Penn Aorta Center cardiac surgeons Dr. Joseph Bavaria, Dr. Nimesh Desai, and Dr. Wilson Szeto discuss the management of bicuspid aortic valve disease and associated aortopathy, including natural history, aneurysm prevalence, treatment guidelines, and imaging classifications; strategies in young patients, including BAV repair and Ross procedure; and the role and challenges of TAVR in treating bicuspid aortic valve disease.
Related Links:
See Dr. Bavaria's physician profile
See Dr. Desai's physician profile
See Dr. Szeto's physician profile
So yeah. Mhm. Okay. So hello to our audience. Um my name is joseph bavaria uh from penn medicine and I would like to welcome you all to a webinar that we're uh putting on tonight called management of bicuspid aortic valve disease. It's a root, not a valve disease. Uh And um this is put on by the pen aorta center and uh I'm joseph bavaria, but also our two speakers besides myself are domestics I uh and Wilson Zito, um all of us are cardiac surgeons in the pen Award A center and we'll be discussing a couple of different um areas. It is my pleasure to present this from the pentagon to center where bicuspid aortic valve disease, both on the valvular side as well as the aortic side is a very very important part of what we do and also very important for basically the entire population of the world as it is a very very common disease. So we'd like to explore some of the new uh challenges, new ideas and new operations and treatments that we presently have. So at the first uh uh speaker, so it might as well go ahead and get on with it. Our first speaker is dr desai uh and he's going to speak to us on the natural history of bicuspid aortic valve disease, aneurysm prevalence treatment guidelines and imaging classifications. So thank you know, mesh uh looking forward to your talk. Thank you joe. It's a pleasure to be speaking to you about something that is such an important part of the program. The aortic program here at Penn and I'm going to provide a background uh set of information about how we tend to think about custody work, valve disease. Uh some of the challenges that we uh see with our patients and management uh and then afterwards Dr bavaria and Dr Zito. We're going to go over some of the cutting edge treatments that we currently have on the management bicuspid valve disease. So the bicuspid aortic valve we titled this session. It's not just a valve, it's it's a root problem and that that's really the case and hopefully in the next couple of minutes I'll show you why we think about the bicuspid in terms of the root complex and not just in terms of the valve leaflets, because it it really does involve the roots so intimately. Um About 1- 2% of the overall population has a bicuspid valve. It is a complex familial inheritance and it has a male predominance of 2-3-1. So it's such a bit more than 2% most likely of the male population, in less than 1% of the female population. Um there is uh some degree of in heritability to it. There's a 9% prevalence in first of your relatives and it's also associated with other cardiac uh and syndrome. Ick issues including cohabitation, turner syndrome and lowest eat syndrome. So it can be associated with other major cardiac issues as well. Importantly, 20 to 30% of bicuspid patients will eventually develop an aneurysm within the tent within 10 to 20 years after their original diagnosis of a B A. V. Whether that was from symptoms or from a screening echo or or from hearing a murmur. 80% of patients with bicuspid aortic valves will eventually develop a New York diameter greater four cm. So that is actually really common and 80 times higher than the rate in the general population. So Bicuspid patients make up actually more than half of the young patients who have aneurysms in their aortic group that end up needing surgery. So by custom valve itself is fairly prevalent for congenital heart lesion, but aneurysms are quite common in this patient population. And the conceptual framework of why this happens is not fully understood, but we know that when the heart uh tube folds and then eventually the trunk except dates into the pulmonary artery and the aorta, that the, the tissue that eventually forms the aortic valve, These cushions that grow out into both the aortic and pulmonary valves, areas as the trunk cept eights uh are controlled by a lot of different mechanisms that we know are actually associated in different ways with eric aneurysm like TDF beta Like Notch one. and so we don't fully understand it. And not only the small minority of patients with my custom valve truly have a genetic syndrome, but somehow or other these genes are that eventually to bicuspid valve also are associated with um the development of aneurysm. And there's a couple of different theories on why aneurysms occur in patients with bicuspid valve. I think the one that is probably predominant today for being a larger part of it, although I think the jury's still out on that is the flow needed. A hypothesis that is that flow very abnormal flow coming an abnormal angle out of the aorta hits the wall and causes some kind of mechanical transaction problem that then leads to changes in things like mm. P. S. College and matrix, all the different stuff that holds the aortic wall together and gives it strength because the aortic wall is not a pipe, it's a very living organism um that is very can change very rapidly when under adverse conditions. And these are just some of the different basic science studies have shown changes and things like the last in because of the cheer stress of the ejection of the bicuspid valve against the wall. Here's some theoretical pictures of how swirling flow through these bicuspid valves can cause aneurysms and different types and different locations. And some computational flow modeling as well showing the different swirling of blood. And the different stress is placed on the aortic wall that may eventually to mark aneurysm and that all seems really theoretical but I want to show you a patient of ours. This is a patient who we operate on recently who had a ross operation for this unique cost aortic valve and take a look at the MRI images and just look at the jet of blood as it hits the aortic wall. This is very abnormal. And it's exactly in the area where you see the aorta bulging out in the still picture. I think that's as we learn more about this is clearly the flow jet through the bicuspid valve has something to do with aneurysm formation, although there's more to it than that. We also know that those neural crest cells, which is, you know, part of the the original embryo logic cells that create the ascending aorta sinuses and the aortic valve are somehow implicated in this as well. And potentially the aberrations and signaling that led to the development of the bicuspid valve may also lead to intrinsic weaknesses in the aorta due to, and mps are smooth muscle cell problems. And many of the other things that we know are associated with the aortic aneurysm in terms of actual patterns, typically the holy orders dilated in probably the majority of patients who end up having aneurysms. Um uh the but they do break down into sort of morphine a types than that. And we tend to see three different groups. One is patients who don't have root involvements with super coronary one group which we call Marfa annoyed or Rufina type. This is a very dangerous genotype. These organs actually behave much more like Marfan syndrome. They're more likely to cause aortic insufficiency dissect or have other aortic complications. So there they're ones that we tend to operate on early. We