Surgical Innovations in Pediatric Cardiology

Throughout the evolution of neonatal and pediatric heart surgery, Lurie Children’s has built a reputation for building trust with families and providing the best possible care for children with complex heart conditions. In this episode, David Winlaw, MBBS, MD, FRACS, the surgical co-director of Lurie Children’s Heart Center, discusses early diagnosis of heart conditions, advancements in neonatal heart surgery, and the integrated cardiac team at Lurie Children’s Hospital. 

“Whether your baby is three weeks old or 15 years of age, we're a good destination to look after you and the family. We've got the right specialists. From baby heart surgery to ventricular assist devices and transplants, there's not a condition that we don't treat.”  

David Winlaw, MBBS, MD, FRACS 
Division Head, Cardiovascular-Thoracic Surgery  
Co-Executive Director, Heart Center  
Member, Lurie Children's Surgical Foundation 
Willis J. Potts, MD Founders’ Board Professorship in Surgery 
Professor of Surgery in the Division of Pediatric Cardiovascular-Thoracic Surgery and Willis J. Potts Chair, Northwestern University Feinberg School of Medicine

Topics Covered in This Show

• Only about a third of babies born with a heart disease, also known as congenital heart disease, will need immediate treatment. Dr. Winlaw says some conditions will need treatment when the baby is a few years older and some may not be found until later in the child’s life.
• Since Dr. Winlaw started practicing medicine in the 1980s, neonatal heart surgery has evolved into a much safer practice. This is in part due to improvements in the heart-lung machine, which keeps the baby’s heart and lungs stabilized during the operation. It’s also due to improvements in the integration of care across specialties.
• Early diagnosis of congenital heart conditions can save lives. However, Dr. Winlaw calls it a “double-edged sword.” Early diagnosis through genetic testing, for example, helps doctors prepare for the delivery and care of the baby, but it can also cause families anxiety and depression leading up to the delivery.
• Dr. Winlaw was the first to introduce a hypoplastic left heart syndrome surgery service to his home city of Sydney, Australia. When a baby has this common syndrome, structures in the left heart are too small. The recommended pathway for treatment is three surgeries in the baby’s first three years, starting a few weeks after birth. 
• Prenatal doctor visits are important, Dr. Winlaw says, because certain conditions, like hypoplastic left heart syndrome, are almost always diagnosed before the baby is born. These visits can help prepare you for what to expect if your baby has a heart condition. 
• Dr. Winlaw calls neonatal heart surgery a “whole hospital endeavor” that begins with a sonographer. Something the sonographer captures in an ultrasound may lead to a referral to a pediatric cardiologist. From there, more specialists will be referred.
• For parents who may be looking for a hospital, Dr. Winlaw celebrates the Heart Center as an enormous resource with unusual capabilities, such that there is no condition too rare or unusual or too hard to manage.

Transcript

[00:00:00] Erin Spain, MS: This is Precision, perspectives on children's surgery from Ann and Robert H. Lurie Children's Hospital of Chicago. I'm your host, Erin Spain. On this podcast, we introduce you to surgeons at one of the country's most renowned children's hospitals to find out how they're transforming pediatric medicine. Today's guest, Dr. David Winlaw, is a surgical innovator and scientist who's dedicated to improving the lives of people with congenital heart disease with a focus on neonatal heart surgery. Dr. Winlaw recently joined Lurie Children's as division head of Cardiovascular Thoracic Surgery and surgical co-executive director of the Heart Center at Lurie Children's. He joins me today to talk about his specialty, the latest innovations in treating congenital heart disease, and how Lurie Children's is able to harness the power of research to provide care for the most complex cases. Welcome to the show.  

[00:01:06] David Winlaw, MBBS, MD, FRACS: Thank you very much, Erin. Great to be here. 

[00:01:08] Erin Spain, MS: You are recognized in your field for new surgical approaches to complex neonatal heart conditions. How did you get into this field in this specific area of expertise? 

[00:01:19] David Winlaw, MBBS, MD, FRACS: Great question. Well, it's really all about the babies. And every baby is different in terms of the ones with heart disease. And it's important to individualize a management plan, according to the problems that they have. And sometimes they're heart problems, sometimes they're other problems and sometimes we're just dealing with prematurity or low birth weight, but all of these things need to be taken into account. And what we are hoping to do is build a pathway that involves as few interventions as possible, but to make those interventions as definitive in their outcome as possible, with the idea that we'll be able to achieve a pathway which carries the least morbidity and, ideally, the least time in hospital, although that is sometimes harder to achieve. 

