In this Grand Rounds conference sponsored by the Department of Cardiovascular Surgery at the Icahn School of Medicine at Mount Sinai, Ali N. Zaidi, MD, provides an overview of congenital heart disease and its manifestation in adults. He discusses the earliest medical descriptions of congenital heart disease and “blue babies,” as well as early surgical procedures. Advances have accelerated over the past four decades, and Dr. Zaidi discusses how better outcomes have improved survival rates to the point where there are more adults than children with congenital heart disease. However, these adults are faced with long-term complications such as arrhythmia and heart failure, and only a small percentage receive treatment. Dr. Zaidi discusses the importance of transitions of care and the latest procedures including mechanical support and transplantation. He also provides a look ahead to innovative technologies and techniques such as 3D printing to model the patient’s heart and personalized implanted devices.
Today, more than 90 percent of children born with congenital heart disease live well into adulthood. Under the direction of Dr. Zaidi, the Mount Sinai Adult Congenital Heart Disease Center serves the medical and surgical needs of this growing population of adolescents and adults.
Good morning, Ice. Good morning, everyone. Um, I have a pleasure of introducing my friend and colleague Dr Ali's 80 Who's the director of our adult in general? Uh, surgery adults in general program here at Mount Sinai. Um, uh, in about three minutes or so, I want to give you, uh, some background on this extensive experience. Um, he completed his medical school at Aga Khan University in Karachi, Pakistan subsequently, uh, combined completely combined internship and residency in internal medicine, pediatrics and pet state. Hey, was a chief resident there following his residency, Um, he completed a combined fellowship in pediatric cardiology and adult cardiovascular disease at Nationwide Children's Hospital in Ohio, which is one of a leading program in congenital, uh, cardiac disease. Um, he subsequently completed additional specialized training in adult congenital heart disease and palmer hypertension. Uh, most of us have our board in maybe one or two specialties. Ali has, uh, six board certifications. Ah, he's born in pediatrics in pediatric cardiology, in internal medicine, adult cardiovascular disease, adult congenital heart disease and adult echo. Uh, he, um after completing his training, uh, nationwide. Um, he was a director of research there, and in January of 2014 moved Thio Mont. If you're, uh, medical center where he was a director off their adult congenital program, Um, I was thrilled when we're able to recruit him to Mount Sinai, Uh, where he started here in January of this year. Um, the adult Congenital Heart Association is the body that accredits programs in adult congenital heart disease. Um, there are 33 programs in the U. S. Uh, there's only one right now in New York, which is at N Y. U um, at least started in January. Within about eight months, we've submitted, uh, application for the accreditation of the program. And it's certainly all thanks to his incredible lightning speed and an effort. Um, we're very optimistic that with the support off our adult cardiac surgery program, uh, that atoms are those cardiology program on Dr Fuster, we will be ableto receive that accreditation. Um, Ali will give you some background on where congenital heart disease stands today. And, uh, please welcome him for thanks so much. Good morning, everybody. Thank you for having me. Uh, Peter and Dr Adams. Ani. Thank you. Thanks for having me think I managed Thio can you still hear me? You know. So, um, so Peter's right. I have an adult. Congenital doc. I just took a long way to get there, which is probably not very smart, but it took me a while. My dad's a surgeon, by the way, and he keeps telling me that I had 10 years of both grad training, and I'm not a surgeons have done something wrong somewhere along the lines, you know, here we here I am. So what I thought I'd do here is that really give you a flavor of adult congenital heart disease. So over the years and where it stands today, a t least here in the United States and and and and in Europe and then sort of take you through a journey to the ages. And this is a little bit of a historical initial, maybe 10 or 15 minutes, and then I'll give you give you some data. So no disclosure. Is this really what we're gonna do to talk a little bit about the history of congenital heart disease? It's a little, um, it's a little nerve wracking stand, you know, stand in front of surgeons and cardiothoracic surgeons and giving you a history of congenital heart disease surgery. Because really, that's what it is. That's really how the the field grew. I'll talk a little bit about transition and then Hartfield of transplantation, which is really sort of raising its head in adult congenital heart disease and then really, what lies ahead. So he will start with the beginnings of congenital heart disease and always show these initial slide saying, Well, when did congenital heart disease start? So when did when did beasts? So I always able It's congenital, right? We started pretty early on. You know, the first day of life is sort of where it started. But when did we start hearing about it? It's a DaVinci. Now that he had it. And not that hopefully this will play. Oops. Some. Well, I was gonna say with Divinci, and I don't think this is playing on this is that Divinci actually drew the heart and he actually drew the fetus. And what he did was he drew anomalous coronaries in his in his paintings, which I thought was fascinating in the 17 hundreds. He was drawing anomalous coronaries again, back, back several several 100 years ago. But really where in general heart disease was first sort of made its landmark. I think this is stuck. Here was Neil Stenson. Now you might know Neil Stenson. I apologize. This is, uh, not actually showing all the pictures, but, um was was in 16 71 on what Neil Stenson did was that he described really, what was tetralogy of fellow? But Neil