take very seriously. Uh And the tubular kind where the entire aorta is dilated. Um And this is just some examples of what these look like. And that route peanut type can look pretty scary looking. Um And very Marfan like um in terms of an atomic classification systems, there is a lot of different ways you can classify the valve opinion why you are. But this is one that we typically this is sort of our standard true bicuspid. So there's no ralf a there's two equal sized leaflets. The that's type 000 Seaver's one which is your standard fusion of two costs usually the right left. Um uh picture here showing it right now and it's actually pretty rare but usually the right left with the raft A. Uh And then the third one which is uh less common but actually you know, we see it quite often younger people which is a unique type variant where you have multiple points of fusion. Usually at least two Raffaele's and a slip like orifice. And typically we see those present in younger patients. Um and then as the patients get older we see more root genotype aneurysms and aortic insufficiency in the 30s and 40s. And then patients over 55. Typically we see more of the classic presentations to notice with or without aneurysm. So what happens to discuss my patients over time? Well, this is the Michaleen a series from Olmsted County. So a service population screening echo study where they looked at what happens to patients with asymptomatic patients who got an echo done purely for screening purposes. What happens to their, what happens to them over time? So, well, the good news is that 20 years, their life expectancy is the same as it would be for the general population. So that's fantastic. We can get by custom patients through all of their different cardiac problems pretty well with good survival. But if we look at What happened like what it takes to get people there, about 25%, maybe even 30% of patients within the first 15 or 10-20 years after their diagnosis do end up having surgery. So these are patients who have zero symptoms were found to have a bicuspid on a screening Echo as a population study. Um and and that's the group that we see coming back, You know, somewhere between 25 and 30%. Um and importantly, if there's anything really wrong with the valve structurally other than bicuspid, like it's thickened, there's calcification or this problem with mobility Actually within 10 years, almost 80% of those patients end up having surgery. So it is common in those patients, even when they're totally asymptomatic, be a v disease does progress very rapidly. So in terms of screening patients and their families with the a v Firstly we generally if we diagnosed an adult patient with baby do recommend screening first degree relatives. It's a class to be indication currently but um uh you know I think the likelihood is high enough that we do find a lot of baby and families. Um and then serial follow up is recommended either with C. T. Or echo or M. R. I. If on the initial screening um Test the word was greater than four cm. Uh And there's a lot of different protocols that we use depending on how large it is and how it's growing over time. But typically we'll try to get at least one C. T. In the first few years after the diagnosis. And then follow up with either echo MRI or ct imaging annually or every couple of years depending on how large the aorta is And you know in terms of making decisions about when to actually operate on the order. This is kind of classic data on when aortic aneurysm tend to rupture not specific to be a v. Uh and this is more current data it really shows that around 5.2 cm regardless of where the three leaflet or two. The risk of the order complications goes up a lot. And and just to put that size in context it's just a little bit smaller than cocaine which is actually huge. Think about having any order. That large family history weighs in on that and if you have a family history of aortic dissection in particular. We recommend earlier interact intervention and obviously all these incredible groups of genes that may be implicated in aneurysm, weigh in our decision, but when to operate as well. We also look at height. Uh so indexing the patient, not just not just looking at their opposite sides but indexing it to their height, uh indexing it to the length of their aorta, not just the diameter. Um but in general, you know, the guidelines today are fairly straightforward. Um we recommend operate on most people buy custody, aneurysms who don't have valvular disease at 5.5 if you have risk factors which include things like cohabitation, family history, rapid growth um we go smaller than that down to five at the time of your valve replacement. Typically get in at 4.5, But importantly, also between five and 5.5 it is recommended to have surgery if you're at a new york Centre of excellence. And the reason is very simple when you're adding a rec center of excellence and I've highlighted where penn would be on this continuum. The risk of the surgery is actually incredibly low That although there are only a few centers that do more than 400 aortic operations per year that when you have surgery at those centres, the risk of a bad outcome is actually under 1%, which is fantastic. Yeah. And just some of the highlights of some of the things that we're working on to help guide our operative strategies here is Alison pouch who is uh imaging scientist who works with us is developing ways to actually recreate the operations we do or even create them before we do them in the lab. So we can look at these images are actually almost surgical. In terms of what we're seeing reconstructed from echo or cat scans, we can actually plan out and even try different types of surgery in a three dimensional model before doing it on a patient. Um And this is kind of what it this is how good it looks. And this is actually like seeing a real by custody aortic valve opening and closing in real time. And just to put it all together again and set the stage for for joe and wilson. You know we have our own sort of algorithm of when to jump in on different types of strategies to repair or replace the aorta. Typically we are going for repair first, strategy for eric insufficiency for replacing. And young people ross is where possible or placing large mechanical or tissue roots. And as you get older we go more towards replacement with bio prosthetics. We are aggressive about screening relatives and in particular aneurysms and young people are going to be very pathologic and should be watched very carefully and I'll hand it to dr bavaria next. Okay um So exit. Thank you very much. Um So uh I just wanna uh well first of all thank you in a match for that. Great introduction uh And um I'll share screen and go to the next talk. Um So let me share a screen here. So our next talk is my customer valve disease strategies and young patients. And I'll really be focusing mostly on baby repairing the ross procedure. So what about presentations and young patients? Less than 50 years of age? Well this is our nightmare. We want to definitely not have a type a aortic dissection. We need to avoid this outcome and we also need to avoid this outcome. This is myocardial fibrosis recently presented and understood in able to search an aortic stenosis patients and they don't do very well either if they start getting amount cardio fibrosis in the heart muscle. So um the first kind of area and we're gonna look at two areas is aortic valve insufficiency in patients with young patients of bicuspid valve disease. And really what we see here is we see three major presentations. We see baby with