Episode 9 - What's New in the 2022 Aortic Disease Guidelines?

Join our special guest, Dr. Sagar Kalahasti from The Cleveland Clinic, for a practical overview of the recently published 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease.  Dr. Kalahasti, who was a co-author of the document, reviews practical tips for diagnosis, imaging, medical management, and genetic testing in common aortic disorders, as well his experience contributing to the guidelines.

Please subscribe to receive updated episodes automatically! Visit https://www.ohioacc.org/cardiohio-podcast/.

For more information, see the full 2022 guidelines, or this summary document of key points.

“Upbeat Party” by scottholmesmusic.com

Please subscribe to receive updated episodes automatically! Visit https://www.ohioacc.org/cardiohio-podcast/.

“Upbeat Party” by scottholmesmusic.com

Transcript

0:04

Welcome to the Cardio Ohio Podcast, a production of the Ohio Chapter of the American College of Cardiology. This is Can Greyal in Columbus, Ohio. This is Ben Allen Cherry Cardiovascular Imaging fellow from Cleveland, Ohio. More information on the podcast, including past episodes, is available@ohioacc.org. And now for today's,

Ben A 0:30

All right, everyone. Welcome back to the Cardio Ohio podcast. This week we have a very special guest, Dr. Ser Kah Hasti from the Cleveland Clinic, from the Advanced Imaging section, and an expert in aortic disease. Dr. Kah Hasti was on the most recent iteration of the 2022 aortic guidelines, which we will include in the show notes. Dr. Tallahassee,

Dr K 0:52

how are you? I'm doing great. Thanks so much

Ben A 0:55

for having me. Yeah, thanks for coming on. so Dr. Khasi, can you just give us a, a, a brief introduction about how you got to be an advanced imager and what led you towards specializing in aortic diseases?

Dr K 1:09

Yeah, perfect. So I consider myself a clevelander now because after my medical training in India, I came to the Cleveland Clinic back in 1998. And God, that sounds so long ago. And I came and did my internship residency as well as cardiology fellowship at the Cleveland Clinic. And I started working in general cardiology after I finished my fellowship. And during that time I was seeing a lot of aortic disease patients who were coming in for surgery at the Cleveland Clinic. And as a cardiologist, we see them all before their surgical or endovascular intervention. So that really picked my interest. As to, you know, this is something that we don't routinely see very much in our fellowship. But you know, it was like, man, there's so much of this disease that, that we are seeing in our clinics. So that's where my initial interest about aortic disease kind of started. And then slowly got into understanding the most common etiologies and the most common patients. We were seeing a lot of. Genetically mediated connected tissue diseases such as Maren or Louis deeds. Louis deeds was not even that well defined at that point in time, but was thought to be something related to Maren. And so, so they kind of went hand in hand my interest with aortic disease and understanding the most common ideologies. And then that got me married into what imaging do we commonly use in aortic disease? and said, oh, ct. Of course yes Echo is used, but CT is like the standard of, evaluating the entire aorta and both in the urgent situation as less in an elective follow-up situation. And then, so then I realized I need to get more skilled with regards to being able to read ct to be able to care for these patients better. I think that's

Ben A 3:02

perfect for our listeners to understand kind of how you got to the advanced imaging part and the interest in aortic disease. And, and for further background, we'll be referring to the 2022 A C H Aortic Disease guidelines. So I kind of want to jump in and answer some of the questions that are very practical for our listen. And the first one we, we just wanna talk about is stable thoracic aortic aneurysms and when to go beyond echo to CT or m r.

Dr K 3:30

Yeah, that's a great question. I think in general, my approach has been to at least get a baseline CT or an M R I, depending on the age and gender of the patient and what history are they coming in to see me with. And that always determines the next steps. And I almost always. Some kind of cross-sectional imaging after the initial echocardiogram, just to make sure that the measurements that we are getting on an echocardiogram closely correlate with the tomographic imaging. And after that, if, depending on the size, if this correlates well, and depending on the initial size, when we are seeing a patient, the, time of follow up. Most often, if it is something which involves primarily the aortic root and the ascending aorta, I generally tend to stick a lot with echo. And that also helps me looking at the left ventricular function, looking at their aortic valve anatomy, looking at aortic valve dysfunction, whether they have, either if it's a bicuspid valve or they have stenosis or regurgitation. So echo is like my primary go-to modality in the majority of. But at least I get at least one baseline tomographic imaging, whether it's CT or m r i in everybody that I see and so having another imaging to clarify the original, you know, dilation or aneurysm change is very helpful in my.

