Chapters Transcript Video Tetralogy of Fallot in 2025: A Review and What's New Interventionally and Electrically Dr Ellis & Dr Saini give an update on treatments for tetralogy of fallot. 18 years now, so y'all have had me for a long time. Uh, I'm gonna introduce my partner to those of you in the room. Uh, this is Doctor Ashienni, uh, who joins us from Akron Children's, uh, this past fall. He is, uh, our co-director of the Adult Congenital heart disease program, and, um. Is an excellent resource for all things congenital heart disease, um. So we welcome Doctor Sandy. You'll see both of us in the hospital here, um, back and forth. So, uh, hello to everybody and welcome to the 3rd annual Kim starring Memorial lecture for those of you who have been around several years, you will remember Kim, um, and I really want to thank her husband Mike and her parents who are joining remotely today, uh, for sponsoring this and, and making this happen. Kim was, um. Just an amazing woman who also had tetrarology of flow, so we'll look at Kim's anatomy in a little bit, um, uh, and ended up with Eisenminger type physiology and many of you, uh, in this room and on this call helped take care of Kim, uh, during, during her life. uh, she was, uh, a truly wonderful person and wonderful patient, and we thank her family for, uh, for helping to make this happen. The 3rd annual lecture maybe next year you won't have to hear from me, so we won't have to do all three, Ellis, um, very, very briefly because this does involve Kim. Uh, Kim was born with tetralogy of flow, but the extreme form, uh, tetray of flow with pulmonary atresia, uh, and she had a discontinuous left pulmonary artery that, uh, you can see I'm not Picasso, but that drawing really tried to show that it was the LPA was fed by collaterals. She had uh a Waterston shunt. We'll, we'll review the anatomy of that, but the Waterston shunt means that the right pulmonary artery is directly connected to the ascending aorta, uh, so she had, um. Uh, she, she had aortic pressure going to her single right pulmonary artery, um, but the rest was unrepaired, so you can, as you can see by the diagram, she had, uh, mixing of both desaturated or deoxygenated blood and oxygenated blood leading to, um, profound cyanosis later in life was sats uh generally in the 60s, although that diagram says 80s, uh, Kim was not generally in the 80s pretty much ever, um, even on a 100% oxygen, um. Uh, Kim did, uh, exceedingly well through life. Uh, she, um, taught in the Chesapeake, uh, uh, middle school system, and, um. Uh, had, uh, really, uh, I think a very wonderful fulfilling life. Um, I always joke with her husband Mike that, uh, the one time he tried to kill her was on a family vacation when they went up to Mount Washington. He drove her up there, um, in the camper and Eisenminger patients don't do terribly well at significant altitude, um, and she let him know pretty quickly that she felt terrible and had SATs in the 40s, um, but coming down the mountain saved her. So here's the outline for today. uh, Doctor Senny and I are going to split the talk. Uh, I'm gonna give the first part. He's gonna give the second part. We're gonna give a bit of a background or update on adult congenital heart disease, uh, and the lifelong health journey that these patients face. Uh, then this is really gonna be a focused talk on tetrarology of flow talk. More about state of the art management specifically with regards to timing for intervention, uh, and we're gonna focus more on pulmonary valve replacement today, uh, than anything else and then Doctor Sandy's gonna talk about EP considerations, uh, specifically, um, ablation and when to consider ICD in these patients. So, uh, why are we talking about tetrarology of flow? Well, we see a lot of it. Um, the bottom left diagram really says that most of what Ashish and I do on the pediatric side is actually a cyanotic congenital heart disease of all forms of congenital heart disease. Remember that's 1% of all babies born, so there are more than 100 babies born in Hampton Roads every day. So we get a new patient every day, which doesn't, of course, compare to what Doctor Adler gets every day. He gets tons of new patients every day, but we get one new baby. a day and statistically those babies are going to grow up and are going to become adults and are going to become our in this room mutual patients. Most of those are cyanotic patients, your ASDs, your VSDs, your PDAs, your coarctation, your anomalous, pulmonary veins, etc. 25% of congenital heart disease patients are cyanotic patients. And now look at the far right diagram for you. Uh, the largest percentage of cyanotic congenital heart disease is represented by terology flow, just over 40%. So that is what we're going to be talking about today. Giving you a historical time frame, tetray of follo, of course, uh, we consider that four separate things. I'm gonna tell you this morning that's really one embryological problem that begets for clinical sequelae was really not described by Etienne Arthur Fallow in 1888. It was actually described by Doctor Stinson, a Belgian physician in 1671, but the. Publisher Parrish mantra dates back even to then. He described it in his own notebooks and told colleagues about it locally, but that stayed around Louven, um, and, and parts of the Netherlands and Belgium. Uh, it did not make it out to the world. So when Etienne Arthur Fellow published it, people like, oh, we've heard about this, but you described it first, so we're gonna call it terology of Follo. But it's really, uh, it really belongs to Stinson back, um, uh, about 110 years beforehand. He described it, but as you can see in the timeline, we couldn't really do much about that until, uh, other than describe it until the mid 1940s when we had the first Blaylock Tausig shunt or now, uh, most people will refer to it as a Blaylock Tausig Thomas shunt. Uh, so Alfred Blaylock, uh, you, you'll see and we'll have some some more pictures there in the center. Then Helen Taussig, the grandmother of pediatric cardiology, um, and Vivian Thomas, the lab assistant, and really the, the, the brainchild behind, uh, developing this and, and his pioneering work, doing it in dogs himself showing Alfred Blaylock that he wouldn't go killing lots of babies doing it, um. That was uh first carried out in 1945. I'll, I'll show you some pictures. Um, the first complete repair was done in 1954, uh, but that was done on a much older child. The neonatal repair was not until, uh, Doctor Adler and I were, um, were, uh, neonates in 1975, uh, and then we really get to, um. Late complications that were better described in 2000 and we started to get more strategies to address