A. I. And a relatively normal root diameter. Such as this where you have a pretty normal route, a pretty normal ascending aorta but you have this very large dilated annuals and you can see the numbers there in this particular case. We also have a b. A. V. Presentation of ai aortic valve insufficiency, relatively normal route diameters but in a sense aortic aneurysm and that's what this looks like. Where you have be a V. A. I. As you can see here. But you have a pretty normal route and you have a large descending aorta after the sign contributor junction. And here's another example of that, a little bit more extreme with a large aneurysm. But really a pretty normal route. This is a B. A. B presentation. This is what the message was just talking about regarding the a setting for genotype. And this is a is an expression of what he was also talking about regarding the heart field and how these aortic roots and a orders are developed. And this is an extreme example of sino tubular definition or a cleft in the kind of em biological cleft. We have a normal route as you can see here in his B. A. V. But a gigantic aneurysm right after the S. T. J. Uh and this is definitely an embryo logical uh kind of manifestation. And then of course we have the last which is B. A. V. With ai and root dilation or aneurysm. And this is what this would look like. You have a classic right left Bicuspid aortic valve as you can see right here followed by this eccentric aortic valve insufficiency and a 5.1 centim aortic aneurysm. And here's another picture of the same type of thing with a 55 millimeter york and the gold standard operation that we do for these in the past is uh a mechanical composite graft that you can see here with a hemi arts in this case or a biological mental or bio mental. But we also have new operations at this point which is and new therapies, which is what I want to try to get into. So this is kind of a picture of a of a B. A. B. Practice and from a surgical setting. And what you really want to look at here is that most of these patients is 1500 patients have either mixed essay I or have a S. And go to a standard A VR or a aortic stenosis pathway. However, there is a significant number of patients that have a I. And by customer bowel disease with or without aneurysm. And there's a number of different operations. We do a mental operations. We can do the re implantation or valve sparing operation with repair or if it's a patient that doesn't have a route procedure, we can do a B a b a B repair uh with uh a super coronary aneurysm replacement with retention of the route. And as we as we just said by customer check valve comes in a couple of different varieties. We're gonna be mostly talking about type one uh receivers, one uh collapsing vis a vis. So can we spare or repair more complicated clinical aortic valve presentations such as bicuspid aortic valve and why is this so important. Well here's an effect of the guidelines that that mess just talked about. So this is a 37 year old woman. She was in ICU Nurseries in ICU Nurse. She has a B a B syndrome, a five centimeter, a senorita mildest TJ effacement and a 4.5 centimeters sinus segment damage. So she has a big aorta especially for her size. But she has traced a mild aortic valve insufficiency. No way. Yes, no gradient and really a pretty close to physiologically normal aortic valve with normal left ventricular function and absolutely no symptoms. This is the kind of patient who should be getting a more advanced operation and not an aortic valve replacement. And what happened with this patient. She went to a place and she ended up with a mechanical composite graft 37 and coming in for life. So we do have a problem. So the problem in the worldwide credit surgical community is that are we ready for prime time? And maybe the answer is no, but that's why we're doing this webinar so we can try to change that. What this is an example of a beautiful bicuspid aortic valve. This is a silver zero. There's no reason whatsoever to take this normally functioning valve and throw it in the bucket and put in place in place. Either mechanical or tissue valve. So I want to get used that as a springboard to get into be a V repair uh and um and bottom line is that it's still an evolution, but we've learned a lot over the past 10 or 15 years and as Einstein said, make everything as simple as possible, but in this particular case no simpler. This slide is a complex slide which shows how these B A B. A. I patients present. But the bottom line with this slide is that or this this figure is that you can get a leaky or aortic valve insufficiency from a dilated route, you can get it from a dilated analysts, or you can get it from a prolapse or a combination of all three. Uh And this is what we're generally dealing with when we have a bicuspid aortic valve that needs to be repaired. So the first segment is what we talked about, which is the root aneurysm with bicuspid aortic valve. And one of the things I wanted to kind of go over because most people in this audience have never seen this is what actually operations, we kind of do. So I'm gonna throw a couple of pictures from the operating room, so here is a picture of the operating room vis a vis we're with an aneurysm. Uh and what we're doing here is really a valve assessment, we're seeing what exactly we can do. We notice that the reference leaflet is very, very nice. It's normal and it moves very well and then we have the con joint custody or the Rafid custom as you can see to the right. And this is kind of when we look at these and we make a decision about what kind of geometry we're gonna recreate whether it's going to be a 1 81 80 a type of repair or whether we're going to respect a little bit of the normal geometry in this particular case was is a 1 60 to 1 70 angular ation. So the uh the first the next part about how to do these cases of what we do is to take these free margins of these leaflets and make them equal. You can see that the conjoint cust has has a much longer free margin. And that's why it's prolapse. Sing and that's why these this patient has aortic valve insufficiency. So uh we do then uh kind of take the rafei and and trim it off and take some of the the fibrous tissue. Uh And that's in fact the first move. And then what we're gonna do here in this picture is we are taking the free margins as you can see here an equalizing them so that uh we have no no more prolapse. So this is basically fixing the prolapse and that's what that's the kind of picture you see. And this is looking at the motion and making sure that we have good motion of the valve so that it opens and closes very very nicely and does not create aortic stenosis. Now, after we finished repairing these, as you can see here in these two pictures, we do want to make sure that we have excellent opposition of the leaflets. With complete free margin equality as you can see. And now we're gonna figure out how we're gonna do the aortic root operation. Uh and whether we're gonna go 1 81 80 or whether we're going to go Within what we call to 10,150, which is more of the typical uh raph, a bicuspid aortic valve to do this, we end up putting structures in below the valve uh to create the conditions for a re implantation procedure and this bicuspid aortic valve repair. And really selection is the key. We found that uh we don't have any difference in our results between a 1 81 80 repairing a to 10 1 50 repair. Uh And so this technical consideration is all about. Selection is not really that important for outcome. And here's an example of the repair has been done at least the first