Kanny 4:52

So saga, this is in Columbus. I wanna just echo Ben and welcoming you to our podcast. A follow up to that question is serial or serial surveillance, you know, Most of us in clinical cardiology now have a lot of patients who we follow, who many have just had an incidental diagnosis of thoracic aortic enlargement or aneurysm. We know there's guidelines out there regarding the frequency of surveillance. I, I, I also know in my practice I have some elderly, frail patients. We have patients we're reluctant to give contrast or, or excess radiation. So, so what factors help you tailor the intervals for serial imaging to a given patient so that maybe we can try to, minimize imaging in patients who don't need it as intensively as others.

Dr K 5:39

Yeah, great question there, Kenny. So the, the way I think about it is that, you know, what is the initial size and what is the rate of change of that particular aortic dilation? Let's say, for example, the first time I see a patient there. Aorta is 4.3 centimeters. And, and we do a cross-section imaging, for instance, let's say CT and M R I, and it is 4.3 centimeters. Then one of the things that I've learned through practice is that patients are extremely nervous the first time you meet with them because they've already read this up on. Google and then they all come with this idea of a ticking time bomb. Right. So in general, I have, you know, always tried to see them for, for an initial visit, whatever the initial measurements are, within about six months, because just to reassure them that this is not what they're thinking, you know, in a realistic fashion, the risk of something bad happening to them. The other point I cannot emphasize enough is that to bring any. Old imaging that they ever had, you know, whether it's an echo or a ct because the indications for the previous imaging may ha, may not have been looking specifically at theor. But having that imaging is really helpful in, and it goes a long way in reassuring the patient that you know, hey, something was actually there five years ago. That's a tremendous amount of relief for the patient. So that's, that's the way I think about it. So just to add to your question about, you know, older and frail and those kinds of people, we don't actually always have to give them contrast, to be honest with you. You know, there are lots of times that we do a cardiac CT without contrast, and if you're purely measur, Dimensions. That alone is sufficient. It's very different if somebody has a dissection. You wanna see what's happening with the true lumen and what's happening with the Folsom and their contrast role is much more. But if you're purely looking for a size, Of the aortic root reining aorta, you could actually do a gated non-contrast CT like we do for a calcium score, and you should be able to get the dimensions without worrying so much about contrast. The next point is, how about radiation? Right? That's that. That I think is if, if it's somebody is a younger patient, like in the thirties to forties or fifties, you're thinking of their lifetime risk of radiation, and that's where I think having Echo as your main tool helps in minimizing that exposure to radi.

Kanny 7:53

I think the key message is, you do want to tailor the surveillance plan to the patient and their age and comorbidity and I, I think it's a great reminder that we don't have to use contrast. In every situation. I know we have some of our imagers here who also will sometimes even do MRI without contrast. For the same reason, just to turn attention to medical therapy briefly, I know there's not a lot of literature, at least evidence-based literature about specific medical therapy, but I know in these guidelines they do kind of point out, especially in marfa patients more than others, there clearly is a role. You know, beta blockers or a c e are there any tips you have in your own practice for initiating the meds or even deciding when medical therapy is needed in these patients?

