Launching a Fetal Cardiac Intervention Program at Lurie Children’s

Two Lurie Children's experts are on a mission to help define the next era of fetal cardiac intervention. In this episode, Dr. Sheetal Patel, a pediatric cardiologist and medical director of the fetal cardiology program at Lurie Children's, and Dr. Alan Nugent, a pediatric interventional cardiologist and section head of interventional cardiology catheterization at Lurie Children's, discuss their vision to use innovation and interventions to treat complex congenital heart diseases before birth.

“Out of the number of mothers that we refer for potential fetal cardiac intervention — where parents can travel and then have a successful procedure — [this] only happens in a small percentage because of all the socio-economic, financial constraints that parents face. It is heartbreaking as a provider to see our parents go through [this], that the option is available if you can afford it. And that does not feel right.” 

Sheetal Patel, MD, MSCI, FAAP
Medical Director, Fetal Cardiology Program
Fetal Cardiac Co-Director, The Chicago Institute for Fetal Health
Attending Physician, Cardiology
Assistant Professor of Pediatrics, Northwestern University Feinberg School of Medicine

“If you can safely enter a fetal right atrium, you can give infusions, you can get access to the head, you can get access to the spine… It opens up a lot of possibilities for the sickest of the sickest … future children who are going to have really, really severe disease.”

Alan Nugent, MD
Section Head, Interventional Cardiac Catheterization
Professor of Pediatrics, Northwestern University Feinberg School of Medicine

 

Show Notes

• Improving imaging techniques such as echocardiography are allowing more and more congenital heart defects to be diagnosed before birth. If heart defects are known prenatally, it provides opportunities for further investigation, mapping out a plan, and counseling parents. 
• Fetal cardiac intervention (FCI), which is done at only two other children’s hospitals in the country (Texas and Boston) allows for opportunities to treat congenital heart disease even earlier. 
• FCI is controversial because it typically involves an otherwise healthy mother carrying a severely compromised fetus. 
• The Chicago Institute for Fetal Health, led by Dr. Aimen Shaaban is equipped with the infrastructure to support FCI at Lurie Children’s.
• Given so few hospitals offer FCI, families must travel long distances to seek this level of care. Often, only families who can afford the financial burden of travel, taking time off, and additional childcare are afforded the opportunity. 
• Dr. Patel and Dr. Nugent are researching other FCI programs to identify necessary clinical and research protocols, such as patient consent and proper documentation and assessment of patients. They are eager to be both transparent and compliant with the Institutional Review Board which authorizes approval of such experimental procedures.
• Among its multiple potential applications, FCI can potentially offer an in-utero treatment for fetuses with ​significant aortic valve stenosis (or narrowing) by ballooning the aortic valve to avoid hypoplastic left heart syndrome. This now works 30-40% of the time.
• Dr. Patel shares that FCI procedures are relatively safe for mothers despite mortality risk for fetuses. She hopes that with advancements in technology, especially the miniaturization of instruments, FCI can become much safer.

Transcript

[00:00:00] Erin Spain, MS: This is Precision, Perspectives on Children's Surgery from Ann & 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, we're speaking with two experts from Lurie Children's who are on a mission to help define the next era of fetal cardiac intervention. Dr. Sheetal Patel, a pediatric cardiologist and medical director of the fetal cardiology program at Lurie Children's, and Dr. Alan Nugent, a pediatric interventional cardiologist and section head of interventional cardiology catheterization at Lurie Children's. They both join me to discuss their vision to use innovation and interventions to treat complex congenital heart diseases before birth. Welcome to the show, both of you.

[00:01:05] Alan Nugent, MD: Thank you.

[00:01:05] Sheetal Patel, MD: Thank you, Erin.

[00:01:06] Erin Spain, MS: I'd like to start off by just hearing from both of you, a little bit about your background and what brought you to Lurie Children's. Dr. Patel, do you mind starting?