Stenson is not known for tetralogy of fellow. He's not known for that. What's he known for is the Stenson's paraded duct. That's what he's known for. But he's the first one who describe a VSD RV, Alfa Tract obstruction and our aviation and misaligned aorta. But he never he never got his name associated with. This is 16 hundreds. So I was talking about Matthew Billion. We. So he drew the transposition hard in 17 hundreds. But I'll bring you to England. I'll bring you England. That's Charles Dickens's and Wise. Charles Dickens's associate with Kendall Heart Disease is because, well, the fascinating story behind him is that the first Children's hospitals that started coming into play the Big John's hospitals really the three the Great Ormond Street in London, uh Jones Hospital in Philly, and then Boston Jones hospitals were right around the 18 hundreds, and Charles Dickens's was actually one of the the names behind great Ormond Street. He was a really a financier to great Ormond Street Will, which is really what became the home fucking jail heart disease, uh, in Europe. But in the late 18 hundreds, you've heard of him, Arthur fellow, and he's the first person who described the four features off tetralogy of follow. But remember, Stenson did it 100 years ago, but never got his name associated to it. But fellow did, and that's what stock was tetralogy of follow in France. That's where he got his name and then William Osler uh, in the in sort of the late 18 hundreds, early 19 hundreds in his book The Practice of Medicine, which is really considered the gospel for medicine. What did he write in 18 97? And there is There is. There is a section on circulatory diseases and a five page chapter for the Congenital Afflictions of the Human Heart by William Osler. Five pages. That's Ah, that's what is written for control heart disease. Um, and then this is in the early in the late 18 hundreds, early 19 hundreds here in New York at New York, Presbyterian just down the road, the Children's Hospital. And this was Emmett Holt, which who was was a sort of a big name in pediatrics. And what he did was he talked about the congenital anomalies of the heart and you can see there. And this was, in essence, a seven page chapter for the congenital anomalies again late 18 hundreds early 19 hundreds. People are talking about it. They're writing about it. There is scandal, heart disease on the horizon. But here's Here's our bill Roth and I like this because he's a you know what? Bill Roth is a general surgeon, and this is what he said. Uh, in the early 19 hundreds, a surgeon who tries to suit your heart wound deserves to lose the esteem of his colleagues. This is like performing an operation to the heart is a tantamount the act of surgical frivolity. This is Theodore Bill Rods, which I thought was very interesting, and I said, My dad's a surgeon. I ran this by him, and he's like, he's like, I don't believe that this this couldn't happen as I know that he said this back in the day. So again, congenital heart disease, congenital heart surgery was being talked about 18 18 hundreds. It is an ailment that is described but is described as not compatible with life. It's not compatible with life. If you have control heart disease, the chances are you're gonna die. Canada early, 3rd, 19 hundreds. And this was fascinating because the regions of control heart disease in the in the 19 hundreds really came from women in cardiology and that that, I think is is again historical in some ways because she was in Montreal and she wrote in 1936 and Atlas for congenital heart disease again, there's no surgery at this point in time. There's no echo. There's no memory, she wrote, and in essence, this. She was writing on pathological specimens. But what happened was that when she wrote this book at Montreal, she was told that she can't practice. She can practice, is a cardiologists, and she was asked to actually be in the pathology lab. She retired that year. She called quit. She retired. Then she died A few years later, James Brown in London, 1939 and this, I think, was fascinating. And what he wrote in this book was that people with Children control heart disease remains sheltered in their homes. They're incapable of sustained activity there, so incapacitated that some have not even walked their under developed. Some are retarded mentally, they don't have the education. He's describing congenital heart disease patients in 1939 in London. But what I thought was really fascinating. This is at the bottom, and those of us who do adults in your heart disease now is that the subject of great affection. There's still the subject of great affection from their parents. They can't do everything but there. And what happens is it is peculiar to note that both parents still accompany the child to the clinic within those of us who do adult control. Heart Do we still see this did this day? Mom and Dad are coming to clinic. I think it's interesting because they were writing about this back in the thirties. But when it's surgery start and this is why the paradigm shifted. This is where congenital cardiac surgery comes to the fall front, and this is where I changed what the beginnings. And I think this is the first surgery for control heart disease that made its mark. The PD allegation. This is done in Boston in 17 in 1938. And this is Robert Grosser of Boston Children's. He did it. He was the first person who did it. He did. A PD a legation on was a fascinating story about this gentleman is That's the That's the child. He did the surgery and that's she went on to live until she was in the in her eighties. But what's fascinating about this story is that Robert Growth was actually the resident. He was not the attending off record. The attending of record was Bill, Lad, the lad Operation and Bill Lad was actually not in the not in the hospital that day. So Robert Gross actually did this without lad being there. I'm not advocating this for anybody. Those if you're sitting at the back or residents, he did it without Bill lad knowing Robert Gross got fired for three months and he was brought back on faculty. Once they said, Well, this