that including trans catheter valve replacements. So, so as promised, here's some pictures of some innovators and from the American Weekly, a now defunct, uh, as most are newspaper from Baltimore, um, in 1947 saying that we're saving our doomed blue babies, um. This was this was truly a uh a a remarkable uh surgical achievement back uh shortly after the end of World War II, uh, so just remarkable technology and this was all done of course off cardiopulmonary bypass, um. So what did they do? They did this thing on the left and hopefully you guys can see the pointer. This is a classic Blaylock Taussig shunt. I point this out because a lot of our patients in this building, when they come here, will have some form of a classic BT shunt, and that's important because we turned down that subclavian artery, meaning they. Will not have a pulse. You cannot get radial access for cats. You cannot trust a blood pressure. So if you've had a classic BT shunt that can be on the right or the left, or I have one patient who's running around here, um, uh, her first name is Garnetta. Many people in this room will know her. Um, Garnetta has had bilateral classic BT shunts. You can't trust a blood pressure except if you put it around her neck or her leg. So, uh, and you certainly can't get radial access for CA or art lines in, in an ICU setting. So a classic BT shunt was what was done in 1947 by Arthur, uh, by, uh, Arthur Blaylock, um, and promulgated by, uh, Vivian Thomas and Helen Tausig, um, and that is what this boy had, and he's holding his, his thoracotomy there on the left, um, and looks really happy about it, um. The modern approach is the modified Blaylock taig shunt where we do not sacrifice the subclavin but use a Gore-Tex tube generally between 3 millimeters and 4 millimeters. Most of them are 3.5 millimeters, uh, due to, um, radius to the 4th power of Posoy's law. Uh, we have a whole lot more flow at just slightly larger sizes. Uh, the length doesn't seem. To matter as much, uh, so a classic Blaylock Tausig shunt is different than the modified Blaylock Tausig shunt. Just again to remind you, a Waterston shunt, if you hear that term, that's not done anymore and hasn't been for decades, but that is what Kim Starring had where her right pulmonary artery came, uh, the back wall of the ace and aorta was anastomos to the front wall of the right pulmonary artery. A pot shunt is the opposite. Back wall of the LPA is anastomos to the front wall of the descending aorta. Both of those distorted pulmonary artery architecture and led to early pulmonary hypertension and are not done anymore because there's no link to those. Uh, we do do central shunts shown down in the bottom left there from time to time still, but those are systemic to pulmonary artery shunts just to remind you because they do happen. In our terology patients, so the reason that we're here talking about this is because this is not a theoretical population. This is not like you may see them once every few years. The, the prevalence, uh, we already said the incidence is about the same through the years and through the countries, but the prevalence. Of uh of adults with congenital heart disease is rapidly increasing. This is data from Arianne Morelli from McGill, uh, published in 2007, where, and this is Canadian data here, uh, so there's the conversion factor to American data, um, it's OK, you can laugh at that bad joke. That's right, exactly, um, so it's it's 70, uh, 70 patients, uh, uh, per 100 US patients anyway, um. Uh, it has been clearly recognized that the number of adults living with congenital heart disease is, uh, is meeting and now exceeding the number of children with congenital heart disease, so. Uh, and, and you'll see that we've had that Arian has published some follow up data recently, but in the US now it is estimated that about 1.8 million, so essentially the population of all of Hampton Roads, if everyone in Hampton Roads had congenital heart disease compared to the US population, that's about where we would be, um, and again, the number of children with congenital heart disease exceeds the number of adults. I'm not gonna bore you with a lot of data, but this is her updated, uh, uh. Uh, Population data that's now 10 years old that proves that uh that conjecture that um that that estimation, um, from back in 2000 that the prevalence has increased by 11% in children but 57% in adults because they're living and importantly the more severe disease, the single ventricles, the transposition patients, others, those are the ones that are living more now that didn't necessarily. In the 60s, 70s, or 80s, so the the, the more severe disease is becoming more prevalent, especially in adults, um, and that's, that's shown um uh in the, in the, uh, top diagram. The, uh, so adults now account for 2/3 of the population of congenital heart disease patients, um, in, in her Canadian data and we find that that's true in American data as well. So this is happening. congenital heart disease now is a an adult problem, not a pediatric problem primarily. So, the number of adults far exceeds the number of children with congenital heart disease, um, and Doctor Adler is the adult in the room in this example. All right. It's also important to note before we get back into into terology specific things that very few interventions that we perform. Are actually repairs. We use that term and that's a misnomer. It's really a palliation. A repair has the connotation that we fix things when Dr. Robertson takes his car into the shop and gets it repaired, he has full faith that that will be completely fixed when he leaves the shop. That is not what we promise our families in the building next door. We promise that we're going to make things better, that we're going to be able to have them survive, that they're not gonna. Be so blue that they can't function, um, but we do not promise that that will uh end their, their lifelong um health journey with congenital heart disease. Most of what we do, um, uh, except if we tie off a PDA or perhaps if we devise closing ASD, although that's debatable, or a bla WPW very little else of what we do is curative, uh, and most will require lifelong, uh, surveillance and re-intervention. Did you want to do this, Doctor Sonny? OK, just do it like this. OK. Yeah. Thank you, Doctor Ellis. Uh, thank you for making me a part of this, uh, uh, Kim's lecture. So this is very important because The the shift is now happening and we are considering congenital heart disease from a lifelong uh perspective. Uh, we, uh, obviously, as Doctor Ellis mentioned, we can diagnose most of these complex congenital heart disease in fetal life. We perform various interventions