[00:01:16] Sheetal Patel, MD: I have been with Lurie Children's for more than 10 years now. And what brought me to the Chicago area after working for a few years in Wisconsin, is my family, and my parents and my family settled here. So I followed and here I am. Also, I loved the academic environment and the challenges that Northwestern provides for us to pursue a career in academic medicine and all the support that we get to do better and do more advancing pediatric cardiology medicine. 

[00:01:48] Erin Spain, MS: Dr Nugent, tell us about your journey to Lurie children's.

[00:01:52] Alan Nugent, MD: I've been here just over five, nearly six years. I've been in the U.S. for 25 years. The first place I went to was Boston Children's and I was fortunate enough to work there. That's relevant for this topic because I was there when the first fetal cardiac interventions were done in Boston. I got interested in it at that time. And I spent over a decade in Texas and moved here just over 5 years ago. You know, I'm an interventional cardiologist. I moved as the section head of cardiac catheterization and, you know, my primary role that occupies me night and day is advancing and advocating for the interventional program here at Lurie.

[00:02:24] Erin Spain, MS: Dr. Patel, you're the medical director of the fetal cardiology program. And you focus on cardiac imaging to detect congenital heart diseases and utero. Tell me about your work and how imaging plays a crucial role in the early detection of CHD.

[00:02:41] Sheetal Patel, MD: As a fetal cardiac program director, I spent a lot of time doing the work in prenatal detection of CHD. So, fortunately, with improving imaging techniques and improving protocols, more and more congenital heart defects are being diagnosed before even baby is born. And the good thing about that is that if we know about the heart defect prenatally, it provides opportunity for us to counsel the parents, understand exactly what these congenital heart defect are, and then map out what we need to do to support these kids soon after birth so that we can have best of the outcome that they could have with their congenital heart defect. So, we do this detection by doing fetal echocardiography, which is ultrasound, that is done through the mother's belly, while the baby is still inside and we are able to assess the cardiac structure. We can also assess the heart rhythm. And then also how well the heart is working or heart function. We do about 2700 to 2800 fetal echo studies per year as part of the Lurie Children's Fetal Cardiac Program. I would say more than 95 percent of the babies that we handle after birth with congenital heart defect are going through our fetal cardiac program and having prenatal detection. So we spend a lot of time figuring out exactly what the congenital heart defect is, making the plans, and then most importantly, supporting the families and parents, explaining what their child has, what their child needs, and getting them mentally ready to have this journey for their child and for their family.

[00:04:23] Erin Spain, MS: So many in the field are interested in earlier interventions when it comes to treating CHD, but that's not without risks. Today, we're talking about a fetal cardiac intervention program soon to be coming to Lurie Children's. Dr. Nugent, can you explain fetal cardiac intervention to me? Why is it important to offer this to patients and also some of the risks involved?

[00:04:45] Alan Nugent, MD: It's actually, Erin, it's very, a controversial question. Give credit where credit's due. Actually, the first ones were done in England, just over 30 years ago, and the Boston program started in 2000. It's a little bit unusual in any area of medicine, but Boston has, over the last 20 years, done pretty much the same number of procedures as probably the rest of the world put together. The reason why it's so controversial is because there's obviously a healthy mother with a fetus that has something really severe. In other words, that has major implications in their postnatal life. And so to justify doing any procedure, there has to be a chance to change a trajectory. And I think it's fair to say that that hasn't actually been proven. There are two major interventions that are done in the fetal cardiac interventional space. And one is an aortic valve dilation and the other one is an atrial septum dilation. They're both performed with a condition called hypoplastic left heart syndrome, where the left sided structures don't grow. So like we're talking about the mitral valve, the left ventricle, the aortic valve and the aorta. There's different variations of that and different severity. We're not really smart enough as a field yet to know, but there's probably multiple causes of that. One of the causes is the primary abnormality is an aortic valve problem and then the left ventricle doesn't develop secondarily to that. And so the notion of those children, that if you open the aortic valve in fetal life, the left ventricle will grow. And that's probably the case in about 30 to 40% of fetuses that have benefit from the aortic valve dilation in fetal life. The other one is a much more sinister condition where you have hypoplastic heart syndrome and you don't have enough of a connection, the top chambers of the heart. So blood has to flow in fetal life from the left atrium to the right atrium, which is the opposite of normal. And if you have an intact atrial septum, that means that there's reduced flow and the theory is reduced development of the lungs. And so the theory is by opening atrial septum, you'll help lung development. Again, that hasn't really been shown with improved postnatal survival. But, the thing that has been shown is that those babies are born in better shape, they're not as blue when they're born. That procedure is now being done primarily in Boston and Texas Children's, and so why should we offer that in Chicago? Well, it's really challenging to get a fetal program up and running. I spent a lot of time in Texas doing model work and preclinical work because I was interested in the field, but starting it up from scratch is incredibly challenging. So Lurie already has the Chicago Institute for Fetal Health. It's run by Dr. Aimen F. Shaaban. They're already seeing fetuses with lesions that require fetal surgery. And as Dr. Patel's pointed out, we're already seeing a lot of children with congenital heart disease that are getting counseling and postnatal planning. So the infrastructure already exists. And so we have the huge advantage of just tacking onto that and offering these really rare technically challenging procedures in the region, you know, in the Midwest. Right now, families that see Dr. Patel, and this happens a couple of times a year, they have to travel to have these procedures. And so we want to have the ability to offer it to people of this region.

