in terms of medical treatment? And then secondly, do you, do you think it has a place for the patients who have a very hyperdynamic ventricle without much a tract obstruction but with symptoms just to treat that very hyperdynamic patient? Yeah, I think that that's a really good question. So I I think it, it can be used early but it shouldn't be used first. Again, there's simple medications that are beneficial. Now, if we ever get data to show that these agents are disease modifying apart from relieving outflow tract obstruction, then that conversation will change. And there's some hope that may be the case since it direct some specific pathophysiology in HCM, that it might have long term consequences. But I think that's going to take us a long time to get data on that since this event rate in HCM is so low, it's going to take many years of follow up to determine that for right now. It, it, it should come after failure of the other ones. But you don't have to go through this rigorous thing of making people go to some massive dose of beta blockers that makes them incapacitated otherwise, or stop their statins because they have to be on because calcium channel blockers interact with that. So there's a bunch of reasons why you can now introduce that sooner. What, what are your thoughts? Yeah, I, I would agree and echo that and, and maybe I'll tackle the second, the second question. Um I think uh it's certainly a hypothesis generating question. Could these be used as a target treatment for things beyond outflow tract obstruction? And perhaps we'll learn more about that as some of the trials looking at non obstructive HCM come in right now. The only FDA approved indication is for outflow tract obstruction and even the trials that looked at obstruction actually excluded mid ventricular obstruction. So those hyperdynamic patients that don't have a tract obstruction, but that mid ventricular obstruction, they were taken out but are being included in some of the non obstructed trials. I, I think it's exciting, we'll see kind of how this evolves right now. If you are choosing to target one of those patients off label, it'd be hard to know how to dose titrate because all of the REMS program and dose titration is based on outflow tract gradient. So I, I'm not sure how I would do that. Um, I, I don't have any specific patients that I'm doing that in right now. But uh I think as more trial data comes in it will be helpful. Yeah, I, I have used it in people that had multilevel obstruction, both outflow tract and mid ventricular or true obstruction to mid ventricle. But the ones where it's just complete systolic cavity obliteration, I haven't, I haven't tried it or thought about it in, in any of those patients yet. But I do think that the data coming from these next rounds of, of trials may be helpful in that regard. Well, maybe I'll, I'll, I'll let you, uh conclude with just 11 more question. Say that you're a practitioner, you're caring for someone with type of trocar that you're thinking about whether to initiate uh mycin inhibitor. Uh What kind of are there any pearls or, or, or lessons learned that you'd want that person to know as they're considering that therapy? Yeah, it's a, it's a great question. I mean, I think right now most of the prescriptions of Mava Campton are coming from HCM Centers because they have a large understanding of all aspects of HCM. Um, there are some smaller areas that are prescribing Mava Campton, but it ends up being complicated and in a busy clinical practice, it's hard to fit in all these clinical assessments. And evaluations. So I would still urge someone if you've never used a Mavic Campton for to, you know, refer them to a center where they can get a discussion about all of the options and then, you know, participate as part of the team taking care of that patient if they choose to go on Maac Campton at that point. And then from there you start to build experience, et cetera. I think that's wise considering, you know, uh informed decision making between the advanced therapy is something that I think, you know, high volume centers are going to be really expert at guiding and then even just some of the ins and outs of frequency of monitoring drug drug interactions. Uh and I think that's, it's nice to have that initial guidance with someone who's very familiar with the process. And, and again, just to remind you if you're, if you are thinking about prescribing Maac Camp, then you do have to register as a prescriber with that REMS program as well. So there's some education requirements you have to fulfill in order to be uh authorized to prescribe. So wonderful. Well, thank you both for joining me. I appreciate it. It's been great having you here as we kind of explore some of the changes and shifts to the landscape and hypertrophic karma.
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