in their first year of life for early childhood. And then there comes a honeymoon phase where you know life is good they go to school they attend colleges they have they have boyfriends girlfriends um and they they they just get used to the lifestyle and and many times there is attrition there is they they get lost to follow up and then they show up in adult emergency rooms and in adult hospitals not knowing what they actually underwent in their childhood life so. So, uh, uh. Making sure that these patients are not lost to follow up, making sure that we continue to transition these patients better is what we are now focusing in our children's hospital. How do we move forward? not on that one, on this one. So another like showing their lifelong journey so the, the need for repeated operations and repeated interventions is is not insignificant in these patients. Uh, they, they, they have frequent emergency department, uh, visits the many young women are now getting pregnant. So what we are now understanding in this population is that they are in a chronic inflammatory condition, and there is data that these adult congenital heart disease patients are actually have accelerated aging because of chronic inflammatory state which is not uh very much different to what diabetes and other like chronic uh uh uh metabolic conditions which we see so. So, so we start from the mic turned off and so I think the virtual. Is that right? Uh. Are we back? Can, can people hear us? OK, yes, thank you. OK, thank you. Yeah, so basically. The need for reoper operations, reinterventions, and like this being a chronic inflammatory state is what the newer data is bearing this out. These patients, they have accelerated aging process and what we have seen is that they they have uh telomere shortening and and these are the really young patients but with old hearts and old brains so. Very different from from uh. From our adult uh cardiovascular diseases. OK. Thank you, Doctor Sandy. All right, so now that we've had a background about uh congenital heart disease in general, let's get specific about tetrology of flow, and we'll review a little bit of the anatomy and physiology and then get into some of what's new about pulmonary valve replacement, um, and. Uh, and, uh, EP issues surrounding that. So tetrarology flows we've already said is the most common synotic congenital heart disease and represents, as you saw, about 45% of all cyanotic forms, but really about 10% of all forms of congenital heart disease. So that's, that's a large number, um. You guys don't have to worry so much about the postnatal effects, but, but babies like Kim who have very little pulmonary blood flow will need to be on prostaglandin to try and maintain ductal patency to give adequate pulmonary blood flow from the duct until we can either stent the duct, do a surgical shunt, or do a neonatal complete repair. Um, most of the time though, those kids stay on the 4th floor. You guys don't hear about those or come over to CHKD for. Observation are watched for a few days and then go home and then come back and have a complete repair between 2 or 3 months of age up to about 6 or 7 months of age. So that just gives you a perspective about when we're doing this and sometimes that's the only surgery they have before, as Doctor Sandy said, they end up here in this building for some reason in their 20s or 30s. Sometimes this is the only surgery that they've had before they meet you. Um, the complete repair will talk about what that is associated with, um, uh. Or I'm sorry, what that comprises of, uh, and we can do neonatal repairs, but oftentimes it's easier and have you have fewer complications if you can wait until the 3 to 6 months of life mark. Uh, importantly, um, right aortic arches are very commonly associated with these conotrunkal abnormalities. Remember your cono trunk also called your infindibuum. Any time you have malalignment type issues with the conotruncus, um, the tetrology flow truncus, uh, double outlet right ventricle transposition, those sorts of things are conotrunkal abnormalities, uh, right aortic arches are commonly associated with it, as well as genetic syndromes. I remind, uh, uh, this audience that, um. The DiGeorge syndrome or 22Q11 microdeletion is highly associated with tetray of flow. We do test every baby with tetrology for this now, but that is a relatively recent thing. We did not do that in the 80s or 90s because we couldn't. So you may find adults living with tetray flow who are undiagnosed with Dieorge and do have calcium problems um after you take them to the cath lab and they get significantly hypocalcemic and effects. Their rhythm that affects their function, etc. um, or they have immune system problems associated with thymic hypoplasia charge syndrome, um, which I put the what the acronym means for those who, who don't remember it and haven't done a pediatric residency or odder or voctoral um association. There's no gene mutation associated with with that the way there is for charge andege. So these are some associated syndromes with tetrology. So what is. Uh, what is tetrology? What does, uh, what makes up the four things, uh, the four clinical sequelae that really are the monology of Stinson. So, uh, for those of you in the room, you, you can see I'm gonna do it, uh, I'm gonna show you with my hands for those, uh, virtual, sorry, um. So, so, uh, imagine I've got my aorta here, my pulmonary artery up here supported by the sleeve of muscle or the infantdibuum. If you have anterior malalignment of the neural crest cells beginning around 8 weeks of gestation up through about 12 weeks of gestation, so you have abnormal anterior migration of the neural crest cells, which is what happens. You open up. A VSD generally in the perimembranous septum, it is still an anterior malalignment VSD because it's being pulled forward. We contrast that to the posterior malalignment VSDs where the neural crest cells move backwards, and that's when you get problems with subaortic stenosis, interrupted aortic arch, those sorts of things because it moves backwards when it's pulled forwards, as you can see, so we have that big ball of muscle on the diagram. That should have attached, where's my little pointer that should have attached to the crest of the septum. So you open up a VSD. That creates because you have that muscle moving anteriorly, that's again I use the Star Wars analogy for those in the room, remember the trash compactor scene. Instead of the walls moving in sideways, the floor here is moving up. The ceiling is not moving. The floor is coming up, so you're. the right ventricular outflow tract, your, your pulmonary valve and your MPA, those aren't