Dr K 8:38

Yeah, it, it really is a good segue into talking about the medical management. I think beta blockers have been like the gold standard that's been established for many, many decades now. And I think more recently ACE inhibitors, yes. But I think there's been a lot larger role for the angiotensin receptor blockers based on a few studies that have been done specifically in MAR and patients. And, and the pediatric heart study published many years ago in New England Journal Medicine and. Multiple other meta-analysis that looked at other ARBs combined. You know, something like Herbi Sartan have been looked at in, in Morphin patients specifically as an extension of that benefit in those patients. You know, I routinely use Losartan in other patients, so. As an initial treatment, I think it the most important thing to look at is that, you know, what is their initial presentation? I see a lot of patients who are quite athletic and this was found on let's say a calcium score, for instance, and they're otherwise performing very well in their exercise capacity is very good. Younger age patients. I generally, I'm a little bit careful about starting them on a beta blocker because some of them are already bradycardic. They come in their, you know, fifties heart rate or younger patients in their twenties or thirties sometimes, you know, you worry about the side effect profile of a beta blocker with, you know, fatigue and aile dysfunction. So I, I, I take those factors into consideration as to which would be my, my first drug in the patient. And definitely I, I use angiogenin receptor blockers quite frequent. And most of the time it's either Losartan or Herbi. Sartan because those have been studied. Are there patients that have to use more than that? Yes, there are a lot of patients who have purely hypertension as their major co-market condition, so I always incorporate these two medications. And in addition to that, they may need to be on others too, like going back to our usual hypertensive meds like a. Like a you know, diuretic based regimen or calcium tunnel blockers or diac baso. So I have used all of those, but in general, the two most common meds that I use is beta blockers and angios and receptor blockers. And I, I titrate them to the maximal doses possible before I start any other, or third medication or anything like that. And Dr.

Ben A 10:50

Khasi just a practical point, if you're, if you're seeing a patient who is on an a. Do you actually try to switch them to an ARB I saw the strength of recommendation isn't, isn't terribly strong, but yeah. What do you actually do in clinical

Dr K 11:03

practice if they're tolerating it very well? Ben I have not routinely changed it. I have continued it in in those patients, but if never been exposed to any prior medical treatment, then in general I have gone to an arb. Okay.

Ben A 11:17

Perfect. So, I. That's probably probably the, the limit of the medical therapy. Now, if you get a patient in your clinic who, who has a new diagnosis, and you have to kind of traverse the waters of genetic testing, I know this can be a podcast in and of itself, but a lot of the recommendations about, you know, identifying the proband and then going down through cascade testing are somewhat nuanced. But is there, do you have a, an easy f. For genetic testing in patients who first we can start with in relatives of patients, and then maybe we can talk about a patient who comes to you with no diagnosed aortic disease, but maybe their relative had it. So do you have a framework that is kind of simplified for the audience?

Dr K 12:03

So I, I think, you know, if you are seeing somebody who is, you know, below 50 years of age, for instance, and they've been diagnosed as having an aortic dilation for instance whether they got, you know, some kind of a screening test, whether it's lung cancer screening, or they have had you know, calcium score or something like that in those younger patients family histories, the probably the most important thing that you can get during that first. Is to dig as deep as possible in trying to explore what their family history is. And if there's any other first injury relative with, with a family history of an aortic disease, I generally send them to genetic testing. And that involves actually not just the testing part, but just actually meeting with the genetic counselor. Because sometimes things that, we don't really think as being, you know, important in a family history. Genetic counselors are extremely. In trying to dig a lot, lot deeper into. again, another important factor, sometimes you get patients who've had a relative with a, with a sudden death and then they're diagnosed with an aorta, you know, which is like borderline, like 4.1 or 4.2 centimeters. There also, I tend to be much more proactive, keeping in mind that we only know, yes, the genetic testing panel now has expanded from, you know, from a few genes many years ago to now we almost test for 38 to 40 genes. The way I think about it is that I feel that most of the genetic, most of aortic disease, genetic, it's just that we have not found all of the possible mutations that is actually causing people to develop genetic disease or, or, or aortic disease. So it's if somebody goes through the pathway and, you know, meets with the genetic counselor and then they get their genetic testing done, let's say their panel comes back as. That doesn't exclude that they don't have a genetic disease. So there the next step is both simultaneously happening, not only the genetic part. Also, I'd recommend all the first degree relatives to undergo some kind of imaging follow up. You know, whether it is echo to start with and if there's any abnormality and echo, then escalate it to. A CT or an M r I. So it's, the way I think about it is just like two different pathways. One is to look primarily at genetic testing, but also simultaneously have their first year relative screened for aortic disease.