is something that says this might change the flavor or where we're gonna go with control. Heart surgery the same year it happened Dusseldorf, Germany. Docile OPD allegation was done. But Robert Gross his name is associated with it because this gentleman who did it actually did the surgery. But his records got burnt in a fire, so it never actually made it out that he had actually done it. Sweden Coordination of the aorta outside the heart. That this is a This is a landmark surgery. Clarence Craft. Ford did this. He did the surgery. He did. Basically what was at that stage was considered a subclavian turned down on Di did co optation of the co rotation repair. But the landmark surgery that got got the flavor going for Kendall heart disease was this was in November 1944 which tetralogy of fellow surgery. And this is a fascinating story that most of you must have heard about. This is Alfred Blalock. This is, um, this is Helen Taussig. And this is Vivian Thomas. This is Johns Hopkins in Baltimore. And this was a fascinating story because Vivian Thomas was actually taking care of these blue baby. She was a pediatric cardiologist in the 19 forties in in Baltimore, and she knew there was something wrong and then she was like, Well, we got to do something. Blaylock was at Vanderbilt and he moved to Johns Hopkins and Vivian Thomas was actually a carpenter who was working in Blaylock's lab, and he was sort of designing pulmonary, uh, mechanics to pulmonary blood flow at that point in time. But this is what they said. They said, Well, you have tetralogy of follow. You can't get blood up into the pulmonary arteries. Here's the VSD this RV Alfa tract obstruction can't get blood out. We're gonna do a shunt. We're gonna bring the subclavian artery down into the left for me. Are you gonna do Bt shunt? And that's what they did. But this is the cartoon that was done. This was the child. This is a fascinating O R O r picture. This is Blaylock that's operating. And this is Vivian Thomas who's standing right behind him, not operating, but actually guiding him to do the surgery at that point in time. So Vivian Thomas was actually in the O. R. At that point in time, helping Blaylock do that first bt Sean and what was really fascinating also was Blaylock at that time had trouble getting anesthesia for this search for this operation. And he actually went to Austin Lamont, who was the chief of anesthesia Hopkins. And he basically said, I'm not doing it. I'm not putting that child to death. Not gonna do it. So he would. Then when? And he actually got Merel Harmel, who was a junior faculty member, to help him do the anesthesia at that point in time. And this became the bt shunt, and that was 1944. It was really fascinating. Was this our report? I got this from the journals and it took me a little while Thio to scan it in and get into this What Blaylock said in his war report, the patient stood the procedure better than I thought better than I thought. It is interesting that cyanosis did not appear to increase very greatly. However the child survive, So he was going in thinking, Well, we're gonna take a shot at this. Probably child may not survive, and that's the That's the hand drawn drawing from Blaylock himself. The Bt shunt that he did that he did the take down the about the shutdown to PS But in the forties, what this procedure did for Kendall heart disease was the twitter of today. This is what it did. It made the headlines. It made the headlines in the 19 forties saying, The blue babies, they're gonna be saved. And this became a major landmark surgery at that point in time. Um, but what people don't know is that that first trial actually died within five months. They died within five months, but they kept doing the BT shot because this is the first time that these blue babies were being were saved. And over the next five years, many BT shots were done. Only about 40% of them were surviving. But the question still remains. Can you get into the heart? Can you get into the heart and again, do cardiothoracic surgeon? I mean, this is in the 19 forties 19 fifties and you was was the concept of the heart lung machine, the heart lung machine that came on in the 19 fifties. This is John Mary Given, But what's fascinating about the heart lung machine and and and John Given was that when he did, his first surgery was in a It was a congenital case. His first case on the heart lung machine was an ASD closure that he did. And this was done in 1953. And this patient actually did fine. But his next five patients died. And in that stage, uh, John Given was like, This is not this is not the seven right direction. Let's not do this. Which then brings me to Minnesota. And this is Walt and Little a High. A landmark cardiothoracic surgeon in Minnesota who then said, Well, if we can do this and we have done this before, we're gonna do this in a congenital heart patient. This was a fascinating again, a landmark case. This isn't a little bit of an older kid with tetralogy of follow. Walton. Lily. Hi. Uh, Dr Gordon and Dr Cohen at that stage got together and said we're gonna do open heart surgery. We're gonna go in, and we're gonna fix this. Um, I just said that That's fine. This is the O. R. Picture. And I think this is fascinating. Fastening picture. This is this is them operating. This is them operating. But would you also see is a second table. You'll see a second table that's going on at the same point in time. And this is so what did they do? Was cross circulation in the O. R. Doing this, which is patient Bump that. And that's what that's what they did in the 19 fifties. This isn't this was fascinating, because when you actually look at the O. R. Record, this is all that he did. He actually went to the Chiefs, the chief chief officer at, uh in Minnesota And this He said this is what he wanted to do. And he quoted a 200% mortality risk. When you look at her and think about everything, 200% mortality risk and that's what he did and this is what they said by all means, Walt, go ahead. This is this is this is a 1954. I thought this was fascinating because this this kid survived. But that was sort of the