part of the repair. And we're now going to fix the aortic root with this new aortic root and a valve sparing operation. And here is kind of a picture of a couple of future's going in and then we take this new aortic root and we'll place it over the valve and re implant the valve inside this new aortic root, which is particularly designed for this particular patient. So it's custom designed and here's an echocardiogram afterwards. And what I think the most important thing to see is is that the reference leaflet is very very has very great excellent motion is opening fully. The conjuring. Leaflet is also doing okay but not as good as a reference but that's always the case. And we have no ai and no particular a. S. In this repair. Now we also have operations for uh for the a setting for genotype as doctor decides spoke about as well as isolated ai where the root is actually a normal diameter. Uh And what we do here with this is utilized a ring underneath are at the annual level just like a mitral valve ring. Uh Same concept and then do the isolated vis a vis repair. Uh And not have to do a full route operation for aneurysm. Uh And this is a good example of this kind of case. This is a young lady who had a wicked ai as you can see here. Uh And you can see the bicuspid aortic valve with severe torrential aortic valve insufficiency. This patient also had a decreased ef and a big ventricle. Uh And here's what it looks like in the operating room. Uh And you can see the big cleft and defect in this bicuspid valve causing this aortic valve insufficiency. Uh And this is what we're gonna end up repairing and we uh take all this uh this excess fibrous tissue off. Close this cleft this opening With some 5:00 suitors and this is the repair ah and this is what it looks like. And this is the completed cleft repair. And we have great motion as you can see right there. Uh And the tests that we do shows no aortic valve insufficiency at all actually repair. So this is a key. The key is motion. Now after we do that, we ended up taking a this this uh this ring and this is what the ring looks like. We've picked a 30 millim ring here uh and uh we're gonna cut the ring and we're gonna put it down at the level of the annual is to try to close the to tighten up the and it's a little bit so that we have excellent co optation. And this is exactly the same concept as the mitral valve repair, acceptance on the aortic valve instead. And you can see how, how this is done. And we put this, put this ring in an appropriate fashion and this is what it looks like again, this particular case. And you can see exactly the same thing as before. The key to the repair as far as it is to have no ai but also to have excellent reference leaflets or non conjuring leaflet function as you can see here. And you can see the motion beautifully right here in these reference sleep, but also reasonable motion of the contrary, but that's never quite as good. We have a good co optation zone. So we're not going to have a leak through this. Uh and RP creating is only six. So this is uh the second types of repairs that we do. And so from the cardiologist standpoint for the echo evaluation, we we need to have angular diameters. We need to know how much of a reduction we got, we need to know exactly how much co optation zone there is uh in the valve from the uh the T. E. We need to take a look at the leaflets whether they're up high or they're down and low into the ventricle. We need to look at the ratio of the annular ratio for diameter between the S. T. J. And the analysts as well as any description of any jets as well as LV function. So this is kind of the t uh cardiology uh situation. And looking at these are results have been really pretty good. This is one of our last uh we have a recent a new paper coming in right now but as you can see, the results of this operation have been pretty pretty satisfactory regarding freedom from ai either plus three plus four. And as I said from the aneurysms, this is in the valve guidelines. Uh and for the first time in the history of the H. A guidelines, aortic valve repair and bicuspid aortic valve is considered uh in selected patients actually in the guidelines now for the first time. So that's a big big deal and it's also in the european guidelines just came out a couple of weeks ago. Uh for aortic valve repair may be considered in selected patients at at centers that do this. Uh So it's in the european guidelines as well. These are these are new issues. So valve repair is in the european guidelines now for aortic stenosis, bicuspid aortic valve in young patients were explosives. It's a it's an important very important topic because there's a lot of young patients who have bicuspid aortic valve presentations. So what is actually the data behind the choice of a heart valve in a 35-60 year old person? Well it's not all actually all that good. This is some data looking at the blue line here and you can see in patients under 40 that uh there's actually a significant um uh chance that they're going to end up having structural voluntary deterioration within 15 years as you can see here. So these results are really uh kind of poor. This is for prosthetic valve a br And this is another study looking at the probability of re operation during the structural valve deterioration by age. You can see that it's close to 50% at 20 years in a 50 year old. Uh So uh this is uh something that we all should know and this is another uh paper, looking at basically the same thing and but it's looking at excess mortality, which is important. Uh and what you can see is, however, uh there was an inferior age and gender matched excess mortality related to uh this is the patients who got a tissue valve at a young age and this is the natural history of the, of that normal population. And there's a kind of a significant excess mortality. Now this is a little newer paper and the excess mortality is not quite as bad for this bio prosthetic analysis, but you can see from this paper that there's also kind of a whole host of reasons why patients don't live as long as they should. Uh And this is kind of what we're trying to get at with with some newer operations, which we'll get into. Um now this is mechanically or valve showing basically the same thing as you get older, say 25 vs. 55. The the excess mortality is uh is less, but it's still quite significant. This is normal matched controls and this is if you have a mechanical valve in place. So um uh the uh this is kind of what we're what we're trying to uh to get better at Now. This is the new England Journal paper that just came out recently a couple years ago looking at aortic valve and mitral valve, but we only gonna talk about aortic valve replacement today from the California state database. And what you can see here very importantly is is that this is for mortality, is that the hazard ratio for mortality starts to to hit at about 62 years of age uh for uh for tissue valves. And um also that the that is statistically Significant at about 52 or 53. So we need to have a better alternative for patients in this lower age range. Um because they're basically dying early if they get an aortic valve And you can see it here in this in this far right panel that there's a difference between mechanical and biological valves in these younger patients were mechanical. It's actually better if you're under 50. Yeah. And this actually has what we call Ross implications which I'm going to talk about. So what is the data behind the choice of a heart valve and a 35-60 year old person? Well, it's not very good and we can do better. The biggest