Kanny 14:18

So so sagar in that, in that framework. Then pa most patients above 50 who have, more say hypertensive associated aneurysms it sounds like genetic testing will still not be used in a majority of. and that you would focus more on maybe first degree relatives when it does look like there's a, some kind of familial basis.

Dr K 14:38

True. Let's say the only exceptions for that Kenny, is that let's say, you know, a, a 60 year old man comes in and he says, you know, my brother recently had an aortic dissection, and I am here for that, and I'm worried about my children and worried my grandchildren. So again, it just comes down to an individualized decision making beyond that age. But I don't exclude, I don't say that, oh, it's not gonna be a value because I have seen some patients where we diagnose them as having these familial, familial, aortic, you know, aneurysm pathways or now it's being called as heritable, thoracic aortic disease. And, and those patients have a family familial pattern of genetic mutations where they don't have a phenotypic feature like amarin or low deeds, and their only manifestation is aortic dissection. But in general, I think that if somebody is younger age, then I think there is probably a little bit better role for for genetic evaluation.

Ben A 15:35

Yeah, that's, that's great. Dr. Cal Hasti, so to distill it down, it's, it's depending on age use imaging plus genetics to kind of guide you in the screening process. The family

Dr K 15:44

history of the patient and families has one of the major.

Ben A 15:48

Perfect. And, and kind of leading from genetics and screening of relatives to now management. And I think one of the main recommendation changes in these guidelines was the threshold for surgical intervention for root and ascending aortic aneurysms. from 5.5 to five and even lower if you have some specific heritable aneurysm diseases. From your experience at Cleveland Clinic as well while being on the writing committee for the guidelines, what has spurred this, this change?

Dr K 16:22

So I, I think just broadly speaking so I think the things that have changed is that, you know, we have got other tools rather than just the aortic size itself, right? So we're now indexing. The patient's aortic size to their, you know, if you take the cross-section area of the largest segment of the aorta to the patient's height, or we are doing an aortic height index. So I think that's, there's been some refinement with regards to risk ratification of these patients. The second part is the, the low risk of surgical intervention. Right? So that's the second. that has changed you know, over the past decade since the, since the first guidelines came out. And then the third part is that the long-term outcomes of elective repairs have also been significantly better particularly like a valve sparing aortic root replacement. You know, for maren patients when the fir, their first surgeries used to be always like a bental type procedure. With a mechanical valve because most of these patients were younger, so we were committing them to lifelong anticoagulation. So along the wave when the valve sparing root replacement came, And people wanted to wait to see what their long-term durability is. And it's been very good. I can speak in, in, in centers of excellence at Steven Clinic, the, the likelihood of reoperation for a valve sparing root replacement remains very, very low at 10 and even longer follow up. So these constellation of factors have really helped us in, in defining, you know, are those patients, are there patients that we should be intervening on? Right. So that's, that's, that's the way I think about these things, and that's where the thresholds have come down. The other thing also is the, the role the imaging plays because you know, you are able to get excellent imaging and you can easily understand the differences between studies and how quickly something is changing. And we incorporated that by, by giving a proper definition. As you know between studies, sometimes you can see a significant variation in measurements and that can create panic. And I think that was one of the things we made sure that we put it into the guideline document, is to look at what is a rapid growth, you know, what would you consider as a rapid growth? And, and one of the things we clarified is that if it's more than 0.5 centimeters, in one year, that should be considered as a significant change. This, I'm not talking about dissected aortas. This, I'm talking about intact aorta, right? So, and then the other thing is that if you have consistently 0.3 centimeters over two years then that's also considered as a, as a significant change. So when you put all of these things into consideration, I think you know, surgery at the lower threshold seems very, very reasonable. What never makes it to guidelines is patient. And patients wish to have something done right. Because some people have had their lives appended completely from the time of diagnosis and despite your best efforts, so I think the guidelines did, in my opinion, we had a very robust discussion about all these things and we, we wanted to give a little bit more pragmatic and more practical approach. And saying that, you know, if you go to a big center of excellence, it's very reasonable to be doing at smaller sizes than just saying, it has to be a certain level for, for you to have a surgery.