changing, but the floor is coming up because of this anterior malalignment of the of the cono trunkus, the infindibuum, so that causes RV outflow tract obstruction, leading to your RV having to obviously generate more um. More forced to eject blood so you get RVH um and of course it's pulling the aorta anteriorly so you get this overriding aorta. So it's although there are four seemingly separate clinical sequelae, it's really a single embryological problem, this anterior neuro crest cell migration problem that begets these 4 clinical sequelae. Um, if you have the severe form, that's what Kim was born with. She had pulmonary atresia. If you have such anterior malalignment that it causes complete RV outflow tract obstruction, that is the extreme form of tetrarology flow, also called tet pulmonary resia. Doctor Vance will call that, uh, uh pulmonary resia VSD, but, but it's tetrology flow with pulmonary resia. Um, so that is the monology of Stinson. If Stinson had described it, we would have be calling this something related to Stinson, and it's really not tetrology, it's monology. It's one problem that begets four things. OK, so now that we understand that. So the first repair was, as we already said, was done in the early 1950s by Walt Lillehi at the University of Minnesota using controlled cross circulation. I show this from time to time just to make everyone remember how far we've come in a relatively short in geological time, uh, relatively short period of time, um. I'll show you a picture of controlled cross circulation, but that is where the parent is both the pump and the oxygenator. It is the only surgery that potentially has a 200% mortality. You could kill the parent and the child, and that did happen once. Cardiopulmonary bypass, as we know it was not even developed in its infancy until 2 years after the first tetrology of full repair. Um, so this is controlled cross circulation. This is a diagram and this is an actual picture from the operating room with the child on the left and the parent on the right in the operating room at the University of Minnesota. Uh, again, you can see the diagrammatic um feature. This is clearly the father, uh, helping the son. Um, but, uh, but, but yes, the, the father is providing both, uh, the oxygenation and the flow. There's a roller pump that's helping that and then it goes to the child. Uh, what do we do? Well, we patch clothes the VSD, uh, so we will cut out this sub pulmonary muscle bundle. Actually, I'm gonna show you this, um. We're gonna cut out that sub pulmonary muscle bundle which on this right diagram is not well demonstrated but here with the number 3 we will oftentimes either cut across the pulmonary valve so for those in the room, if my hand is the pulmonary valve annulus, we'll cut across and open it up and then put a patch here, so called transannular patch, which of course compromises the integrity of the pulmonary valve, um. Uh, so it will open up the RV outflow tract, but of course you no longer have pulmonary valve competency and generally that's accompanied, as I said, by cutting out muscle bundles or uh the infindibuum that hangs down below the pulmonary valve. Sometimes you have to augment the main or branch pulmonary artery. So when we describe it in childhood, we'll say tetray of flow with pulmonary stenosis, and then we'll describe it. Is it sub valve, valve, supra valve, or multi-level RV outflow? Tract obstruction and then of course we're closing the VSD uh generally with a Dacron patch uh with running suture that's important for our EP colleagues as you'll hear about later on, um, because they're near each other, the, the infidibular resection, uh, and the RV outflow tract and the VST patch, but not right next to each other, and oftentimes, uh, they'll need, uh, a, um, and they'll they'll need a ventriculotomy to get access to this nowadays we're doing it through a right atriotomy. So here in this uh more schematic diagram this is a transannular patch that extends from the RV outflow tract as seen in this other diagram up across the annulus and it it comes on to the MPA um this is an actual CT from a patient, uh, Doctor Trejo, you will be cathing her tomorrow. Um, you will be putting a harmony valve in her tomorrow. Um, she did not actually have terology flow. She had pulmonary stenosis and was operated on in the 1950s at MCV, um, and yes, it's MCV, not VCU, uh, she was operated on at MCV, um. Uh, in the 1950s, which is remarkable, she lives at Harbor's Edge. She is 79 years old, um, and this is her actual CT that we obtained here, um, and that is a big, uh, uh, you would, uh, a big RV outflow track patch. It's not transannular, and that's what's gonna be great. Her pulmonary annulus is right here and that is quasi intact enough to hold, uh, we hope, a sapien valve tomorrow, um. But we have a harmony valve on backup, uh, upstairs, um, that will return because they're like $50,000 if we don't use it. Uh, so that's an actual CT showing an actual transannular patch. So what are the late complications? Well, uh, most of it we have always believed, and I think that that's true, but I'll show you some some research in a minute, that we have said most of the complications are due to the pulmonary regurgitation that we cause by either Hagar dilating the pulmonary valve or doing a transannular patch. We do do some valve sparing tetrology repair. But that's more in the modern era in the, in the earlier era, most of the pulmonary valves were, were surgically altered or or or removed um the pulmonary insufficiency begets, uh uh of course right ventricular dilation which we, we in pediatric cardiology, um, uh. Told ourselves didn't really matter because we had kids in elementary school, middle school, high school, college who did really quite well um with free pulmonary insufficiency or or or free PI uh and and didn't seem to be terribly limited although they weren't doing triathlons or marathons they could they. Could, you know, do some sprints they could do PE they could, they were like reptiles they could do short bursts of energy. They just didn't have much in the way of stamina, uh, and they did pretty well but then we started to notice that there were subsets of kids who weren't doing so well and a lot of adults started to have progressive exercise intolerance, um. Their RVs got bigger and bigger. They developed RV failure. They developed, uh, what Doctor Patel loves both atrial and ventricular arrhythmias, um, and many ended up having more residual branch pulmonary stenosis, some residual VSDs from peri patch leaks, um, leading to endocarditis, um, and they didn't do as well as we had wanted them to in their later kind of teen years up through