first open heart complete repair for tetralogy of floor That was done by Walton Lily. Hi. Late fifties. This is the doctor sending with switch. So this is within atrial switch. This is a transposition RV to the aorta l v to the pulmonary artery, and this became a landmark surgery. Well, what they did was an atrial baffle. This was modified by Dr Mustard in Toronto. And what that really means is you're doing atrial baffle in the 19 fifties and 19 sixties. So these kids were then surviving transposition and then brings me the 19 sixties, where this was the first time that interventional cat that raises it said Thus far, it's all surgery. So where was scat at this point in time? And this was This was Bill Rash skin at Chop, where he basically said, We can Palley eight These kids, we can value these infants because at that point in time, the surgeries that were being done were still being done. A little bit of an older kid, So not the really true infants or the new units. And what Bill Raskin said was, Well, put a catheter into the heart and we'll create an atrial septal defect. We gank. And those if you ever go to a pediatric cath lab and see this procedure is one of the most, uh, jaw dropping procedures because Catherine goes up, there's a big balloon. The baby's about this big, and they actually yank the yank, That catheter, the whole baby shakes You got to keep the baby down because it create They create that asd to create that sort of shunting in the heart. And this is what Bill Raskin did in 1966. 1st interventional cath procedure. And since then, there were PD a ligations and devices and so on, so forth. I'm gonna bring you to the Fontane in the mid 19 seventies. Single ventricle physiology, single ventricle physiology. Thus far, not Palley ated on Fontane basically said, Well, this is strike us with atresia. This is blue blood coming from the I V C. You can't get into the RV. So what he said was I v c coming up. We're gonna take this to the left pulmonary artery. So this is there's a valve conduit here. I v c atrium atrium hooked up to the left pulmonary artery. SBC comes down disconnected to the right pulmonary artery. Blue blood goes to the lungs, comes back to the single ventricle, goes out into the aorta. First time the font and valuation was done, and this has sort of, in essence, has changed modified over the years. But the font and procedure is still to go to procedure when it comes to single ventricles. 19 seventies. Now we're in the 19 seventies. This is in Brazil. Back to transposition. This is, uh, guillotine. Who was? It was a cardiothoracic surgeon. What he did was Let's not do in atrial baffle, but we'll do. An arterial switch will translate. Kate will move. The great vessel will translate it. Coronaries will do the 18 arterial switch. And this is now the go to surgery for transposition. Why am I bringing doctors? 18 up is because once he was all said and done, he actually quit cardiothoracic surgery. Became the health minister for Brazil. And that's how he retired. Came up with this landmark surgery, but he went into politics. At that point in time, we'll bring you the late seventies and bring the prostaglandins. Thus far, these kids, they're still getting surgery. They're still getting evaluated with Cath labs. Prostaglandins comes into the market and this is what sort of changes the flavor because now you can keep shunts open. PD is open and then in the early eighties is hypoplastic left heart syndrome, which, for those of us who control heart disease instead of a life threatening condition. And Bill Norwood comes up with his his procedure. It's a it's a uh um, it's a damos. It's a new aorta that's in essence, being created as a modified bt Sean. And this is, um this was in the early eighties, so now we're in the eighties now, we're in the eighties where we're doing this sort of procedures, but I'm gonna jump about 15 20 years, come to this, which is the melody valve. And the melody valve was the first time a trans catheter valve was deployed successfully in an adult patient. And this was done in London. Um uh, industry developed in Paris and then done and then and really done in and sort of taking forward in England on this was sort of the first time a trans catheter valve was done. And since then, obviously the valves have taken off, which then means to me now. So we're now in in sort of the birth of a C h. D. So the question that comes to our mind is that what I've just taken you through is over the last 60 60 years or so where control heart disease has gone from, in essence, being an ailment that's not compatible to life now doing open heart procedures. Well, what about adult congenital? When does that come in? And in the mid 19 seventies, again at the Brompton in London, the Lady Jane Somerville writes about adults and young heart disease, and she calls it grown up congenital heart disease. Gooch. And that's in 1975. And she wrote this paper, which is which is actually fascinating. It's in The Lancet and and she says, This is gonna be the tsunami that's gonna come and hit in about 20 years in 1975. This is this what she said. And this then moved to Joe Perloff in 19 late 19 seventies and U. C. L. A. The first adult congenital clinic in the United States at U. C. L. A. In 1977. So again, still pretty early on. But that's when they were leaders in the field. They were thinking about adult congenital heart disease. So what about outcomes? I'm gonna shift gears a little bit because the story really starts with the last 60 years. Well, where were we? 60 years ago where we now and this is what it is. It is the most common congenital congenital defect, not congenital heart defect for Children, the most common congenital defect for Children's. If you take everything else cystic fibrosis, you take sickle cell cancers. Congenital heart disease is the most common. The initial goals will get them to one year of life, get them to adolescence and know what we know now is that there survived. They survived to adulthood, and the data is fascinating because in the 19 sixties and do, let's say in the myth out 2000, this is this blue circle is kids