Welcome to heart to heart with Anna featuring your host on a Gorski. Our program is designed to empower the CH d or congenital heart defect community. Our program may also help families who have Children who are chronically ill by bringing information and encouragement to you in order to become an advocate for your community. Now here is an edge or C. Welcome to
the ninth season of heart to Heart with Anna. Our theme nous season is advancements of congenital heart disease and we have a terrific show for you today. Today shows advancements and treatments for H. L. H s heart warriors, and our guest is Dr Edward of a Dr Edward Elbows is a renowned pediatric cardiac surgeon and an internationally recognized expert on the treatment of complex congenital heart disease. He joined the faculty at the University of Michigan, is director of pediatric cardiovascular surgery and became head of the section of cardiac surgery in 1999. Dart Abo ve was inaugurated is the first Helen and Marvin Kirsch, professor of surgery in 2005 and chair of the Department of Cardiac Surgery in 2012. Dr. Bove has received support for his research from the American Heart Association, the National Heart, Lung and Blood Institute, the National Institutes of Health and L. A Duke foundation. He has given hundreds of presentations on heart surgery around the world. He has served on numerous committees, including the American Heart Association, The Society at Jurassic Surgeons, the American Association for Thor ASIC Surgery in the American Board of Drastic Surgery. Dr. Bove A serves on a number of editorial boards, has published over 300 manuscript, dozens of book chapters and edited to books. So Welcome to Heart to Heart with Anna Dr Bove A
Great to be with You.
Well, I have to start by telling you that when I announced on Facebook you were coming on my show. We got a lot of likes and hearts, and people are so excited that you are on the program today. In fact, the woman who introduced you to me, Carrie Van Eck, told me to tell you that Jonah is now 20 years of age and doing great.
Well, that's great to hear. Believe me, it's so rewarding to see these kids grow up to be adults. Can't tell you what
I can just imagine you have been kind of like one of the grandfathers of a chile chest surgery, haven't you?
But you're making me feel old when my patients are now adults and coming back to see me. I know it's, uh, it's a pleasure again.
Well, you certainly have been at the forefront of Rachel HS surgery, So that's what I want to focus on today. Even though I know you are an expert and have done so many other surgeries, I think at least in my world, you are best known for your Rachel HS surgery. So let's talk about the prognosis for babies born with hypoplastic left heart syndrome are Rachel Hs prior to the 19 eighties?
Well, that obviously was a very dark time and essentially the prognosis with that these babies all died. It was uniformly fatal condition when the diagnosis was made, and generally it was made. I think in that era, probably after the babies were born, parents were advised that there's nothing that can be done and that they should just take the baby home to die and clearly thatwas something that's no doubt stimulated many people to start thinking that there should be something we can do. But before 1980 it was a very dismal outcome.
Murray. So what was the turning point for babies born with? H alleges that allowed them to begin to survive.
You know, it's hard to point to one single turning point, I think, quite candidly, with so many things that started to come together, certainly not the least of which is the pioneering efforts of people like Dr Norwood, who first showed that in fact, survival was possible. And this indeed, I think, ticked off a tremendous amount of interest and many centers throughout the country to say, Well, if we can do it once, why can't we do it more often? So it really was the unwavering efforts of the entire team of surgeons, cardiologists, the nurses, the anesthesiologists and, first and foremost the parents who were unbelievable in allowing us to try and make efforts full well, realizing, certainly back in that era that survival was highly unlikely,
right? Right. So what Advancements actually led to the survival babies with a geologist ahead, Dina Barber on my show earlier this season, and she actually gave us a 30 year perspective over congenital heart disease in general and she talked about prostitute land in in the play Lotto Stic shut. Can you tell me a little bit more about those kind of advancements that led to the survival of babies with HHS?