their 20s and 30s, and a lot. Develop arrhythmias. This is gonna be an underestimate with a third I think, um, it is clear that terology patients have a huge arrhythmia burden both atrial and ventricular, and even with a good repair they're at an elevated lifetime risk for arrhythmias, um, and some of that is because of the surgical technique, the ventriculotomy, the atriotomy, the VSD patch, the the significant RV outflow tract resection, um, but some of it is because of the hemodynamic post-op sequelae. What we're finding though, the good news is an appropriately timed pulmonary valve replacement plus or minus some EP, uh, uh, magic can really improve and reduce the long term risks of of RV failure of life threatening arrhythmias down the road, etc. um. All right, this is super busy. You don't have to pay attention to this other than one of the things and, and this is an update lecture. So one of the things that's new is we're now characterizing congenital heart disease but based on anatomic complexity and physiologic stage you will see that in our notes, um, and the idea is that. Not all ASDs, for example, are created equal. Not all tetrology patients are created equal. So we will grade things on anatomic complexity, simple, moderate, or severe, and then what physiologic stage they're in. Please don't try and read this, but the reason I point this out is repaired tetrology actually fits into two categories. You can have some that are moderate to anatomic complexity, but you can have some that have severe anatomic complexity. And then depending on how much valve disease they have, how many arrhythmias they have, um, what their RV pressures are, generally they're in physiologic stage B through D, um. So I mentioned that because this just came out 3 months ago. This is a new paper talking about suggested surveillance testing for patients with repaired tetrarology of flow. Please only focus on this last column because that's all you guys will be really encountering are the adults and notice. That's the reason I showed you the last slide is because it depends on what stage, what physiologic stage they have as to what the recommended frequency from everything from outpatient visits through cardiac CTAh, MRI down at the bottom, um, uh, Holters, um, uh, it, it matters. So, uh, and I'm happy to provide that paper for you. This is that paper, um, or one of the two that came out. This just came out, as I said in December of 2024, so it's 3 months old and in circulation. Again, please don't look at this diagram, except to know that we no longer think that it's just the RV outflow tract that causes problems. The VSD patch is also problematic, and we have recognized that for the last couple of years, but, but now we actually have data to back that up, and that specifically is because we typically have either a residual VSD or by doing the. closure, we disrupt the support mechanisms of the tricuspid valve. Specifically we impair the septal leaflet of the tricuspid valve that begets tricuspid regurgitation that we hope that our EP colleagues don't make worse with leads and and other things, but it often happens and we cause a chronic right bundle branch block by closing the VSD which begets electrical dissynchrony within the heart. So that aspect has been less. Uh, has gotten less attention, shall I say, than than the pulmonary valve sequelae. So now there's this enormously complex ridiculous algorithm, um, to say after you've had tetrology repair, is it just the pulmonary valve or RV outflow tract or is it sequelae from the VSD closure? So, uh, just, just know we are paying attention to both of those aspects. So what's different about the right ventricle and why do we, why do we in pediatrics care about the RV more so than than the LV um. And and why is it harder to help the RV? Well, people, uh, especially the med students that come through my clinic have, have seen this slide, um, and have seen this. So the LV, of course, uh, has, has fibers that can contract base to apex. It can contract anterior to posterior and it has circumferential fibers or torsional fibers, so the LV does that. Anterior, posterior base apex, and it has torsional fibers. The RV can do base to apex. It's actually pretty good. You guys will of course will see by echo, uh, that your basillar segments generally are better than your apical segments for the RV. RV does this. RV's pretty good at anterior posterior, but it doesn't have circumferential fibers. It is not as well evolved, uh, so to speak, um, Darwin was less nice to the RV so it doesn't have circumferential fibers so it cannot do torsional contraction that leads to problems, uh, and. Uh, and in terms of contractility, it is not as robust and when you start giving it a lot of pulmonary insufficiency, you go from what is shown on the left here, this geometry where you have the acorn shape or bullet shaped. I'm a pediatrician, so I don't like bullets, but you've got more of an acorn shaped. Left ventricle and a croissant shaped. I'm very Dr. Adler knows me well. I'm very food based, so this is a croissant shaped RV where you have an inflow through the tricuspid valve, a body and an outflow, whereas the LV of course just has an inflow and outflow. There is no body of the LV. Look at B when you start dilating it, and this is especially important for our EP colleagues that Ashish will go into in a minute. You get this water balloon like dilation of the RV that completely distorts the architecture superimpose on that what you see on the right, which is if you start increasing the afterload, if they have residual pulmonary stenosis or if we put a new pulmonary valve in them and then we ignore it or they don't come back for 20 years and then they have, you know, 3.5 m per second across their pulmonary valve, the RV hates. and cannot because of the fiber differences cannot is not as resilient to the effects of afterload compared to the left ventricle. So we need to address both the pulmonary insufficiency so we don't get these geometric changes to the RV and we need to address the afterload. This is from the same paper. This is needlessly complex for who gets a new pulmonary valve, so we have simplified it to this, this table where for, for the imagers in the audience and for the others we look by MRI or by CT and we have a protocol to be able to do this by CT. RV diastolic volume indexed to body surface area of more than 150 mL per meter squared and we will um we will do this to ideal body weight if we need to. RVN systolic volume index to BSA of more than 80. restoration more than 160 milliseconds, uh, your RVEF being lower than 47, um, that's Boston. Many of us can consider a normal RVEF 44 or 45 to 