one of the biggest new issues is uh and I have to give dr um tola from 1978. This is an old because this is an amazing thing to reread that the most important valve determinant for survival is to have a big vow. This is the problem of patient prosthesis mismatch. And he was amazingly prescient to be honest with you in 1978 to understand this. A recent paper has just been put out from the societies and the definition of process prosthesis patient mismatch ah is a moderate, is a patient of uh area per meter squared of 0.85 to 0.65. But more importantly severe patient prosthesis mismatches when the valve as 0.65 centimeters squared per meter square. And to put that into perspective, this is an older paper looking at human beings uh and a normal uh aortic uh Manulis uh Diane I mean area is between is about 2.5 centimeters to 2.22 point five centimeters squared per meter squared. Uh and the minimum for for to have you know zero problems is 1.5. Remember that 1.5. Now this is 6.65 is pretty severe. Now this was maybe one of the worst paper. You know, this was a very significant problem paper. This was this is myth. This was the paper put out 20 years ago. Um and from from the clinic looking at survival after a br appears not to be adversely affected by moderate prosthesis prosthesis patient mismatch. Uh And uh eight years later the same paper, the same group. You've had this particular uh paper which is really really important. Younger patients had worse than expected survival that was further diminished with the insertion of a small prosthesis as you can see here in this page in this paper with excess mortality especially in younger patients. And this was the same group. So they kind of backtracked on the original assertion. Now this is a huge paper looking at a big data set from the sts database in the centers for Medicare for CMS. Uh And what they showed is that uh severe PPM as you can see here with the p value that very very significant shows a decreased survival rate and it starts to get significant very early. And this is an admissions for chf and this is survival. So the bottom line here is that severe patient prosthesis mismatch, which is small valves for patients is very bad. Ah And it's something that we we have to try to get around. Um And this is another uh one of the very similar study of a large pages series from europe. And you can see over here that if there's a moderate to severe uh patient prosthesis mismatch. The mortality is much higher. Uh every outcome that peri operative one year, five year and 10 year. So it's very significant. So this brings us to the ross operation, the ross operation. And I'll ask dr Zito and doctor decide a little bit about the operation and how it's done in our in our chat. But our discussion but the ross operation is is being revisited And here is an example of just some of the data from a prospective randomized trial of 109 patients, 108 patients in each group. And you can see that the most important part of the slide is this line up here, the blue line is the normal is the normal life expectancy of a patient. Uh in this prospective randomized trial, you can see that the ross procedure completely equaled uh this uh this uh life expectancy and a home a graft and other bio prosthetic valves uh are are decreased and these are all younger patients. So this is a really a big, big deal. There's lots of papers out there showing that Ross has actually has a pretty reasonable 2025 even 30 years survival. This paper was was one of the people rose original papers. And and this is uh this is looking at this PPm vis a vis the ross and the black the dark black things are ross patients. And you can see that the ross patients all have very low uh mean gradients and very large even with exercise uh compared to other valve options, whether it's a mechanical valve, a tissue valve, or even a stent lys valve. And uh so this is an important point. So, the ross has excellent thermodynamics uh and has has basically normal, we remember that 1.5 centimeters squared per meter squared. The ross is really at that level, as you can see in these black dots And uh again at the at the guideline level for the first time. Uh the uh the guidelines show uh have a uh an advocacy for the Ross procedure in patients less than 50 years old, with uh at a comprehensive valve and aortic center. So this is actually entered into the guidelines now just last year. Uh and this is the guidelines looking at young patients less than 50 years of age. And you can see that there's a mechanically it goes towards mechanical a VR. But um if there's appropriate anatomy and a bio prosthetic valves preferred, the recommendation is to go towards the Ross. So the rosses is in the guidelines for young patients. So in conclusion, be a V. Presentations and young patients less than 50 consider baby repair and reconstruction and aortic valve insufficiency patients with the A. V. And consider the ross operation in baby patients with the aortic stenosis. And this is some of the new things that we're doing at the Penny or to depend on our new operations that were We're offering at the Penny or two centre. So thank you very much. So I'll stop sharing and then we'll go to um Dr Zito who will be presenting some of the really controversial stuff regarding uh roll and challenges of Tavern in treating black custom, aortic valve disease. Uh And dr Zito has a long experience with this. Uh Wilson Zito, thank you. Thanks joe, appreciate that I'm going to share my screen. Mhm. Oh well we're going to switch gears a little bit today uh at this point and and talk about T. A. V. R. Or trans catheter aortic valve replacement in the treatment of bicuspid aortic valve disease. Um To put it in proper context. As you alluded to earlier. Gerald this, we're now talking about aortic stenosis patients. These are patients that are that have valves that are not repairable in general. As many of you know that bicuspid patients are younger but not often completely necessary that we don't seem older patients. But in general these are younger patients with are relatively longer life expectancy. And I'll come back to that point in a little bit. So here are my disclosures. I'll start with a clinical vignette. I think that often frames the discussion in a very nice way to get us thinking about patient centric discussions. So this is patient S. L. You can see here that he has a bicuspid aortic valve. Uh At least with deceivers Classification is Deceiver zero. There's no Rafei. He's young. He's 68. Uh severe symptomatic aortic stenosis by echo. You can see here Intact ejection fraction Eva of .56 With a mean grant of 42 mm. Mercury And an sts predict the risk of mortality for surgical aortic valve replacement at 1%. So what do we do with this patient? And this was a patient we actually saw a while ago. This was a few years ago. Yeah. Um And here is his arteriogram and as you can tell by the slides he underwent a tvR again put it into proper context. He was enrolled into a partner three Bicuspid registry. This is a this is in 2018. So this was a little bit ahead of a head of the curve and getting us thinking about what are some treatment options. So by no means was this a routine practice for us back then and certainly not for us as well current. Um here is the deployment, you can see on the left hand side, your standard TVR deployment under rapid pacing with a balloon expandable platform and now fairly reasonable outcome with a completion angiogram. So with these images, um advocates of TvR would say the discussions done let's do T V. R. S and everybody. And of course the story