Yeah, I think it was, perhaps, unfortunately, but it was the very mistakes we were making which taught us how to change and then go back and correct him to improve. No doubt, the biggest advancement, at least in my own opinion, was that we began to understand the physiology of a single ventricle heart better because the reality of it, Woz, we didn't really have a good idea what it meant to control blood flow to the lungs to make sure that you didn't have too much to do all sorts of things that we now take for granted. Back then, we really didn't understand. I couldn't remember in the early days thinking that when you did the original palliative operation that higher the oxygen saturation, the better off you must be well. Now we learn how obviously that was wrong. So I think understanding the physiology allowed us to direct our surgical efforts to mimic the most stable type of heart we could do. And then personally, I think the other biggest advance waas the second stage, the so called volume unloading procedures known basically as a bidirectional Glenn or a Hemi fund pan. I think back to the earlier when we finally got patients to survive Stage one. But we really didn't know an awful lot about what to do next. And then we started using second shunts because they'd outgrow the original one, and that was disastrous. And again it was because we didn't understand. But that really met not because the surgery was bad, but because the whole idea was wrong and we did Montanes early in life, and that didn't have a good outcome. So, really, the ability to do the Super Vienna cave at a pulmonary artery connection now really gave us the opportunity to remove the risk factors, stabilized these young Children and allow them to grow and develop to become good candidates for the Fontane without all the risk factors associated with some of the other operations.
Wow, I had no idea that you all learned those kinds of things, and that had such a big difference in the lives of Children with actually just I actually met a gentleman Brian Roth line about for a book called The Heart of a Father, which was the companion to the book that you wrote a forward to for me, the heart of a mother and in the heart of a father. Brian Roth line was actually born in the 19 seventies, and he was form with HHS and had a play Lotto Six Cent and then years later had another play, Lotto section, and he is still alive today. Of course. Since then, he's had a Fontane procedure. But it's interesting that you said that people who had that done prior often died.
Well, they did. Obviously not all did, but the babies would then outgrow the original shunts we put in when they were newborns. Again, we were learning that the smaller the shunt oftentimes better you were not the other way around. And then it just logical Well, if they've outgrown the shunt will just add another one or give them more blood flow to the lungs and this will be perfect. And then what that did essentially would produce so much of what we call a volume load on the ventricle that the ventricles began to fail, even quit. And this is what I meant earlier to say that these advancements were kind of made on the backs of unfortunately learning not necessarily how to do the operation per se, but how to tailor it for this type of physiology, so that we could safely get these Children to older ages when they can tolerate Fontaine's much better.
Wow, Wow. It's amazing to me how you doctors have been able to work on such tiny, tiny hearts and learn what you have said that you can have this success that you have today. It truly is amazing, but we do need to take a quick commercial break. Don't leave yet listeners, because when we come back, we're going to talk to Dr Bove. A. About current trends in saving H. L H is heart warriors will be back in just a moment.
The most common here is why
she always needed a lot of attention. She had strokes,
even though it's a national inclination to withdraw from the CHD community, I think being a part of it, help me be part of the solution Heart to heart with Michael, please join us every Thursday at noon. Eastern. I'm Michael even, and I'll be your host as we talked with people from around the world who have experienced the most difficult moments
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You are listening to heart to heart with Anna. If you have a question or comment that you would like to dress down show, please send an email to Anna Dworsky at Anna at heart to heart with anna dot com. That's Anna at heart to heart with anna dot com Now back to heart, to heart with them. Welcome back to
our show. Heart to Heart with Anna Chauffeur The congenital heart defect community today show his advancements in treatments for Rachel Hs Heart Warriors and our guest is Dr Edward. So I really enjoyed talking about the history of a Chile just treatments. But let's talk about current trends for babies born with hohs. I'm amazed that they can actually do surgery in utero, and they've been doing this in Boston for a number of years. Now, can you tell us what you think about the ability for some babies toe actually receive treatment in utero, Dr Bob?