with congenital heart disease. This red circle is adults with control heart disease. In the sixties, more kids eighties equivalent two thousands, their arm or adults with congenital heart disease than Children with congenital heart disease. This is national data. This is data that's from the United States, and I'll show you some data here in a second to what we're seeing is that over the last 50 years, the survival per decade has improved by 10 or 15% where we now know that it's over 95%. It's even more than 9% more. About 95% of these kids survive. So these air kids surviving with congenital heart disease. So what does that mean is that we get them to adulthood, We get them to adulthood. Thio, this is data from Toronto and I'm just showing you complex and heart disease. Try atresia, transposition co optation. What does this mean? What this means is yes to survive. But this is a job death. They do die, there is mortality. And what that means is that they die from sudden death. They drive from heart failure and they drive from from other complications, including strokes. But the average age of death is somewhere in the late thirties. This is data from Canada that yes, we get them. But we were dying from heart failure in the dying from arrhythmias. This is data from Europe and just breaking this up not to belabor this, but when they survived their surviving with the burden and the burden is arrhythmias and heart failure. And I'll drive heart failure home in in a couple more slides in the second. Because roughly 25 to 30% of these young adults will have these complications is again the main ages about 30. That's where that's when they that's when that's when the problems start, start to arise. So what does this mean in the United States? What this means in the United States that we have mawr adults than Children? We have more modern and complex congenital heart disease. And now we have two million adults with congenital heart disease in the United States. This is national data. They're out there. These kids were surviving. They're now adults. We know this. So what does this mean? What this means is that this is what they're. These kids were dealing, not these kids. These young adults are dealing with a lot of complications. And those of us who do adult control, heart disease, where my medical hat on and these air. This is what we see. We see the arrhythmias, we see heart failure. We see pulmonary hypertension, these air young patients in the thirties forties, maybe in the twenties, that are dealing with this. The reason I'm showing you this is because I'm gonna come back to this slide in a second. But the question I wanna ask is, Well, where are these patients now? they're two million patients in the United States were in New York City. Where are these patients? Well, this is what happens. They get lost to transition. What happens is thes kids air fine when they're in their 12 15 16 18 year old cohort, they're doing okay. They're running around. You know, Peter and Rag have done great surgeries. They're doing well and they're fine. Fine, fine. But then in the twenties and thirties, they start getting sick, and this is what happens. This is national data. They show up in the G. P s office. They show up in just a general cardiologists office, which is completely fine. They show up in orthopedics office in the dermatology office. They show up in ER s r i C U s because they're not transitioned, they get lost to care, and this is what happens. And this is again data that's out there that shows that these patients getting like this is a fascinating slide. And I'll just show you this that this is the number of patients that are being reported in the United States. Now we think it's two million. This is about five years ago. We without was 1.3, but on Lee, 42,000 patients in the United States have ever touched a adult congenital heart position. Oregon's so most of these patients are still lost to care. They're lost together. And this is just fascinating data because the question then is well, what happens to transition? But why are we know these kids are being seen right? They get the operations, they're being seen in general hospitals across the country. Where do they go then? What happens? And this is the the issue with transition is that we're now. So I'm gonna wear my pediatric had on for just a second. I'll bring it back to Internal medicine. Is that transition means you're gonna move from family centered care. So, Mom and Dad, if a child is sick, be it congeal heart disease be They're gonna go to the pediatrician, they're gonna go to the pediatric cardiologists, they're gonna be seen in a German hospital and then the child grows, grows, grows, grows, grows. Now you're 18 21 22. And that transition never happens. They get lost because now you're moving from family centered care. Do specific individual center again. Now the onus is on the patient. Now the Otis is for that young patient to sort of take care of his or her own health. And that doesn't happen effectively in a lot of centers. Not not just not just in New York but across across the country. This is national data. We lose them. There is morbidity, there is mortality. And this is single center from Toronto again, just showing you one center, one clinic. This is starting when they were six years old, 100% follow up. They go to 22 only 39% being followed. Same center, same same hospital. You have a pediatric Children's hospital, you have an adult hospital and only 39% of patients were followed. And that's because these patients feel good. They're feeling fine. Then they sort of get lost. And this is something we see all the time. This is a patient I saw when I was a nationwide, the highest state when I was a fellow. This is a 32 year old Fontane lost to follow up shows up in the Childrens ER shows up in the Childrens er with shortness of bread. This is a single ventricle. And this is a big rhombus that was sitting in the Fontane circuit. Um, and clearly this patient was in trouble. Was that had a demon society has lost to follow up? Eventually got a hard transfer land again. And this is this is something that