[00:07:59] Erin Spain, MS: Dr. Patel, can you tell me about some of these cases and how difficult it can be for families to try to travel to have this type of intervention?

[00:08:08] Sheetal Patel, MD:  As a fetal cardiologist, what I have seen is that many times we would diagnose these fetuses with the perfect lesion that would be a candidate for fetal cardiac intervention. We promptly send the images and information to Boston Children and Texas. However, what I see is that it is extremely difficult and challenging for mothers and fathers to drop everything and then travel out of state. What about their other children? Who's going to look after them? The train tickets, the staying over there? So, out of the number of mothers that we refer for potential fetal cardiac intervention, successful procedures that the parents are able to travel and then they have successful procedure and then coming back, only happens in small percentage because of all these socio-economic financial constraints that parents face. And this almost creates difference or a socioeconomic challenge, which is really heartbreaking as a provider to see our parents go through, that the option is available if you can afford it. And that does not feel right. We, in Chicago, have more population to serve as compared to Boston Children's. We in the Midwest do not have a program that is close by. Either they have to travel East Coast or South or West Coast to California. And therefore, I think that given how large of a population we serve in Midwest, being a referral center, I think that we would be able to offer it to more patients or parents, if we have it right here, and if families don't have to either travel, or even if they have to travel, they can drive here. So I think that it is, even if we offer exact same procedure as Texas Children's and Boston, and don't do any other innovative procedure, even at the baseline, it is important to bring that service close to home to the families in Midwest, is how I look at it, having taken care of some families that wish to go, but could not go for all other limitations.

[00:10:24] Erin Spain, MS: Dr. Nugent, can you tell me the steps that you and the team are taking right now to prepare to launch a fetal cardiac intervention program at Lurie Children's

[00:10:34] Alan Nugent, MD: We've been very fortunate here with our program in Chicago to be well supported by the Founders Board. They had a Winter Wishes fundraiser for these interventions as well as the Heckman Foundation. And I must say we've probably done more preclinical work than anyone else in history. We've done a lot, and we're really fine tuning our team, both our echocardiographers such as Dr. Patel, Dr. Shaaban, who's our fetal surgeon, and myself as the interventional cardiologist. We're doing a lot of work.