Yeah, I think to be frank, the jury is probably still out. We are doing this as well here in Ann Arbor, but have an active program. The efforts that are being made for in utero intervention are really aimed at trying to prevent or perhaps minimize, sometimes devastating complications that can occur in certain hearts with H l A jet, for example, if the atrial septum is intact or nearly so, there's no way to get blood out of the lungs and then, therefore the lungs are badly damaged in utero, so that when these babies were born, often the damage is irreversible. And of course, the prognosis is extremely grave. So the thought is putting in utero intervention by opening this septum. In other words, relieving the obstruction or perhaps opening up a thickened valve that is obstructed to allow blood flow to go across the heart and allow growth of the heart muscle. Can this really affect the outcome? I think a lot of the problem arises. Can we intervene early enough on these babies to improve their outcome? Or are we to put it somewhat crudely, sort of kicking the can down the road? In other words, things may be better, but we may still not be able to completely avoid the complications. I think that's an outstanding way to go. We obviously share in the group of Boston who really pioneered this, that this is something we need to be looking at. And I have no doubt that as technology gets better and things get safer, we'll be able to intervene, perhaps earlier and perhaps really improved. That segment of babies were already born with devastating conditions that we can no longer treat.
Yes, it seems like for the people that I've talked to who have gone that route, it's a lot more operations than the babies who have the traditional three stage procedure.
Well, it may well be. I think it depends a little bit on the precise condition that you're trying to affect in utero, and sometimes they may need more operations to try to recondition their hearts or allow some of the chambers to grow. But as I said earlier, I really do think that the jury is out if we're really able to, for example, allow left ventricle to grow better in utero so that we can eventually do a two ventricle repair rather than single ventricle approach. Don't know that we really been able to show that yet to be on
right, right. In fact, when I first heard about this, that's what I thought the outcome would be that those babies would have all four chambers, and so wouldn't that be amazing. They wouldn't actually be born with Angela jest. But now that I've actually talked to some of the survivors, that's not the case. It's just that they may have a better chance for survival, but they'll still have a naturally just heart. Is that the case? In most of the situations that you've seen?
Yes, I think it is, and that's what I meant earlier by saying, Are we kicking the can down the road, or are we really mature, really affecting the outcomes, and I don't think we know. But I think your point's well taken. What we are seeing is that the ventricles may grow a little bit better, but we're probably as of yet, not intervening early enough to allow them to be normal. If I could put it that way, ventricles, that could really support the circulation,
right? Right. Well, it seems that there is another trend, and that is that of the hybrid surgeries. Can you tell us about the hybrid surgeries available for HHS babies?
Yes, hybrid surgery or something that we do frequently here as well. And for those who may not be familiar with it, it basically means that you're sort of doing heart of this in the cath lab as a catheter intervention, such as placing a stent and part of it surgically. So for a baby with hyper plastic left heart syndrome, the hybrid surgery generally means that you're putting a stent in the duct as a way of making sure that blood flow proceeds to the body unobstructed, and then you're restricting the blood flow to the lungs by placing some bands or ligatures, if you will around the two arteries into the lungs. The right left pulmonary arteries. Hybrids are used as the primary motive therapy. In some centers, I think candidly, they were often used in centers where traditional Norwood surgery had poor results and therefore hybrid surgeries turned out to be a safer way and lead to better survival. But I don't think that for the standard risk baby with hyper plastic left heart syndrome, there's really an improvement or better outcome for hybrid surgery. It does, however, play an important role, we think, for the very high risk baby. So, for example, babies that are born with extremely low birth weight, where doing the traditional surgery is fraught with increased difficulties or babies that are significantly premature. And so your concerns about placing them on the heart lung machine giving them heparin, which prevents blood from clotting during that time, are riskier if you will, because you could start to see other complications so that Ah hybrid operation, which allows you to avoid some of that, may well allow these babies to survive and then to grow and develop to a point where they then become better risk candidates for the standard. Norwood So, at least in our own center, what we are doing is we're utilizing hybrid surgeries for the very high risk babies. And then when they outgrow that risk, so when they're premature, he gets them out to say, full gestational age or their birth weight. Very low birthweight gets them up to what we would consider a safer size. We then convert them to the Norwood operations, then so, in essence, an extra operation, but one that we hope will improve survival for that very high risk group.
Okay, well, it seems like the newest trend that I'm reading about is stem cell therapy. Do you feel that this is the up and coming treatment for H alleges babies and possibly even for older hohs heart warriors?