we see. So the problem is this. This is this is a national phenomenon. This is not something that's unique. This is a national phenomenon, that there is a bridge. There is a pediatric system, There's an adult system. And there we got across that that that that bridge there's a national data that I'm showing you so well. The question is, I'll show you this in a second is like How we how do we get around this? Well, we now know that adult congenital heart disease is not a pediatric illness. It is not a pediatric illness. It is an adult illness. It is an adult congenital from It's something that we live within adult medicine world in adult cardiothoracic world. So here's a fork in the road and only bringing the fork in the road. I won't get into this for today's stock is pregnancy. These women now get pregnant. Not only are the surviving, they get pregnant, they want families. They want Children. But they want to get through this and again not to belabor this. But when we get into pregnancy that lots of lots of different things you look at and this has become the multidisciplinary approach. And and again, not not for today's talk it, but but But what I'm gonna bring you to is what lies ahead. I'm gonna spend the next 10 minutes just maybe seven minutes or so talking about what lies ahead. And this is that tsunami that that, uh, that Jane Summer will be talked about in London in 1975 that these patients will grow up and they will get into the twenties and thirties and forties and fifties. And this is us, and this is them. And we see this a lot where these patients come in with with with heart failure and arrhythmias. And then they get very, very sick, very young and, uh and and I just took care of. We were taking care of some someone recently. It was a 45 year old transposition, very sick. Gentlemen, that's what happens. Sort of law you got lost to follow up, got very sick, and then eventually it was it was heart failure. So what does this mean? What this means is we innovate. We go forward with innovations, and what this means is that we're now moving towards. Repeat. If we had to repeat operations, what can we offer them? We offer them personalized devices. So now the cats world is coming to a stage where there's a new valve that's being developed and a lot of different per Catania's valves. But this is what's called the harmony valves, and it's a funny name is harmony valve. What they're doing is that they're designing with stem cells and there's a scaffold and it goes through a catheter into the heart, and it's designed to every specific, RV out foot track in this room. So every one of us has a different RV outside tracked, and this is our right sided valve. But this is being developed, so we actually you get the model. You get the stem cells, you get it engineered and it goes in, and it actually models to your sisterly, and I honestly, because it becomes a part of your RV Alfa Track and they said This is now being deployed lead. Let's pacemakers sub Q I CDs. You know all this three D printing big. It's raising its head, especially for congenital heart disease. And this is this. This is a dissipation where we were trying to figure out a bad placement for a right ventricle on Ben. We did this model, and what we could do is we could take out the entire wall of the right ventricle and go to the surgeon and show them and say, Well, this is what the inside of the RV looks like. So when you do the calculation, where will the cannula go? We can actually show where the moderator band, the muscle bundles will said So the surgeons know that going going up front. So So again, this is this is innovation. This is 40 flow. This is Emery, and this is pretty cool. This is a co optation. You can actually see the turbulent red blood. It should all be blue and smooth sailing and and and and lemon are This is a Fontane. This is SBC coming down. I v c coming up. It should all be nice. It should not nice, be nice and blue so we can cheat turbulence now with 40 flow on Emery. So this is all that's that that's that's on the horizon when I spend five minutes and talk about heart failure, mechanical support and transplant because this is raising its head in the world of adult congenital heart disease, you know they see this in 19 sixties. This is landmark surgery. This is not not to do with adult congenital heart disease. But why am I showing you this is because I don't know if you know about this in 19 8, you probably do was Leonard Bailey. This is a fascinating story and is that Leonard Bailey was at Lo Melinda and this was a hypoplastic left heart baby, and he actually did a cross species transplant. Broad probably knows about this is and and he did transplant the heart of a baboon into the child on This was done in 1984 and the child survived for five days, but then had a lot of immunological reaction. But that is the baby post transplant that the baby will transfer. This was in 1984. I never, never, never did again. But why Heart failure And why am I talking about hard play again and again is because we're seeing this in my world now in the nets, where my adult cardiology adult congenital heart disease on is that these patients have heart failure. The problem is, how do we define heart failure for adults in general? Patient Because you might be dealing with the transposition systemic right ventricle. You might be dealing with an arterial switch, a single ventricle. You might be dealing with somebody with shunts and things along those lines. What's really interesting with heart failure is people have tried to define heart failure and congenital heart disease. Over the last 20 years, they're given multiple different definitions and nothing really has stuck. Nothing really, as stocks and not not a believer that but this is fascinating. And I'm gonna take the last 10 years, just last 10 years, data that I'm going to show you. Is that what they're saying and what There's data that we know off, and that is hard feeling. Adults in general starts from birth. So in the sense that you're not gonna be a hard feel that day one but it. Flip flops. If this is your system sys symptom threshold