[00:11:03] Sheetal Patel, MD: Just to add on to that, the research component of studying what we are doing, but also having appropriate counseling. Are we saying all the things that we should be saying and having oversight of our institutional review board to make sure our consents are appropriate? Our clinical criteria of who should and should not have this procedure are thoughtful and learned from other centers' experience. We want to be very accurate in those things. And that's why when Dr. Nugent and I have collaborated and talked with other centers. Most other centers are doing it as a clinical protocol, but we would have IRB and make it as a research protocol just because it is our own. I feel like our own structure, for us to have accurate documentation of who are these patients, have very detailed and accurate assessment by fetal echocardiography before they go, and then have accurate fetal assessment by echocardiography after the procedure, so that we are keeping track of what we are doing and then publishing our experiences. I believe, and I think Dr. Nugent shares that vision, and so do other team members. We would want to have an experience for us to learn and document and publish so that we are as transparent as possible. And the best way to do that is making it a research protocol. That way we are obligated to keep track of what we are doing. We are also fortunate to work with Northwestern Biomedical Engineering, and great talent that are pursuing development of newer technology. And hopefully our fetal cardiac intervention program provides that infrastructure to promote that developing technology and bring that to the clinical realm at some point.

[00:13:03] Alan Nugent, MD: I'd like to just reiterate what Sheetal just said. I think on the last point, we sort of talked about our first step is to offer clinically what is currently being done, and do that well and be transparent about that. But yes, we have already in a research environment started to do what could be new and to add to the field and working with, you know, primarily with John Rogers's lab through Northwestern. And that's very, very exciting. And the other thing I just want to reiterate for the people listening to this is the Institutional Review Board, the IRB, which is basically what you have to do when you're doing any form of research. It's kind of like an ethics review. These review boards, they have lay people, they have lawyers. They have, in other words, you're just looking at it from a patient side and making sure your research is ethical. And it's very, very true that fetal cardiac interventions at other programs are just done, they're just done, and we've decided from scratch that we would do this the right way and we would have an IRB, and have a separate informed consent as a program. So I think that's a really important point. It tells our families of our mindset and how committed we are to this.

[00:14:05] Erin Spain, MS: Does that also mean that there won't be that many candidates for this surgery once you go through such a rigorous process?

[00:14:14] Alan Nugent, MD: Yeah, it's actually the minority. It's very, very rare that you have a fetus with specific forms of congenital heart disease that are in that window that are potentially amenable to an intervention. So it's actually quite rare.

[00:14:28] Erin Spain, MS: Can you explain what that ideal candidate could look like?

[00:14:31] Sheetal Patel, MD: It is an evolving science, but we have learned a lot from the first two decades of this being done at Boston and other centers. There's actually just a whole registry for fetal cardiac intervention, that tracks some of the outcomes. And the clinical criteria have gone through some updates as we learn. So I believe that what clinical criteria right now are, are better, as compared to the early experience, which did not show very successful outcomes. So the ideal patient, let's talk about the aortic valve ballooning. So a fetus, maybe let's say around 20 weeks gestation or so, has good development of the right and left ventricle, which are the pumping chambers of the lower two pumping chambers. At this point, there are two good ventricles, but the first problem that we see is that the aortic valve is not opening well. So if we leave that unattended, then what happens, and we can see it on the fetal echo one week later, two weeks later, four weeks later, is that if you do not open up the aortic valve, then the left ventricle stops growing, because it just cannot squeeze the blood out of the aortic valve, and there is reduced flow, and if there is no flow, it stops growing. So with the left ventricle stopping to grow, that is the baby that by the time the baby's 40 week gestation does not have enough left ventricle to support the systemic blood. So the goal for the fetal cardiac intervention for aortic valve is identify the fetus who has aortic valve stenosis or narrowing, but still has a good function and good structure of the left ventricle. It still has a lot of energy in that left ventricle. And at that time, if we can identify the fetus at that time and send to the team, and Dr. Nugent will then hypothetically, let's say, we did the fetal cardiac intervention, and Dr. Nugent can describe the technique a little more, but basically, it is entering the left ventricle while baby's still inside, through a needle, and then ballooning the aortic valve. So same thing as what Dr. Nugent does very successfully after baby's born, doing it while baby's still inside. And if we can successfully balloon open that aortic valve, it promotes the growth through the left side and continues the left ventricle growth. Therefore, hope is that by the time baby is born, you still have adequate left ventricle and baby does not have what we call it hypoplastic left heart syndrome. So this is trying to change the natural history of what happens to the fetuses with significant aortic valve stenosis, that if you don't touch it, it will become the hypoplastic left heart syndrome. And if you can open it, then hopefully now again, it hasn't been proven to work in 100 percent of time, but as Dr. Nugent said, 30 to 40 percent of time, you can change that and maybe would have adequate left sided structures.