60%, but Boston likes 47. LVEF less than 55%. If you have sustained. Tacky arrhythmi is related to right heart volume load. Many of my EP colleagues tell, tell me, well, how can we tell you that it's exclusively related to the right heart volume load versus scar line? So that part is nonsense. It's just if you have sustained tacky arrhythmias and it's either atrial or ventricular. If you have a big RVOT aneurysm, that's super subjective because many of these transannular patches really do have patuless outflow tracks. If you have an RV outflow tract uh obstruction where your RV systolic pressure is more than 2/3 systemic. The mantra for us has always been more than half systemic, uh, but now people are saying, well, maybe we can go a little bit more. That of course flies in the face of this data that says, hey, the RV really hates afterload. So we will generally in our area, uh, Doctor Senny and I will generally recommend if we start getting more than half systemic RV pressures, generally we'll. Start to notice some dysfunction in them and there are a couple other things that we usually have. So we don't generally go with the 70% or 2/3 systemic we generally go more than half systemic. If you have severe branch pulmonary artery stenosis, moderate tricuspid regurgitation, or more, or aortic regurgitation, those are indications for pulmonary valve replacement. Um, so in my last three slides before I hand it over to Doctor Senny, um, how do we replace the pulmonary valve? Well, I don't think we have any surgeons in the room, but in, in fairness to our surgeons, they have excellent results with pulmonary valve replacement. Uh, they really do, especially if you have residual RV outflow tract obstruction, if you have a dilated or patuus or aneurysmal RV outflow tract, if you need to repair the tricuspi. They're a plethora of reasons to go to the operating room, and they have excellent results. If there's a residual VSD, those sorts of things. The top left is a paper. This was just published two years ago or so. It's a single series of many hundreds of patients from the largest surgical center in the UK, the Royal Brompton Hospital in London, and their surgical survival. Over 15 or I'm sorry, 18 years there was essentially almost 100%. It was like 98% of their patients, hundreds of patients, pulmonary valve replacements survived, but the type of valve that they used matters, um, and you can see the, the end. Here, but the, the, the perrymount valves tended to do better and even some of the Hancock valves tended to do better than the mosaic valves. Um, homographs though really didn't do as badly. We tend to to have a pejorative, uh, feeling about homographs, um, uh, but. But they put in about 180 homographs and their freedom from reintervention by 18 years was almost 80% so that's pretty, that's pretty darn good. So don't discount surgical pulmonary valve replacement. Uh, what's coming up very briefly, tissue engineered valves, uh, this is being looked at for congenital heart disease patients as well as acquired heart disease patients, um, and, and, and you guys have probably, um, you're, you're probably well aware of this. What's exciting in our field is that these can grow with the patients in, in some cases. So you're taking decellularized um collagen. Uh, type scaffolds and adding stem cells onto those from the patients, making them biologically active but non-immunogenic, and they can grow with our smaller patients, which will be great and may calcify less, etc. just wait 10 to 15 years we're we're going to see it in our practice lifetime. So for our structural colleagues like Doctor Adler, what about our trans catheter pulmonary valve replacement? Um, we had the melody valve that that Philippe Bonhofer developed in 2000, um, in at Necker Hospital in Paris and, uh, also at the Royal Brompton in London that was approved in the United States in 2010, um. The Sapien of course was approved in 2006. Harmony was approved in Europe in 2017, but not in the US until 2021. So we have about 4 years of Harmony valve data and then Alterra, which is the adaptive priestsent or just a scaffold where you can implant a sapien within that, um, was was approved after that. But I, I will show you that we have less good data for Alterra and congenital heart disease, so we here locally do not use that. Um, we use it to relieve conduit stenosis or RV outflow tract obstruction, um, and, uh, and we can put, uh, some of these valves, including both melody and sapien in a valve and valve type, uh, situation, um. This is the melody valve. It's been implanted in over 18,000 patients globally. Um, it is exclusively for use in in a conduit or in a valve and valve. Uh, the sizes are more limited than Sapien or of course than Harmony. Um, it is balloon expandable, um, generally because the platinum meridium stent is not as strong, we often will pre-stent. The conduit or other things with with a Genesis XD stent or something that's that's a biliary stent that that's that's more rigid and provides more of a a good support so we don't get stent fractures. There does seem to be an increased risk from these bovine jugular venous valves that are sewn into the stents. The endocarditis risk is higher than a surgically placed valve and it's probably a little bit higher than the sapien valves. What is the harmony valve? I was gonna show a video of the implantation, but we're not gonna do that. This is, uh, again a um. This is a self expanding uh stent that actually comes out like a butterfly. It expands when you put it into the um you can put it in annular or supra annular position um and it can expand uh at the bifurcation of the PAs and down into the RVOT with the valve being centered in in the middle. um this is a night and all stent and houses a porcine pericardial tissue valve, um. And as you release it, it flares out into the into the uh distal MPA and into the RV outflow track so the scaffolding or flares hold. You can see in the bottom it comes in a 22 millimeter or 25 millimeter. Um, we've only ever used the 25. I, I think that the company's finding really very few people are candidates for the 22 millimeter, um, which is both longer and smaller, so it's just not as good a valve as the 25, um. Uh, what we worry about is what Doctor Patel worries about, which is that we're gonna cover up, uh, uh, uh, nises for VT and that these tines or that the flares of the scaffold are gonna induce more VT and indeed they do, um. Uh, but generally that's pretty well tolerated. Uh, this is my last slide. I'm gonna turn over to Doctor Senny. We have excellent freedom from trans catheter pulmonary valve thrombosis. This is a new study just published last month, uh, from