Kanny 19:34

And I would echo that, you know, in my 20 years of practice, it's amazing the spectrum of patient you see from those that want to jump right on something and get it fixed immediately to those who wanna avoid anything if possible and are willing to accept some uncertainty. So I'm really glad these new guidelines do offer some some nuanced, criteria that can be individualized. Cuz I think sometimes we didn't always have that. since you do talk about your center there a little bit, one question that always comes up is when does a patient need to be referred to a center of excellence or a heart team? Is it once they actually meet the criteria for surgery or intervention or. do you feel like there might be patients who are just being monitored serially that could still benefit from establishing a relationship at a center like yours?

Dr K 20:17

I think depending on depending on the initial size, when they start with, let's say, you know, they're, they're seeing their local physician, local cardiologist and their size has been very stable at like, say 4.1 or 4.2 centimeters, I think they can continue to follow with their physician. But anybody with, with rapidly changing or significant family history, or somebody who has, who wants to have a surgery at a smaller diameter, for instance, you don't want to, have them undergo surgery at smaller volume centers. Because, you know, again, going back to that point about valve sparing, you don't, you don't wanna. In a center where the surgery gets done, when they get their valve replaced, when there was no need for the valve to be replaced. So, so all those can be, can be important factors to consider. And some, some patients do prefer to come and establish with, with the surgeon and they just wanna see a surgeon talk about it. Just to kind of understand what's, what's coming down the line and, how can, how well they can prepare for that. So I, I think just to give a broad points, I probably would say, I mean of, of course if somebody gets diagnosed the first time, it's at 5.2 centimeters. We would like to see them sooner than later. But if those patients who are, let's say 4.5 to five centimeters, they could, they could come and see us for, for an initial evaluation and establishing with somebody and they can visit us maybe once every two years if there is, if, if there is stability.

Ben A 21:34

Yeah, that's really helpful, Dr. Khasi, speaking in the management, we've been kind of focusing on chronic aortopathy, so to speak, or thoracic aortic aneurysms. Now, just to switch a little bit to acute airto. And looking at the guidelines, not much has changed as far as like type A dissection. Mm-hmm. but I, I do want to kind of get your thoughts on, on how you put into some sort of mental framework, the, the intramural hematoma and the penetrating aortic ulcer and how they kind of go hand in hand with Type A or atos.

Dr K 22:10

So as far as the type A, the classical, you know, dissection and involving the, you know, ascending, you know, they still go to surgery, right? So that's, that's, that's a standard acceptance. I would say the same thing applies for type A intramural hematoma. So they would also should go to surgery. As the initial management. In general, we don't see so much of of an ascending penetrating ulcer, right? So it just doesn't happen as commonly, just purely based on their embryologic origins. I think they generally tend to ha occur more in the distal large to descending to abdominal aorta. We do see a lot of incidental penetrating ulcers. In a lot of patients who go through ct they have very atheros. Descending aorta and abdominal aorta. You see these valleys and, and you know, peaks inside their aorta with some undulating type you know, atherosclerotic plaque. So those patients, you don't necessarily have to think about surgery, but they can present acutely, particularly if they come with an arch rupture or a dis descending rupture. So that's, that's where you know, endovascular stent treatment would be very helpful in those patients. So if you. Again, if you look at the Type P dissection, I think one of the things that the new guidelines have done is, is put an an increased roll of endovascular approaches because I think that's, again, another area that has really changed a lot in the last decade. And aortic specific mortality is actually better in those patients when you, when you intervene with with an endovascular stent early on. So to go back to your question, so I think if it's a type A intramural hematoma, the treatment is still surgical. And in general we don't see penetrating ulcer there. But if somebody presents within penetrating ulcer, rupture in the or type B dissection, then you generally tend to look at. An endovascular approach to those patients along with your, the medical treatment is still the same, right? You still do impulse control, but, but what strategy you use depends on depends on the location of that. And I just wanna add that there is more based on some of the studies that are actually done in the clinic by Dr. Roselli and their team is to do more extensive repair. At the initial surgery itself which is some, you know, something that has been pioneered here, which is the branched you know, single anastomosis, frozen elephant trunk or a be safer approach where you do an ascending aortic and arch repair and then you do a descending repair at the same time. While taking care of the arch branch vessels, coming from the from the acute situation. So that's being studied now. So we will know probably five, 10 years down the line, did we make a meaningful difference with regards to their long-term aortic remodeling. I think that's that's definitely something very exciting to, to look, look forward at the same time in if, if the patient goes to a smaller center, right? So not everybody can get to a large center. There is still role for and limited. Is sending aortic grafting as a, as a tool to make the patient survive. So your patient selection becomes important as to who is that patient that you can do more extensive repair compared to which patient you wanna do a limited repair, and also which center you are in. Yeah. That's