Absolutely. We've made a big investment here of the University of Michigan into stem cell research and have a very active laboratory with number of MD PhD and PhD doing research in various parts of stem cells, from growing heart muscle to growing blood vessels to growing valves to growing conduction tissue. And I often joke with them. I keep saying, Why don't we just grow a heart? Because we got all the parts,
right? That sounds good to me.
I'm all for it because I don't know of any car company that just makes transmissions and breaks without the idea of thing looks make it into a car,
I think we're gonna get there someday. I really d'oh one of my colleagues in congenital heart surgery, Dr. Ming See is actively looking at ways. See if he can not just take stem cells, convert them into cardio mile sites or heart cells, but then actually provide a blood supply to them. So so far, then, some therapy has shown some promise. But by and large to be frank, it's been disappointing in the heart for a number of reasons. Getting these cells to actually convert into heart cells and beat as heart cells. We've been able to do no problem. Well, that's putting it move, maybe mildly. But it's certainly able to do but getting them to beat Congress. Lee, with the rest of the heart without rhythm problems, getting them to maintain health and survival so that therefore they need nourishment. This is what your blood supply does to provide oxygen and nourishment. That's been the issue. But I think just progressing really well. I'm extremely proud of the fact that we have a group of basically four or five researchers, some of whom also do surgery, really actively engaged in looking at this, and I think it's going to be very useful in the future.
I'm excited about the prospect of us possibly being able to grow a heart. But since my own son was born and diagnosed with H L a chest, I'm curious if there's some way we could use the stem cells to kind of beef up his own heart so he wouldn't need a transplant. Do you think that something like that is possible in the future?
Yeah, I do, and that's a very good question. It's exactly what we're trying to look at. For example, older people who have a heart attack and lose part of the muscle of their left ventricles 10 we in some way inject or implant themselves to rebuild that weakened wall so that, indeed, their heart muscle overall will improve. Hopefully, this is the same sort of thing we might be able to do with patients who have single ventricle hearts like a plastic left heart syndrome and other conditions. Can we in fact, grow heart cells to implant into the patient's own heart cells to strengthen it, for example? That's certainly one area of research in one area that I hope will be able to do. We aren't there yet, and it's obviously hard to predict when we will be there. But I think the advances that have been made in the last few years of really phenomenal and certainly I'm hopeful and optimistic that we will get there and then not terribly distant future
that is so exciting to hear. I just am thrilled with what I am reading about the stem cell research. And of course, when you go out on the Internet now on YouTube, you can even see some of the researchers and what they're doing. It's just phenomenal. Exactly. Well, we need to take another quick break, but don't leave yet listeners, because coming up next, we're going to talk to Dr Bove A. About what the advancements that have occurred for HHS babies means for HHS. Adult heart warriors will be back after this quick commercial break.
When I saw 70 of the C H Street groups growing, I found family just ready to join May. Anyone who is a member of the adult congenital heart defect community can be a guest on our show. We have a great year plan that we look forward to sharing other interesting topics. Heart to heart With Holland David serving a CHD community Wednesdays at noon Eastern.
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the congenital heart defect community Today show is advancements and treatments for HHS heart warriors and our gas is Dr Edward. We just finished talking with Dr Bove, a about the current trends for babies being born with a chile chest. But now, in our last segment, I'd like to focus on adults with HHS. So now more hoh is adults or surviving than ever before. Can you tell us about trends you see for adult HHS survivors specifically, is transplant necessarily stage four for a chili dress? Adults?