[00:02:03] Erin Spain, MS: Take me back down your path to medicine. How did you end up in this space? 

[00:02:08] David Winlaw, MBBS, MD, FRACS: So like lots of enthusiastic young surgical residents, everything I did I thought I wanted to do. So I was going to be an orthopedic surgeon and a plastic surgeon, but when I saw cardiac surgery, I was like, wow, that's really immediate and compelling. And so I did that. And then, of course, I ended up in pediatric cardiac surgery for a rotation and the variety of operations and the obvious difference that it's such a team sport, looking after babies. The cardiologists and the cardiac surgeons work extremely closely together, and the people who are looking after the babies at 2 a.m. in the morning are as important as the people who are doing the operation in terms of getting a great outcome. So, all of those aspects of working in a team and ultimately leading a team are very attractive to me. There's nothing like the morning after a big operation and going seeing a baby who's just quietly sitting there, breathing away, looking good, all the monitors showing great values where we would expect them to be, and hopefully the mom asleep in a chair next to the bed. That's the sense of calm and achievement that really feels good. 

[00:03:13] Erin Spain, MS: I know you said there are many different reasons why a baby may need heart surgery, but congenital heart disease is the most common type of birth defect. What are the typical causes of congenital heart defects and how can they range in severity and then end up in the operating room, these children? 

[00:03:30] David Winlaw, MBBS, MD, FRACS: Of all the babies that are born with congenital heart disease, happily only about a third will need to have an intervention as a baby. There's a number of conditions which you're not going to be able to leave the hospital without having addressed. But there are others, some of which get better by themselves: You can come for an elective operation, you know, around the age of 2 or 3 and there are some that are not discovered until later in life. But for the babies that need surgery, surgery is a highly technical process, which really starts with a diagnosis and, you know, certainly includes the operative phase but continues well into the recovery period, getting back to the normal baby stuff like, feeding and growing. 

[00:04:13] Erin Spain, MS: So while we don't always know why these conditions occur, and as of right now, there isn't necessarily a cure for these conditions, surgery can make a really big difference, and it's one of the top interventions that can repair the heart and save lives. Tell me about this type of surgery and how it's evolved since you began your career. 

[00:04:32] David Winlaw, MBBS, MD, FRACS: When I started in cardiac surgery, it was the '80s. So at that stage, neonatal surgery was really starting to get its legs, so to speak, in that good outcomes were being achieved on a more reliable basis. Prior to that time, our general approach had been to defer any operation that it was possible to defer because the outcomes in babies were not great. Part of that had to do with refinements in the heart lung machine that supports the baby's circulation whilst we're operating on the heart that were yet to happen. And partly, also, it's an experience thing, and there was a lot of learning that needed to occur and people needed to evolve the way they practice surgery. You know, do I try and do this really, really quickly and sacrifice some accuracy? Or do I take my time and get a perfect result, but that's going to take a long time on the heart lung machine? And so, people were sort of transitioning from the former to the latter. And as the heart lung machine got better and our techniques for preserving the heart whilst it was stopped improved, so too did the results. So we're traveled a pretty impressive pathway over the last 30 years. When some of these operations were first designed, the mortality rate was, you know, around 30 or 40 percent. Which was, you know, must have been a terrible time for a parent to be watching this and to be part of it. And now a similar operation would be done with the expectations of mortality of less than 1 percent. You know, there have been some great innovators in our space, many of whom are still alive. We're a pretty young profession in that regard. 

[00:06:04] Erin Spain, MS: So much change really in a short amount of time and another area of change is early diagnosis. That's something that now you're able to do a lot of times during prenatal care. Can you talk to me a little bit about early diagnosis and how important it is that you're able to start interacting with families early on when they find out before the baby is even born? 

[00:06:25] David Winlaw, MBBS, MD, FRACS: It's a double-edged sword. From a medical perspective, it's really very important because it allows us to plan the delivery or care of the baby after the delivery and to make sure that there are no unnecessary moments where the baby is not receiving adequate blood flow to the brain and body, and that's a really important thing. An example would be, for some of the babies who are born with just one ventricle, it's important to keep this little connection between the heart and the lower body open, and that requires medication. It's a very simple thing to administer, but if that's not available straight away, then the baby's going to get super sick in a hurry. Similarly in transposition of the great arteries, sometimes there's not adequate mixing of the circulations to the lung and the body. An intervention needs to happen that sometimes needs to be delivered within 15 minutes. So it's a really major logistic exercise, making sure that such babies are expected and can be dealt with in a timely fashion. And it's those kinds of things that have really improved outcomes over the last 15 years. You know, the technology's been available, but the teamwork and integration of care between the fetal doctors and the rest of the team is really tight now. And that's something we do really well at Lurie. 