isn't as simple as that. And there are many more discussion points that we can follow up on in the next few minutes. But I would have to say that it is a potential option for some of our patients and I think proper selection uh in the context um is important and we'll talk about what those means. Just a little bit of background. We are just getting into treating th treating bicuspid valves. To be our as you can see here a recent publication, it we're just scratching the surface a lot to learn lots of knowledge gap. Only 3% of patients in our sts a ccTv the registry or pie custard patients treated with TvR in contrast or experience as demonstrated by joe bavaria with his talk that we have a lot more Knowledge, although not, you know, complete knowledge. A lot more knowledge about treatment, bicuspid aortic stenosis with surgical techniques. About 30 in most studies in this one particular Deadwood's commenced trial um had bicuspid valve treated with bio prosthetic aortic valve replacement surgical. This was a recent article published in some of our journals. Again, lots of excitement, lots of investigation into bicuspid valves. And I think this was interesting that as a sign note that we're now getting more and more sophisticated in how we talk about bicuspid valves. And given us a common language that we can all share and talk about. You can see here on the top left the most common one that we often see diffuse by custom aortic valve, a baby, the two sinus and a partial fusion and of course associated by custody, autocracy that Ramesh had just talked about earlier. I won't believe at this point. Uh But to what the message said earlier, it is a a unique um syndrome in the sense that it is just not a valve issue. It does have other aspects particularly associated in your Ismael pathology. Um And that really does add an account for us when we talk to patients about treatment options. Mhm. So let's talk about some of these anatomical considerations. When we're thinking about how to discuss with the patient when they have bicuspid aortic valve severe aortic stenosis. That's symptomatic and now as a shared decision making with a hard team including the patient, what do we do? What are we talking about in terms of how we choose surgical versus a catholic base option. Of course we look at valve morte ology, this we do this for all of valve treatments. Whether it's try custard or bicuspid, we looked at the calcium, look at coronary heights, we look at the left ventricle and mythology. So two semesters point earlier, it is not just a valvular issue, it is a nordic root complex issue. And certainly any evaluation for the best treatment option should include all of those aspects Snooks in terms of a raffle and classification. This was a nice representation of the different prototypes. The variation of how classification can present in a bicuspid valve come from severe to none. Um and from a directional standpoint from transfers to longitudinal etcetera. But again this I think expresses and demonstrates to us that it is not a monolith that monolith bicuspid valves are quite variable with the different types of Vienna typical expression. And we have to keep this in mind as we think about treatment options calcium. What about calcification patterns you can see here at the bottom of the panel. Um quite extensive classification that is not only at the aortic valve leaflet but also extend it down into the L. B. O. T. Into the micro valve across the order micro continuity. So again to my point earlier about that patient, I demonstrated I was in enrolled in a partner free by custom bicuspid registry. I think the data that we have to be careful about the caveat is that these studies up to this point with bicuspid valves and TVR or highly highly select the patients would appropriate anatomy and I can assure you that someone with this type of anatomy was not or was excluded in some of the earlier bicuspid registry studies. So I think we have to keep that point in mind carefully. Yeah, coronary anatomy um you can see um also plays into the calculus in terms of whether a TvR is the right treatment options for patients whether they're young or old coronary occlusion such as impatience will low take off of the coronary arteries or carefully screened in some of our earlier trial registries for tv armed bicuspid valves. These sometimes are exclusions as well. So again, careful selection and highly selective patients are often uh enrolled in these studies. So it's certainly not all comers when some of the earlier experience. This is a publication recently out of J. Jack 2020 looking at some of the factors that may be predictive of poor outcome when it comes to T. V. R. And bicuspid valves. Um And this is exactly what we were just referring to the last few slides higher risk anatomical features resulting in worse outcomes include the presence of presence of very large annual list. Hence parabolic bulky leaflets, rafei or L. V. O. T calcification concern also for verifiable leak but it's just as important route ruptures and of course the presence of rafei infused. Com assures. So I think we have to there's still a lot more to be learned regarding which patients are ideal for tvr versus surgical aortic valve replacement. Both joe and the mesh talked about guidelines are not going to belabor the point you can see here. Um These are the recent guidelines regarding S. A. V. R. Versus tavi in patients in whom a bio prosthetic aortic valve replacement is appropriate as you can see here. Uh The focus really uh has not only talked about age and durability but really thinking about life expectancy. Um you can see here at a class of recommendation one level of evidence a because these have been randomized studies, patients with severe aortic stenosis In any indication for autograph replacement who are less than 65 years of age or have a life expectancy greater than 20 years. The data at this point would still suggest a surgical aortic valve replacement. Yeah. A deeper dive. Looking at some of the factors, we talked about anatomical factors for younger patients. Um At least certainly at this point with the limited data we have on durability for patients with bicuspid aortic valves. Uh The data at this point was still favor or tilt towards a surgical aortic valve replacement. Just briefly, you heard about this ready from the mash patients were top Kathy. There are recommendations for treating those as well. Um 5.5 class. Uh recommendation 15.0 and a center of excellence to a to repair it can common aortic aneurysm disease. um and a 4.5 if you're having a indication for aortic valve replacement, you should be addressing the aortic aneurysm as well sir. This is a summary slide of um what we should think about for patients with bicuspid aortic valve stenosis. It was just recently published by the mesh in Jack 2020. Really nice schematic here of looking at patients taking an account all those factors. We just talked about whether the patient had a or to empathy, low intermediate risk age anatomy etcetera. Um And I think this based on our current evidence is where we are today, what we're thinking. Mhm. I'm a relatively simple guy. So I like to sort of take all that data and think about what I talked to a patient about when they come in. Uh And it is a shared decision making because I do think a patient needs to be part of the discussion as I mentioned before. I try not to focus too much on age. Age is a number. I think of it as life expectancy. I think this is the controversy that joe was referring to. What do you do with the patient who has a very long or expected long