for heart failure right here these patients go up and down and up and down and up and down and up and down. And that's what these patients do from from when they were kids. So we're not dealing with the 65 78 year old with the scheming heart disease we're dealing with 5, 10 15 2030 35 year old with a systemic RV, for instance. And they fluctuate. They have surgeries, have arrhythmias, every interventions they have decompensation. And there's a underlying risk of sudden death that remains in these patients. And it. This is sometimes difficult because the question, then is, well, how do you manage them? What we know is that heart failure admissions have tripled, have tripled in the last 10 years for adult congenital heart disease patients. They keep rising mawr, and more of them are showing up with heart failure and again going back to the data that I showed you that these patients can die from either heart failure, arrhythmia or sudden death. So what can we offer them? This is standard therapies. I'm wearing my adult cardiology had on Gold Director Therapies, CRT. I know tropes, but really, the question is, can we offer them mechanical support? Can we offer them transplant? Is this where what is the data that's out there? So there's roughly in the last decade. This is an adult with you. I'm gonna show you this case. Just show you what the heart looks like. This was a 45 year old that I took care often a couple of years ago had Epstein's at a trick us but valve repairs. She was a school principal doing fine out in the community. But she had asides, and she was being Paris into east every three months. She was seeing her just regular Coriolis out that she was getting Paris and T's. And this is what a hard looks like. Just let this play. This is Anne Marie. Um, this is this is enter. This is both interior. And this is this is when I saw her. This is just to show you what the hard looked like. This is the whole right heart. This whole thing. This is the little LV down here. This this whole thing is still the heart. This is The whole thing is so large. She was being Paris NTC because when they were scanning her, they couldn't get the entire heart. So they were only getting this sort of edge of the TR jet. And they're so there's not enough DRC doesn't make sense. Must be something else. They couldn't get the whole heart in on the echo images. Eventually she got a transplant and is she did find so well, what are the guidelines for adults in general, Right. So what are the guidelines out there? So we have shown you sort of data, but what are the guidelines? This is in 2000 and 12. 7 years ago, first time that somebody from the H A said we're gonna write a statement for adult congenital heart disease and this is again on. I'll show you what It shows that the bottom is that Here's what it says. Most patients with complex control heart disease are not candidates for M. C s. Ah, careful. Preoperative consideration should be done. Individual patients and at me may permits to successful I don't know what that means. I mean, no, I have no idea what that means. That this was the first time it made it, made it out there. This is 2000 and 13 looking in the Journal of Heart Lung Transplantation. They came out with guidelines, but what they commented on was the use of ads. They said, What is the data for VADs in adult control? Heart disease. And I thought this was fascinating. So let me just show you this. So this at the bottom, if you if you look at the sort of the the bar, the bars that have this sort of pattern on it, that's congenital heart disease at the bottom. The other is non Qianjiang heart disease. And over the last 10 years or so, look at the use of M. C s in acquired heart disease verses in Kandahar disease flat. It's flat for the last 10 or 12 years, and that's really been the case. That's and again, the question is, Is this an era effect or not? And these are actual numbers off outcomes with bad At the time of transplant with congenital heart disease, 20 were transplanted. 17 died five removed and look at with it without these air again acquired hard do so the numbers are small. The numbers are small across the country, and there's reasons for this because it's the anatomy becomes a precluding factors. So so So we know this so that that bad zehr there, but it's it's difficult to use them. So can we improve the utilization of ads or mechanical circulatory support? Well, there's now data arising that maybe we need to use technology. We need you to get the image ing. Um, do we need three D printing? And we get our surgeons to the working with the non invasive docks and say, What can we offer mechanical supports? Another statement came out three years ago from from from the h A and this is what they said that yes, you can use VADs with staged D heart failure. You can use it if there's an adequate an atomic and physiological conditions. There's severe limitation of excite. So again, ah, lot off a lot of broad strokes, lot of broad strokes, Nothing that was really hitting home. Um, but that's where we were at. That's where we're at in 2000 and 16 comes transplant, and this is a fascinating side again. I'm going to show you this This is adult control, heart disease. So this is again heart failure. You're gonna up and down, up and down you again, then get listed for transplant. But within listing for transplant There is a nutrition for adult congenital heart patients. Why listed they start dying wild list of this national data. But for non congenital heart disease, If this is your symptom threshold, there is a little bit of a flat line. They dropped, they get transplanted, and then this sort of remain e. I mean, there is attrition. I'm not saying that there isn't, but in adult congenital heart disease they go up and down and then they get listed and then they die while they get listed. So what does this mean? What this means is, if you look at the old classifications listening one a one. B two. This is the reddest transplant. This is adult in general, non adult. In general. What this really says is that yes, patients are getting listed. So this is these are adults in