[00:07:37] Erin Spain, MS: You said it's a double-edged sword. Can you talk more to that? 

[00:07:40] David Winlaw, MBBS, MD, FRACS: I guess you put yourself in the position of either finding out at the time of delivery that there's a problem with your baby's heart versus knowing 20 weeks prior. And I think, for those parents who find out 20 weeks prior, there's a lot of anxiety and concern that's generated. Some of that can be certainly helped with providing good information. But at the end of the day, people, quite naturally and understandably, worry. And we know that anxiety and depression are quite common in affecting both mothers and fathers when there is an anticipated delivery of a baby with congenital heart disease. And so, providing all the supports that we can is an important part of looking after these families, from the point of diagnosis onwards. 

[00:08:25] Erin Spain, MS: Tell me more about that. How do you build trust with families and help them through all of this medical decision making? 

[00:08:31] David Winlaw, MBBS, MD, FRACS: We need to first have a clear idea amongst the medical and nursing people as to what exactly the pathway is likely to look like. And once we have that clear, then we need to explain to the parents what we do know and can expect and the things that we don't yet know and have to be evaluated and determined after delivery. But we can usually paint a pretty accurate picture on the basis of the fetal echo. That's the ultrasound done. And, uh, we would mostly have a good idea in more than 80 percent of cases exactly what things are going to look like in the first couple of weeks of life. And I think by providing enough information, but not too much, early on, or at least providing information over several interactions with the families helps a lot. And you talk about building trust and relationships. I think it's really helpful for the parents to meet people who are going to be involved in their baby's care ahead of the baby's arrival. And that's the kind of interaction that we seek to provide on a regular basis. 

[00:09:33] Erin Spain, MS: I know that you've been investigating genetic testing to improve diagnosis and treatment strategies and prognosis for your patients. What do you see as the challenges and future possibilities when it comes to genetic testing for congenital heart disease? 

[00:09:48] David Winlaw, MBBS, MD, FRACS: It's a really, really dynamic area. And sometimes, with expecting parents, it's not a great time to discuss. They're also sometimes, you know, issues of personal responsibility if there is a genetic anomaly that's identified. There's a set of genes that if we knew whether they were normal or not, ahead of delivery, would really help us, because not knowing can lead to investigations not being done and issues not being identified ahead of time. So I think that's an important part of it. But really the fullest realization of the benefits of a genetic diagnosis is not just in understanding what's caused the heart disease, but it's about how we can modify the treatment going forward to avoid complications, to choose the best pathway. You know, do we do surgery early or do we do surgery a bit later? How do we construct this care? What are the most appropriate medications for your baby? And that this is a personalized assessment of care rather than just something that we do for everybody. We need to be able to make a statistical correlation between the presence of a particular genetic variant in a baby and a later outcome, and that's where a lot of our research is focused. So really there's two parts. Part of it's about the cause of the congenital heart disease and the other is how we make the best of what we have going forward.  

[00:11:06] Erin Spain, MS: You mentioned before that sometimes it makes sense to do surgery right away, maybe even hours after a baby is born, but other times you wait. Tell me about the difference between doing surgery on a very small newborn versus a toddler, and then also why those decisions are made to do it now or wait until they're older. 

[00:11:24] David Winlaw, MBBS, MD, FRACS: There are a lot of changes happening in the circulation soon after birth. And for the heart doctors, the thing that we're really watching is the relaxation of the arteries in the lung over the first few days of life. So initially the pressure in the lung circulation is quite high and then over the next week it drops dramatically, and so there will be a lot more blood flow going to the lungs in a week after delivery than there will be at the beginning. And this is relevant if you don't have a separated circulation between the blood going to the body versus the blood going to the lungs. This is the situation for many of our kids who either have a hole between the two sides within their heart or they have just one ventricle which is sending blood to both, the body circulation as well as the lung circulation. So it's this time-related decrease in pulmonary vascular resistance, which is something that our management needs to take account of. So if I give an example of hypoplastic left heart syndrome, which is a common condition. The babies will follow a pathway of three steps to a Fontan circulation, which they will live with, which is a good solution. It's not a normal circulation, it's not two ventricles at that point, but that's the current pathway. So if you operate on a baby in the first week of life, it's a very big operation, it carries important risks and the baby will take some weeks to recover, maybe in the hospital for six weeks afterwards. Feeding and growing are difficult things at the end of all of that. But if you're able to defer that operation, as we sometimes do now using these new approaches, where flow restrictors, little disks of material that reduce the flow of blood to the lungs, what we can see is that we're able to get the babies through to a later operation, so maybe two to three weeks of age, rather than one week of age. And the maturity that we see in these babies makes them really a different prospect altogether. They get over the operation much faster. Their heart function is better afterwards. And, although it's more interventions, it's actually a better outcome in many circumstances, and we don't do this for everybody who has hypoplastic left heart syndrome, but it's a really handy way to defer an operation which still needs to be done but can be done with better outcomes. 