Well, once again, we are learning as as time goes by. And certainly our group here has an enormous number now of patients who are into their teens and twenties as we follow them and learn from them what issues will develop. I think we know, of course, that the surgery that we now do fairly routinely is excellent surgery, and it provides the best pal e ation we can. But we also realize that we are not creating normal heart, and that will likely be a limitation in the type of heart that we can surgically reconstruct as these patients grow into adulthood. I don't think we know yet when that's going to be. I don't think we really even know if it's going to be for all patients, but I think we do know problems will occur. You're right in saying that at least today, transplantation is the mode of choice. If I can put it that way for patients who have a failing Fontana, the Fontane may fail for a different reason, sometimes because the heart muscle weakens sometimes because the lungs air perhaps not as healthy as we'd like. And so the replacement of the heart with a donor for chamber heart currently is what we really have to offer. Transplantation remains limited because the donor hearts are limited. And of course, we are seeing a large pool of these patients now getting older. And so it's going to put a strain on the availability of donor heart. So what a lot of research is now going into. And I am also proud to say here in Ann Arbor were developing a very vigorous research plan and looking at a device. I'm sure everybody knows that there are these various kinds of assist devices for patients who have serious heart problems or have cardio myopathy ease, and that we could even replace the heart with a total artificial heart. But we're looking at ways that we may now be able to design some of these devices some of which are not a lot bigger than your index finger to support the single ventricle. Can we recreate potentially a configuration of the heart that would allow a totally implantable assist device to be placed that can essentially provide a pump and provide a blood flow source into the lungs and or to the body, depending on what's necessary? So I see that these were the two biggest areas basically so far transplantation and the development of really patient friendly assist devices that we will begin to learn more and more about and probably used more and more as these patients reach adulthood.
So are you saying that there's a possibility that some of these assist devices could actually help the surgeons to create a four chamber heart for Children born with HHS?
Well, it's not really creating a four chambered heart, but it's mimicking the physiology of having four chambers. We already know we can put a totally implantable heart in a patient, take their heart out so you can place that in. But the goal would be to utilize the patient's own heart. But perhaps head and assist in other words, perhaps at a right ventricle by that, I mean the assist device, which could help propel blood through the lungs. So when that becomes necessary at an older age, there may be a way to do this with an assist device that provides what's lacking in it. Dealt with hypothetical heart syndrome, namely the right ventricle.
Wow, that's amazing.
Well, I should say they're pulmonary ventricle. Is there an atomic right ventricle is now they're systemic,
right? Right. Wow, that's amazing. And then they wouldn't have to worry about immuno suppressant. Drugs are rejection of the heart because it would be their own heart. Do you have to worry about rejection of the device?
No, you really don't. And you know, our belief has always been so long as your heart is working, you're better off with your own heart than you are with a donor heart. So, yes, we can provide many more years of really useful and good quality of life with these kinds of assist devices, which I think again will be reality. Then we can avoid the issues of insufficient donor hearts, immuno suppression, etcetera.
Wow, that's amazing. Well, now that we have more hohs adults living longer with their Fontane hearts than probably anyone thought they
would. What are
some of the things that you forsee affecting the HHS population earlier this season? I had a wonderful doctor. Dr Fred Wu came on and talk to us about liver complications. My son had an aortic aneurysm. It developed, and he ended up having to have a frontier revision. Can you touch us about some of those kinds of things that you see happening to an aging actually trust population.
But you've already touched on one. And that's the liver issues and more studies air coming out, looking at patients at certainly in their adult years, trying to discover or least examine liver function. And we are finding out that liver issues are probably going to be more common than we thought, likely due to the Venus hypertension, if you will, that affect the liver so that there's some Stasis and the liver, as blood doesn't progress through the liver as efficiently as it would with a normal heart. We also know that patients they're going to develop a rhythm, problems heart rhythm problems and likely need pacemakers. Already well known is this conditions so called protein losing interrupted the or P. Ellie, which was still very poorly understood as to why some patients seem to lose a lot of protein in their stool. Surprisingly to me, we have rarely seen late complications of the surgery itself, such as obstruction to blood flow into the lungs at the aneurysm, which you alluded to, or other problems which we sort of expected, since we're doing all this reconstructive surgery in a tiny heart that when they grow to be adults, well, isn't there going to be some issue? The way we've done it here in Ann Arbor, and I think a lot of other centers do it the same way. We're not seeing that. I don't mean to say it never occurs. But it's been a strikingly uncommon to see that even a large number of now fully grown patients in their teens twenties and beyond. So that's been rewarding to see that we haven't needed to go back and deal with valve problems. Very often, aneurysms, obstruction in the lungs or obstruction in the aorta occasionally happens occasionally. We can deal with it often if we need to. We can do with it in the cath lab, but so far anyway, that's been very
wow. Well, that's good to hear. I'm wondering if there's a connection between the P l E and the problems with the liver.