general, patients that are being listed. But there is a nutrition. We know there is a nutrition. They die while they're being listed. They also clinically get worse because the wait is long. So about 25% of them die. They did this meta analysis that looked at the risk factors. And if you look at this hazard ratios renal, They started developing renal function. They were nice use for a long time. Ventilated for a long time, they started having liver dysfunctions again, not substantially different from a quiet heart disease. But 25% of adult control patients die while being listed. So what this then meant was somebody did a meta analysis and I thought this was fastening. This is about just two years ago. They looked at all the data that's out there for heart failure. Transplant 6000 papers for adult congenital that air out that they're being reported on Lee 13 Onley 13 hit the metrics for acquired heart failure Transplant in the sense that you're actually looking at, the guidelines were acquired heart transplants. They can you apply to control heart disease. Only 13 papers that actually followed any sort of programmatic approach. Ah, lot of it was anecdotal was we do this, we do this. Our center does this so and the problem with this was that I think there is an era effect and I'm going to finish her in two minutes. Just trying to explain this is that this is now mechanical support to transplants. You get them to transplant and what happens? Well, this is 30 days. So this is the dark blues congenital heart disease. This is non congenital heart disease Onda. What they showed was aimed. So yes, they have a rough. Of course, 30 days. This is one year, but 10 years out there is the higher 10 year survival for adults with control heart disease. So what does this mean? Does this mean that we are seeing now? The patient's getting transplanted to a 30 40 50 years ago who were initially palliative surgically in the sixties and seventies, and we're really not seeing the patients who are being so as the surgical procedures and the expertise is improved. Well, this was era era data from Europe, and this is just showing you that 1988 to 2000, 2000 and 6 to 2000 and 12 they split it into two cohorts on what they showed was that one year data is now getting better. It's getting better now than what it was 20 years ago. So the data that was out there that one year transplant outcomes are worse might be in. It might have been an era effect. What you're seeing now is that the new transplant data says yes, there are sort of catching up. This is just showing you median survival. Congenital heart disease is 15 years. Ischemic cardiomyopathy is nine, then non ischemic is about 12. So if you get them through, they live longer with transplants. So this is on. I'm gonna end with this primary. This is two months old data two months old data from David Morales in Cincinnati. And this is fascinating because they looked at the era effect. And they said that well, in 2000 to 2000 and 8 2000 and 9 to 2000 and 18 this is all a C H D transplants across the country. One year survival was 78%. If you go back 20 years, it's 86%. If you're transplant in the last 10 years, so again looks like there's an era effect that's happening. The five year survival is roughly is again better if you're in the last eight or nine years, so the survival rates are better. And then just this last slide showing you when you compare them to acquire heart disease. And that's really where the money is that when you compare the quiet heart disease, So the similar similar survival at one year. Now, this is two year old data that if you compare adult congenital to non adult congenital, their survival is roughly the same. And I think this is because now you're actually seeing the era effect coming in, which we didn't before. So why are we seeing it? I'm gonna just run through the showing you that this is a Fontane with with a failing liver. I'm just showing you this because this is something that I mean I've talked about before to is that these patients are now running into dual organ transplants. This is a single ventricle, Fontane with cirrhosis on. DWI actually ended up doing a dual organ transplant on her. So this is on block. We did a few before I came here. So this is something that's happening. So a CH transplant. It's certainly a village. I'm gonna finish here. We're just gonna show you this do slides? I know I got laid. I'm not sure. Oops, sorry. That's my phone telling me they're not understand. Um, here's accreditation. This is what Peter started with. We cannot do adult congenital heart disease. Now, by just putting a shingle up that way, just can't do it anymore. The guidelines are that you have to have a program that has accreditation, which basically means you're gonna work together as a multidisciplinary force and really bring in the experts from both adult cardiology, pediatric cardiology, cardiothoracic surgeon. I do believe that adult control harder disease becomes that link. And that's really what it needs. We're in the process of accreditation this point. I want to thank a lot of you in the room who helped me with this. And I know Robin of all on me. Peter. Dr. Adams, this is gonna eventually come to your desk to um but this is what's happening is that we have to go through site visits now, um, that we have the surgeons, we have the i C. U S. We have the mid level support on. We've got to give them outcomes that we can do this and this is This is where we're at now. So this started in 2000 and 16 in June 2016. We're in the process of accreditation now, and that's where we're going. I'm gonna end this here by saying that that's what I really took you through sort of world wind journey. And this is where Joe Perloff said is congenital heart disease and adults is the future of congenital heart disease and Children. And that's really what's happening is that these kids are now in this world in this world of adult cardiology and adult cardiothoracic surgery. So I'll stop here. So I apologize. I was delayed starting and I had some computer difficulties. But I'll and I'll stop right here. Thanks, guys. Thank you.