[00:13:41] Erin Spain, MS: I think it's important to talk a little bit more about that syndrome that you just mentioned, hypoplastic left heart syndrome and some of the procedures that you're able to provide to even the smallest of patients. 

[00:13:53] David Winlaw, MBBS, MD, FRACS: As you may know, I, trained in Australia and I was an attending surgeon there and led the unit there for some time before coming to America and  I introduced surgery for hypoplastic left heart syndrome in New South Wales, in my home city of Sydney. So in hypoplastic left heart syndrome, there are a couple of important problems, but the biggest one is that all the structures associated with the left heart are too small. And so as part of the so-called Norwood operation, named after the person who described this and was able to demonstrate that it was an effective pathway for a condition that was previously thought to be unmanageable in a medical approach, involves reconstruction of the outflows from the heart and placement of a shunt or some kind of connection to bring blood to the lungs. Because part of the reconstruction of the blood flow to the body involves using the pulmonary artery, which would normally be sending blood flow to the lungs. So there the two important parts of the operation. And it's a really big operation in a small baby. Because there's only one ventricle to work with. These babies don't have the usual, if you like, auto adjustment systems that our normal circulation with two ventricles has. You know, the right side delivers exactly what it's supposed to, to the left side, whereas after the Norwood operation, what we're doing is balancing those two circulations all the time. And part of that is related to the size of the tube that we place between the heart and the lungs. And part of it's also to do with this time-related change in pulmonary vascular resistance that we were talking about, and managing that in the postoperative period.The antenatal counseling is such an important part of any hypoplastic left heart syndrome program because it's an easy diagnosis to make antenatally. And so, the majority of babies come with an antenatal diagnosis. So when we look after families that have an antenatal diagnosis of any condition, but particularly hypoplastic left heart, it's a really important thing that the families understand the pathway, and that that pathway includes a fair bit of time in hospital around the time of the Norwood surgery, which is conventionally done in the first few weeks of life. And that they're working towards their second operation around three months of age. And then usually a good period of trouble-free motoring around to about three years of age when we do the Fontan surgery. And that is the end of the scheduled surgery plans for that particular condition, although we know that kids with a Fontan will need ongoing medical care and, in some cases, further procedures. 

[00:16:25] Erin Spain, MS: There are so many people involved with the care of these babies, from the time the diagnosis is made all the way through their final surgery. Can you describe that team, the roles that they all play to make this go so smoothly? 

[00:16:38] David Winlaw, MBBS, MD, FRACS: It's huge. It's a whole hospital endeavor. And it starts with a sonographer who identifies the anomaly on the standard fetal scan. And very often, that will trigger a referral to a pediatric cardiologist. And there starts the process of providing contemporary information about outcomes that would be expected for a baby with this condition. And the quality of information provided is so important at that time, you know, the setting of realistic expectations. And also the process of building confidence in the family that the group of people who are doing the counseling are also well connected to the people who will be providing for the care after the baby's born. And that they know what they're doing and getting good outcomes. The internet is a wonderful thing, and we know that all the families are researching the outcomes and the profiles of people providing care, and so we see that as a good thing. Building a community of care locally as well as internationally has been so beneficial for this group of patients. 

[00:17:34] Erin Spain, MS: I think it's important to note that the Heart Center at Lurie Children's is nationally ranked for pediatric cardiology and heart surgery by U.S. News & World Report. And the center brings a spectrum of cardiac specialists together, like you mentioned, to care for patients and their families, even with the most complex heart conditions. Tell me about the comprehensive nature of what you're able to provide at the heart center and how this might stand apart from other centers around the country and the world. 