life expectancy in a good anatomy for T. V. R. I think the data, as I mentioned before at this point still leans towards surgical valve replacement. However that's a shared decision making. Certainly patients who have a light a long life expectancy and a risky or high risk anatomy, surgical aortic valve. I think it's the way to go. Patients would decrease or not unexpected long life expectancy. Um Both a suitable anatomy as well as unsuitable or high risk anatomy, tvr versus medical therapy. And of course as I mentioned, if you add in a patient's way or to apathy, certainly consideration for repair and aortic aneurysm for patients with long expected life expectancy. So thank you. And I'll stop there and I look forward to the discussion. Excellent and I'll stop sharing my screen. Yeah. See here. Mhm. All right. All right. Can you do that? Here we go. Stop, share great joke. Okay um So good work everybody. Very interesting topics. Um So I would like to maybe uh start off uh this discussion a little bit uh maybe start off with both of you uh regarding aortic stenosis and the ross. You know uh that's why you know the ross was used. It was a little bit out of favor for a while. It's kind of had a resurgence. Why is that? I think I think we've learned a few things first of all That there's been a huge push towards moving away from mechanical vows, right or wrong over the last 20 years to the point that Uh you know, we're using biologic valve and younger and younger patients. And even in patients who are, you know, we see patients frequently 30s and 40s coming back to us with bio prosthetics that have failed already. Um and so, you know, we know that that's not a great lifelong solution. Um we know that um in patients who have, you know, that's sort of unique historic stenosis or bicuspid gnosis, that the the proposed failure rates of the ross operation, which would be dilatation of the route, which typically happens either at the annual level or at the aortic level can be addressed with a much more complicated operation technically, but one that eliminates a lot of the potential failure modes of the raw. So the knock on the ross was always that the in my custom patients was always that the aortic root tended to dilate over time. And I think that, you know, with the, with the evolution of therapies, we have adopted an operation that is extremely resistant to dilatation later on. And that comes with a whole host of technical things that we do during the surgery, eliminating areas where that where dilatation tends to happen, but then also in the postoperative management of the patient selection as well, selecting people have appropriate analysts for this. Um And then on the postoperative management side. So as we have reinitiated our ross program, we actually had a very busy ross program since we restarted perhaps one of the biggest busiest in the country. Um uh that we also very detailed plan of ambulatory blood pressure monitoring uh and uh surveillance imaging on these patients to make sure that they don't dilate their autographs. So, Wilson um there's a question from the audience. Um what is the maximum according to Andrew that you would tolerate in a Tavern patient for B. A. V. And also for T. V. For that matter? I think that's a great question. Um And I think it all depends on the patient. Um an 85 year old patient um who is a redo uh multiple comorbidities. Um I'm not really sure it matters. Uh If I can do A. T. A. B. R. And he has aneurysm of six centimeters and he's not a surgical candidate, I don't think it matters. You just do it because it all comes down to treating that patient um as you go younger. And as you go, someone with longer life expectancy um where a surgical option becomes less um associated with morbidity mortality. I think that discussions um becomes more jermaine. Yes, absolutely. Tvr wire manipulation in the award out with an aneurysm is at risk for some sort of dissection or rupture, but I think to put a number on it, it's hard for me to put a number on it. I think it's just talking about the patient on life expectancy. Okay, so um let's talk about Tavern baby a little bit more. Um Two things uh and both of you would like to hear your opinions about it. The first one is uh related concepts. The first one is that it seems to me Wilson when you go through your talk and you're looking at some of the the the guidelines. And that's based on some of the some of the papers it almost seems like most be a. V. S. Except for quite a quite a minority. But most babies we'll have one of those considerations you just put up on your slide such as excessive calcium where annular calcium or calcium guts going into the outflow tractor or calcified rafei that's asymmetric etcetera etcetera etcetera. So the first question is aren't most of the patients would be a V. Have that especially if they're under you know 80 and have a. S. D. S. Score less than three. And the second question which is related is what about the selection process and all these be a V. Tavern uh trials it seems like the selection process is incredibly robust. Uh And that that may actually make the make the data or the result actually not applicable to regular practice. So um so Wilson once you start with that and then uh and then uh the mesh why don't you answer as well because you did that great paper as well. You know joe I think those are great questions. I'll start with the first one you're absolutely right early on. Um as we were learning um what tvr um characteristics can be tolerated. Um and what is considered safe uh We continue to you know learn today um um you know you know severe calcification, what is severe classification? Right? Everyone's threshold for what they can tolerate different. But you're absolutely right too early on. The trials were extremely selective with anatomy. You know having said that because of the careful selection the outcomes were extremely positive, relatively low para barbara leak rate, relatively low mortality rate. Um In the durability at this point does not appear to be any different than to try leaflet valve. So my point is yes agree carefully. Select carefully selected processes were in place but to put on my wire hat well that's good. Carefully selected patients with bicuspid valves should be able to be treated well with T. V. R. So I think that would be my other perspective. Um But much more to learn. And as you know the key question in my mind will be durability in terms of these patients with reasonable life expectancies and um you know more to come. So the real quickly, what is your answer to that question? Yeah. No I thoroughly agree Wilson that the you know the appropriate geometry although rare you know can be treated well with tavern. Um I don't think these registries or even you know some of the proposed randomized clinical trials, which I don't think we'll ever see the light of day. Um I don't think I'm going to answer the question of uh that that we all want to know which is, you know, can we apply tab or to a much wider range of baby patients because they're all gonna be very selective. Um and I think some of the things that, you know, as a Tavern operator, I know Wilson and I are both always concerned about is, you know, you end up downsizing the valves. You spoke so eloquently about patient processes, mismatch. Well, you know, a bicuspid, heavily qualified to have were almost always downsizing our valve to a smaller valve. Then we would put in surgically because you can't, you know, you can't move the raffi over. You know, you can't really do a lot with the conjoint leaflets. So in that younger