Kanny 25:19

very insightful. Thank you. So is the endovascular approach, even within thoracic aneurysms, is that continuing to grow at your institution? And what do you see as the future for endovascular options versus surgery?

Dr K 25:32

So I, I think it, the field is definitely moving, moving towards more towards the ascending Yoda. So there are now branch devices that are that are being made by many companies looking at arch replacement with, they actually have separate branches coming from the native graft and how you can use those to, you know, treat the arch branch vessels at the same time you're fixing the aortic arch. And Dr. Relli did a trial looking at emergency for. Patients who are not operative candidates for a Type A dissection. There's a trial that looked at the feasibility of doing an emergency stenting in the acute descending dissection patients. And the initial results seem pretty promising. But again, you know, unless it's applied in a wide variety of groups and, you know, long-term follow up, up and, you know, the way I think about it is like, it's like tavr, right? So when the TAVR first came in, they, they. Tried in people who are inoperable, and when you establish the safety and feasibility, then you go down to the next level. So I think the same kind of. Innovation, I think is gonna happen with type a aortic dissection where we can actually treat more patients who are not operative candidates, that you can actually do an endovascular repair initially just to allow them to recover. And if their health is otherwise okay, then you know, maybe you may be able to use that as a bridge to more definitive repair. For.

Ben A 26:53

I just wanna say thank you for, for everything. You know, we talked a lot about the genetics, you know, stable disease, acute disease, med treatment, surveillance, and I, I want to thank you because being a guideline author on, obviously a very influential document is is an amazing feat. I'll just remind everyone that the 2022 guidelines we'll have in the show notes, and it talks a lot about other things as well, such as pregnancy and aorta disease. So make sure you reference that. But just since we have a, a primary author here, Dr. Khasi, what was that process like and and how does it feel having a finished product there?

Dr K 27:32

Yeah, it's actually very humbling and, and, and very honorable you know, experience in the sense that, you know usually guideline authors are you know, people are considered as experts in the field. It doesn't always have to be research. It could be very clinical, and they are, their peers recommend them to these guidelines. And that's, so I got recommended. Some of my colleagues who are a ACC C committee members, and that's how I got the opportunity to participate in this guideline. And what I've, what I will always tell is that even though the process sometimes is time sensitive and time intensive, It's a humongous educational opportunity in the sense that you get to talk to so many experts in the field across the country, and they all are doing great work. And to listen their perspective from, from their institution standpoint is, is very enlightening. Although we want every patient to be treated in a big center like Cleveland Clinic, so the guidelines. A more document that anybody across the country, not just in USA but you know, across the world, will be able to look through these things and say, does this make sense? Is this pragmatic or is it just catering to the, the, the you know, only certain institutions where we cannot reproduce these things. So I, I think that has been the most valuable experience. And, and I, I, I'm very happy and thankful that I've got the opportunity to participate in a document like this.

Ben A 29:03

Yeah. Dr. Kasi, just wanna say thank you so much for, for all your contributions and thanks for sharing both the factual parts and also just your experience cuz it's a, it's a pretty awesome feat that that you just accomplished. So thanks a lot on behalf of my co-host, Dr. Kenny Greyal, and we appreciate you coming on the

Dr K 29:23

podcast. Thank you so much for the opportunity and please feel free to reach out me if if any questions come.

29:29

Thank you for joining today's podcast. For more information about the speakers or the topics, please go to Ohio acc.org,