Harmony, um. So we have very little in the way of thrombosis. There is some halt and ham, which you guys deal with in this building associated with this, but what we're finding is it, at least early on, it doesn't seem to affect valve function all that much. Um, it may lead to, uh, or it may get better all by itself, um, and most centers are not scanning their patients into oblivion, um, and radiating them and then putting them on oral anticoagulants. We're really looking at echo derived gradients and and trying to to say if we're seeing more stenosis over the 1st 6 weeks to to 3 months that we're putting those subset of patients on oral anticoagulants, not just on aspirin. There's very little endocarditis thankfully, 3 year less than 4% rate of of endocarditis, and overall survival is excellent. Um, there is some, uh, for Doctor Adler, especially some coronary compression that that has happened, um. Uh, and, uh, I think it was not well recognized. um, there's a report that I was gonna show, um, that I can't show now they've, they, they've amended their report that looks like this, um, the, the, the reports where they give the, the engineering analysis, um, but now they actually draw in the coronaries on the report, um, which, um. Uh, we'll show tomorrow morning at our conference for these patients that are having their interventions tomorrow, um, because coronary compression has happened in a couple of patients, um, at, at UCLA and at Stanford, um, and in Boston. Um, but it, it has been relatively, uh, uncommon, but people are recommending, uh, coronary angiography after you initially deploy the first, uh, set of, uh, of scaffolds up top and especially before you release the whole thing just to make sure that you don't get, uh, significant coronary compression, um, uh, now transitioning to EP. Um, uh, this, there is more, uh, there are more ventricular arrhythmias after implant. Alterra, as you can see here, had significantly more than, than harmony, um, and you had longer hospitalizations. Uh, what we typically. See, and this is from data that was just presented in February. We have much less in the way of VT after the 1st 12 hours, and most of it is gone by 3 to 6 months. Most of these patients do end up on beta blocker for that short period of time. But the ventricular arrhythmias get better and actually go to lower levels than baselines. So doing this initially we thought, well, we're not sure that we're making a big difference in their lifelong arrhythmia risk. We couldn't prove that, um, but Paul Carey from uh from UCLA, his data is showing that regardless of what kind of pulmonary valve you're putting in, although it's much better with harmony, as you can see in that B uh slide, we're reducing their risk of ventricular arrhythmias long term. So Uh, the EP considerations in uh terology of fellow patients, uh, as previously mentioned, the arrhythmia burden in repaired trology is not insignificant. Um, it continues to increase as patients age, uh, both the atrial tachyarrhythmias as, as well as, uh, sometimes, uh, the fatal ventricular tachyarrhythmias. Um, in addition, the atrial arrhythmias, uh. Uh, like they are majority of the times they are of intra intra reenter kind of arrhythmias, uh, atrial ca tricus, uh, dependent, it will flutter, but atrial fibrillation also as patients age, uh. And beyond 50 years of age, almost every 2nd patient has some um some form of arrhythmia, but the holy grail of what we want to prevent is sudden cardiac death, um, which is the risk of sudden cardiac death in these patients. is about 2% per decade, uh, but it increases by 2 or 3 folds as patients are more uh age beyond 25 years of age and this is what we see in our clinics, uh, in our ERs and hospitals. Uh, most of the times I think this can be prevented if it is recognized and most of about 90% of the times it is monomorphic kind of VTs. So a lot has been learned about the type of ventricular arrhythmias in this population, uh, the conotrunal uh abnormalities, and as I said that about 90% of times it is monomorphic, and that's where our EP colleague. Can really help us. There is about 10% of the times when it is related to myocardial fibrosis, and that's a polymorphic kind of VT which is different, but that is a minority of the populations. Uh, the monomorphic VT is a reentrant. Type of arrhythmia and just to refresh our memory about reentry uh and I'll I'll show why it why there is a setup for this monomorphic kind of arrhythmia. So if we have healthy tissue and we have an impulse coming from atrium, it reaches the ventricle at the same time, but. If we have if we have substrate where there are like because of progressive remodeling and if we have an appropriately timed PAC or PVC and that goes through the areas of slow conduction, it's set up, it is a perfect set up for reentry and that's what we see in these patients majority of the time because if we look into this cartoon here, there are 4. Anatomical isomusses which have been identified in patients of tetrology. The first one is uh the one between the RVOT patch and the tricuspid valve annulus. Um, the second isthmus is between the RVOT patch and the pulmonary valve venulus, which is less nowadays now that we perform transannular patches rather than ventriculotomy. The 3 critical isthmus is between the VST patch and the pulmonary valve venulus and the 4th 1 is the VST patch and the tricuspid valve venulus. Then there are other minor forms um which are not very frequent, but the isthmus 1 and isthmus 3, these are the most commonly encountered uh ventricular arrhythmia uh VTs in this population. But to add to the complexity, as Doctor Ellis mentioned, all tetralogies are not the same. So there is a lot of heterogeneity in within terology also. We can have just PS, uh, so this is a cartoon which shows different kinds of surgical repair. So having an operative note before taking this patient, these patients to the EP lab is, is paramount. Um, the, uh. So we can have just a VST closure in patients with uh with out outflow tract VSTs or we can have just PSSD patient getting the harmony valve tomorrow. We can have tetrology and then we can have extreme forms where we have placed RV to PA conduit or done raselli type of repairs. So in all these different varieties, there are different, uh, the red shows the. Critically uh uh like uh uh the anatomical um isthmus which is a Like which is the uh uh set up for re-entry. The yellow patches are the surgical patches and the gray areas are the scars. So every uh electro anatomical map in the EP lab will be very different from uh two patients with tetrarology. So, the proposed pathogenesis of ventricular tachycardia in