patient, you know, and you still have ejection typically that's not straight in line with the order, but it's still going in the same direction as the original bicuspid valve is so consideration for future aneurysm formation. Uh and patient processes mismatched to the things we talked so much about today are still questions in Tavern. Younger patients with bicuspid valve disease. So now let's pivot to younger patients um regarding bicuspid valve repair in the aortic valve insufficiency patients. Um there was a question from the audience regarding the heart ring or an internal ring device as opposed to what I showed, which was an external ring and or a david five re implantation procedure. Uh No mess, you want to start with that? What do you think of that of internal ring devices? Uh And just to be a v repair paradigm in general? Right, So so you know, there's a lot of different techniques to do baby repair our approach as you showed. Uh So eloquently is generally to do an external ring or to do re implantation technique with with complex leaflet repair when necessary. Uh Internal rings are, you know, we haven't had a huge interest in them, primarily because they tend to, you know, I think my own feeling is they tend to be quite rigid. Uh And they force a very specific anatomy, especially in the bicuspid scenario where, you know, you talked about, you know, having a symmetric valves and having a symmetric prepares that are functional as opposed to putting in a rigid ring that forces one particular aortic anatomy, it may be a little bit more reproducible. Um uh For for those who don't kind of do a lot of it, but I think it, you know, having the ability to try and match or repair exactly to the patient's anatomy to me makes more sense physiologically than using something that forces you into a geometry, Wilson real quick. Do you have any comments on the internal ring? Uh Not much more to antenna moesha. I would agree. And echo, what is coming to our, I mean, I think the concept is lethal. It work And in annual stabilization, however you achieve that. It's like using different types of futures. It doesn't really to me the core principles, the principle. So I think the internal ring uh is reasonable, especially reasonable. Three cups valves, ah maybe also pretty reasonable in um and uh you know server 01 81 80 valves because that's how the ring is designed. The ring becomes less and less. This is the answer to the question of Carlo Rosati. Uh the ring becomes less and less appropriate. I suppose the better word as you get more and more asymmetric and when you have a very asymmetric valve, it's actually dangerous. Uh so those are, those are kind of the gritty answers to that question. Um So there's another question for you, Wilson uh the one of the audience members we have, we have a choice of two of the Taber valves in our hospital. Which one has a better outcome and by custom valves? Um I think the data was suggested or equivalent. It's dealer's choice whatever you're comfortable with. The outcome were very similar with both animatronic and Edwards platform. Uh so I don't I have a personal preference. Not because of data, but just because what I do, I like to balloon size first to confirm sizing and then put an average safety and valve, but I know colleagues around the country have had great success with medtronic veloute um as well. So I think they're both equally effective. Yeah. Yeah. I think I would just add that in patients who do have those higher risk features that are non operative candidates that self expanding platforms um might be a little bit safer. Although again we don't really have good data on that. And I think the risk of root rupture at least is lower in those situations paradigm really hard to know, agree. But I we often end up ballooning anyway because the B. P. V. O. So you still end up ballooning it because it leaks. Yes let's just go look at that. Uh the uh let's go back to the order because we all know that bicuspid valve uh syndrome and become sort of diseases both the valve disease and an aortic disease and almost everybody. Uh So what is the power of indexing and the mess? Do we should we index uh is indexing appropriate inappropriate uh What are your thoughts about the different indexes? Because I have to be honest with you as most everybody knows I tend to use indexing pretty liberally. Uh And uh your thoughts? Yeah I mean listen a 5.0 centimeter or 4.9 semi or to turner's patient who's four ft, You know seven is pretty abnormal, right? So we have to think about size of the Aorta in the context of the patient definitively. Um And younger people use these scores right to actually really try and nail down like sort of what the size of the order should be in someone that age or that size. Um And in adults we kind of moved away from it and went to just large numbers, but uh the size of the aorta to the size of the patient is very very important in terms of the indexing parameters. I kind of just very briefly went through them but indexing to the aortic diameter to the patients by surface area or height, the cross sectional area of the order to their height, which is I guess the Cleveland clinic approach. Um or even looking at aortic length which you know, I think is a involving area all have importance. And I think, you know, when we look at patients, I you know, I tend to look at two or three of those measures to try and understand, you know, what their potential risk is. Um But I understand, you know, understanding risk is it goes way beyond size, right? We have a paper that we published in Jack years ago that basically showed That, you know, the average or to increases in size by 28% at the time of the aortic dissection. So all the studies where we kind of retrospectively looked at how big the order was when it dissected we're all off in terms of the size of the Aorta. We went back and model our own patients and found that 90% of patients who have a work dissection would never have met guideline based criteria for intervention. So size, you know, sizes a number, but it it's not a marker of biologic susceptibility necessarily. We know when they're big, dangerous when they're small, who is at risk. I think that's a very important point regarding be ava or to apathy. And uh is that uh we really actually need to learn a lot more about about these, about these a orders when they when they rupture, when they don't rupture because that's an incredible statistic. You just said that um that, you know, genetics and other factors besides uh pure aneurysm diameter are probably as important or maybe even more important than diameter. So it's a huge area and this gets into the whole idea of our aorta clinics uh and the availability of genetic testing and genetic um and images as a whole in our and in the Aorta clinic because it's really a it's actually a pretty big deal. So um we are out of time. Uh and I would like to thank uh dr Zito and dr Desai for their excellent presentations. Uh dr Zito's presentation is highly controversial and was really great. Uh and uh the doing an interesting information coming out regarding the doctor decides presentation I think is very very important. Uh and from the standpoint of B a v as a valvular disease uh is really an aortic root disease, we we believe that's true, and I hope we helped uh elucidate some of the treatment uh paradigms that we've been using in the aortic center. Ah and hopefully we've we've been educational uh tonight in this area. So again, thank you very much, Wilson, thank you very much to mesh. Mhm. Thank you, joe. I have a good Hi everyone, yep. Yes.