Well, yes, it likely has something to do with all of that. Again. If you think about the whole physiology of the Fontane, you have higher Venus pressures than you should have. You have lower pressures in your lungs than you should have. So it's really has been coined. So called Fontane Paradox. This is over. The course of time is going to cause some difficulties with the intestines with deliver and hopefully not, but perhaps with other organs that we honestly don't even know yet,
right, because we haven't had people surviving to their fifties or sixties with this kind of heart per se, we have seen other heart effects that were treated with the Fontane Have any of those studies that have been done on other patients who were treated with a Fontane helpful to what you can predict might happen with these long term hohs survivors?
I'm not sure that there's been any single group that's been studied better than the H. L. H s patients. If you think about it, one of the most remarkable things about the development of treatment, and I don't mean just surgery. But medical treatment for Hln Stacy has been the spin off to all patients with forms of single ventricle. So the things we have learned we have now been able to apply to patients who have other types of single ventricle and learning how to avoid mistakes. With palliative therapy, Adam optimize their lung and heart function for the future, I think. Well, for one, I would say that we and others have published some late studies looking at H. L A. Jets compared to other single ventricles to try to find out. Are there differences? By and large, there haven't been a single right ventricle. If it's healthy, seems to hold up just a CZ well as a single left ventricle, so long as it's healthy. But I don't know that we have enough data to really substantiate that, particularly into the forties and fifties. We do have patients with single ventricle who are in their fifties, but if you think back 50 years ago, treatment for any form of single ventricle was so primitive that really it was rare for any of these patients to survive and survive, avoiding the risk that we now know we need to avoid.
Right, right? Well, given your history with H L. H is over the last several decades, what do you think the next big advancement will be for the HHS community?
Well, I think, you know, as new problems or diagnosed, innovations and ingenuity will undoubtedly need lead to new solutions. Just happened 25 years ago when we began this journey with hyper plastic left heart syndrome. I'm always amazed at how, when things happen where Abel as a specialty as a team, to be able to look and figure out a way to go back and solve it, I think right now is we already talked about it. I certainly think that the major advances air going to be in stem cell therapy and in devices that are, if I can put it mildly, patient friendly so that they are things that will allow these adults now to still go on and lead active healthy lives. That's where really much of the focus is. But I think that there will continue to be small advancements, things that may seemingly not seem all that important that we'll be able to go back and say, Oh, we can avoid this problem when they're 30 if we do something like this when they're one. So these kinds of advancements, we'll just continue to occur because that's been the history of this condition for decades.
What about gene therapy? Do you think that that is something that you'll live to see happen that might even prevent a baby from being born with a chili? Just,
Yeah, I don't know if I say that. I think we're getting into such issues that go beyond just the medical use of it and into, of course, what is the right thing to do ethically and morally. But I certainly see that field developing as well that we will be able to perhaps modify a gene, discover the gene that may lead to the types of heart conditions and be able to modify that even before conception or soon thereafter, to be able to avoid these conditions.
That would be amazing, wouldn't it?
Well, thank you so much for coming on the program today, Dr Bove A.
Well, it's my pleasure. It's really great to talk to you and to talk to your audience.
Well, that does conclude this episode of heart to heart with Anna. Thanks for listening today. Please come back next week on Tuesday at noon, Eastern time for the final episode of the ninth Season of Heart to Heart with Anna. Until then, please find a follow our radio show on speaker. And remember, my friends, you are not alone.
Thank you again for joining us this week Way. Hope you have been inspired on, Empowered to become an advocate for the congenital heart defect community. Heart to heart with Anna With your hose down, Dworsky can be heard every Tuesday at 12 noon Eastern time.
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