about 90% of the patients is that the substrate is determined by initial anatomy and the kind of surgical repair in these patients. Um. And, and, and over time because of progressive degenerative remodeling, these, these patients develop um like areas of slowed conduction because of volume pressure overload QRS prolongation and when we have triggers, uh, like an appropriately. Timed PVC or PACs or non-sustained VT that that can set these patients up for ventricular tachycardia which the whether it is going to be hemodynamically tolerable or intolerable will depend upon the cycle length of that VT. So, so this is where we, we, we uh kind of put all things together like trying to eliminate the sequela from the initial uh repair and uh and trying to make things better and altering the lifetime trajectory of these patients. That's why um sort of timing the pulmonary valve replacement is so critical in these patients and trying to avoid uh sudden cardiac death. Uh, as, as mentioned before, um, like. The timing and as well as the type of pulmonary valve replacement, whether it is going to be a surgical pulmonary valve replacement or a trans catheter pulmonary valve replacement has to be a well thought out decision because we have now newer generation of balloon. Expendable as well as self expanding valves, and we do not want to make our EP colleagues inaccessible to those critical substrates or critical estimics so that we are unable to, unable to ablate them. Um, So over time we have learned a lot from these patients. There we have identified there are multiple demographic, electrocardiographic, as well as hemodynamic and other risk factors which increases the odds of appropriate ICD shocks in these patients, but There is no one risk factor which has stood out. We have developed some sort of a broader outline with a composite scores which include if a patient had a palliative shunt. So surgical era matters if a patient had non-sustained VT ventriculotomy incision, and the ventricular function. Um, both the right ventricle and the left ventricular dysfunction is a, uh, is a very significant risk factor for development of VT and uh sudden cardiac death. So, uh, so we have, we try to categorize these patients as low, intermediate and high risk patients based upon. Those risk scores and although not perfect, but these risk scores when applied in real-time patients, the Kapln Meyer curves are certainly different in low risk patients versus the high risk patients as far as the freedom from appropriate ICD shocks are concerned. So again coming back uh to this like there is a new school of thought that when we are going to implant pulmonary valve replacements, should we take these patients for a pre-procedural EP study uh so that we can have an electro anatomical map and try to obliterate those. Substrates for future development of VT and alter their lifetime trajectory of these patients. The jury is still not out, um, but it is something that's why we put all our minds together when we plan such procedures uh in our conferences. Um, what I, what we go over is the demographic details of these patients, the clinical history, there is syncope, um, which is very. Syncope palpitations, it's like a very suspicious. That is a very, that is a red flag and electrocardiographic and ventricular mechanics and try to put all these aggregate of all these risk factors. If it is a low risk patient, it is OK to have annual surveillance and they can go on to have pulmonary valve replacement without. An EP study, but in a high risk patient, obviously ICDs are there, but in an intermediate risk patients, I think doing an invasive uh EP study is uh can sort of uh help sort out uh the high higher risk patients than the other patients. So our broader sort of mental framework at the time of pulmonary valve replacement is to consider the role of VT ablation, either in the EP lab or also in the surgical OR like we can do if we have. Good electro anatomical map we can do as well as epicardial ablation in the younger population with good functions and minimal scar, whereas patients with older who are relatively old older with ventricular dysfunction and extensive scar, we lean towards placement of trans venous ICDs in those patients. So we still continue to learn from. A lot of these, and uh it's, it's not done like we don't have class one recommendations. Uh, these, uh these uh class, they are at best class 2 A recommendations with level of evidence maybe B at this time. And that's with that I end. Thank you, Ashish. Thank you, and I think that's the, the, the, the holy grail is really trying to figure out how we can protect our patients both hemodynamically and electrically, um, and the, the, the ICD decision is, is not easy. I've, I've lost two patients in the last two years that didn't meet criteria and that had, uh, probably a rhythmic deaths. Um, neither were tetrology, one was a single ventricle, one was Epstein's, but this question is still going to, to face this entire population, um, and. So, uh, the, the last thing I'll say is, uh, Ashish and I are very, uh, are, are, are thrilled to be working with you guys more, more closely now, and we are especially grateful to our structural heart team, uh, to Doctor Adler, Doctor Treia, Doctor Summers, um, and to our EP team, um, uh, where those services and, and, and Doctor Robertson too has been an exceedingly good partner with our congenital heart disease patients and is an excellent cardiac imager and clinician. Um, we, we value, uh, our, our work with, with, with our EP colleagues, um, uh, and with our structural heart colleagues, and we look forward to working with you guys more closely. Uh, uh, 11 quick thing, uh, as Doctor Sandy was just saying, VT in these patients is going to be handled the way you'd handle it in any other adults. So I wanna be, be clear about that if you're called about VT and a. Patient with tetrology, you're gonna follow ACLS you're going to do exactly what you would do with anybody else. Um, it's not, uh, it's, it's not different how you acutely manage them. The question is when the dust is settled, how do we, how do we proceed at that point? So, um, so you don't have to call and say should I shock this patient. That's not, you know, that's that that is something that that you guys are, are, are exceedingly good at and you don't need our input on that in in the in the heat of the moment. Good, thank you again. Published April 8, 2025 Created by Related Presenters Alexander Ellis, MD Cardiology Children's Hospital of The King's Daughters Ashish Saini, MD pediatric and adult congenital cardiologist View full profile
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