[00:18:01] David Winlaw, MBBS, MD, FRACS: We pride ourselves on integration of care. That's the thing that we're always trying to work on, to make sure that all the parts of our organization are working together in a coordinated way and in a timely way to provide the best information and then the best procedures and outcomes. And what that means is a lot of pre-planning. It's a lot of discussion about the baby's particular condition and how best to manage it, and then orchestration of the intervention itself so it's done at the right time by the right people. I think also looking back at the outcomes of what we've done and seeing how we can improve is also a really important part of a tight, integrated team, and we do that really well. 

[00:18:44] Erin Spain, MS: And tell me about the role that scientists play in this comprehensive team and research. How do you facilitate research and continue discovery to improve care for all of your patients? 

[00:18:55] David Winlaw, MBBS, MD, FRACS: This is something that I have a big investment in and was able to secure the investment of all the parents involved a long time ago when I worked in Sydney. We put together a bank of DNA specimens which has been used for research into congenital heart disease by ourselves and others around the world and actually now has almost 5,000 specimens, which is a huge resource. And it continues to be managed by my colleagues in a way that will shed light on individual conditions, but also as a way of really pushing things forward to not just understand diagnosis, but also understand how better to manage the treatment phase of care. This is the process of identifying particular variants. I mean, we all have many variants in our genes, some of which don't seem to make much difference and others might have a really big influence on, for example, how the heart might work after a heart operation. And so identifying those variants and being able to modify, knowing how to modify treatment going forward is really the focus of the interface between genomics and clinical care. 

[00:20:05] Erin Spain, MS: Are most parents open to the idea of being part of research, of sharing their children's DNA into this registry? 

[00:20:13] David Winlaw, MBBS, MD, FRACS: The point that I'm meeting the parents, they're always wondering what it is that they've done which has caused this problem. And so one of the first things that I need to do is reassure them that it's almost never something that they've done. And to take that burden of guilt off their shoulders is a good part of my job. But they don't want any other family to go through the experience that they're going through. And so when asked to contribute to a DNA registry, they're usually very happy to proceed with that. 

[00:20:41] Erin Spain, MS: So there's a lot on the horizon here for the future. Tell me what you would like to see happen in the next five to 10 years here at Lurie Children's to really push the field forward. 

[00:20:51] David Winlaw, MBBS, MD, FRACS: I think for highly technical crafts like our own, the scientists who are in the lab are also engaged in a highly technical craft and having the scientists and the physicians talk more often and to focus on an area that they can really bring all their skills to is a pathway which is bound to produce meaningful results, you know not just papers and journals but actually different treatment paradigms and hopefully new drugs to prevent congenital heart disease. And if not prevent it, then certainly be useful in the care of kids with congenital heart disease. This kind of translational work and working with scientists who are such experts in their domain and really, you know, helping them understand the clinical situation, providing the necessary samples such as the DNA from the DNA bank, is not just a joy but it's our responsibility as physicians. And one of the things that Lurie and associated institutions is doing is really building up that translational piece, the interfaces between the clinicians and the scientists and its work, which is costly but will pay enormous dividends down the track. 

[00:22:02] Erin Spain, MS: As we're wrapping up today, what message would you like to leave with parents who are listening, who are considering coming to the Heart Center at Lurie Children's for maybe a second opinion? What would you like to share with them? 

[00:22:14] David Winlaw, MBBS, MD, FRACS: We're going to look after you, we're going to understand your concerns and we're going to do our best to give you really clear information. And where there's doubt, we'll explain why there's doubt. We will hope to inspire in you the confidence that we're a good place to look after you and your baby. Whether your baby is three weeks old or 15 years of age, we're a good destination to look after you and the family. And what we make sure of is that we've got the right specialists and one of the great things about Lurie is we have the full range, you know, there's not a condition that we don't treat, from baby heart surgery to ventricular assist devices and transplants, we're able to do all of those things. I think we're the third busiest childhood transplant unit in North America, which means the third busiest in the world. And so, there is an enormous resource and capability here, such that there is no condition which is too rare or unusual or too hard for us to manage. 
We're all part of a machine, and we want to make sure that we're gearing up the machine to look after people as well as we possibly can. And I just feel a great joy in being able to harness the right people to provide the right care at the right time. It's an enormously and personally satisfying thing. 

[00:23:30] Erin Spain, MS: Well, thank you so much, Dr. David Winlaw, for joining me today and welcome to Lurie Children's. We're so glad that you're here. 

[00:23:38] David Winlaw, MBBS, MD, FRACS: Thanks Erin, it's been great chatting. 

[00:23:40] Erin Spain, MS: For more information, including how to make a referral or an appointment, visit LurieChildrens.org.