[00:17:44] Erin Spain, MS: So there's been a lot of groundwork laid for this program. Tell me about the timeline. When could we expect this first surgery to happen?

[00:17:52] Alan Nugent, MD: Well, we're hoping in the calendar year 2024 we will be ready. Obviously, once we are ready, we have to wait for that first appropriate patient who, after counseling, consents to be our first patient, and hopefully, perform our first fetal cardiac intervention to add to the procedures that the Chicago Institute for Fetal Health can offer. You know, it's our role to provide this to our region and it's kind of a phrase that's used in society a lot about it takes a village. When you're talking about fetal cardiac interventions, it takes a big city, not a village. Here in Chicago, Dr. Shaaban's Institute for Fetal Health was already up and running. And, and so we get to piggyback on that as a cardiac program. And you know, that's a huge advantage for us.

[00:18:32] Erin Spain, MS: Tell me your expectations for the next five to 10 years in this field, what should we expect?

[00:18:38] Alan Nugent, MD: I don't think we can be sure. I mean, I think we can guarantee that we'll do our best and we'll be transparent. I think if we make a contribution and the field knows or can prove doing an intervention in fetal life changes the trajectory of cardiac development or lung development, then it's going to become more widespread. And I think, obviously once that happens, then you can start to expand. I think the big, big thing that I'm looking forward to is getting access to a fetal circulation and doing non cardiac things. If you can safely enter a fetal right atrium, you can give infusions, you can get access to the head, you can get access to the spine, you can get access, and it really opens up a lot of possibilities for the, you know, the sickest of the sickest, you know, the future children who are going to have really, really severe disease. But the cardiac world is not like myelomeningocele or spina bifida where it's been shown that a fetal surgery makes an impact on postnatal life. We're not at that stage yet. So hopefully 10 years from now, we're at that stage where we know what we're doing is the right thing, or we know what we're doing is not the right thing and we stopped doing it.

[00:19:45] Sheetal Patel, MD: I'm hoping that over the next five to 10 years, these procedures become more safe. Right now, I would say they're pretty safe for mothers, I think, going by the Texas and Boston experience luckily. It's a procedure, so I'm not undermining that, but it's safe for pregnant individuals. But there is still some mortality for the fetus, which I'm hoping that over time with better technique, better protocol, smaller instruments, and this miniaturizing of instruments, technical development where we have more catheters and more balloons that are suitable for such things, would make it safer for fetuses. And if we can do that, then that would be a good contribution. I think there are many other possibilities if we can establish a safe way to enter the fetal heart, not going directly you know, puncture through the heart the way it is done right now, but safer ways, then that opens up a whole lot of different options to take care of other diseases. And then, opportunity to collaborate with other specialties, as Dr. Nugent was mentioning, like maybe genetic therapy or infusion of this or infusion of that, collaborating with our electrophysiology colleague to see if we can take the pacemaker that is pretty big right now, so you cannot put it in fetus, but what if you can make it really tiny, and then put it in the fetus who is having heart block. So those kinds of thoughts, you know, how you think about 20 years ago, where we were and what we have in our hands right now, like the iPhone, like how much technological progress we did. I don't know where we would be, but it will be exciting to see where we land in 10 years from now with all these technical improvements and development.

[00:21:34] Erin Spain, MS: Thank you so much for your time today, Dr. Sheetal Patel and Dr. Alan Nugent. Thank you for your thoughtful answers about this rare and specialized surgery. We appreciate it.

[00:21:45] Sheetal Patel, MD: Thank you, Erin.

[00:21:47] Alan Nugent, MD: Thanks for having us, Erin.

[00:21:48] Erin Spain, MS: For more information, including how to make a referral or an appointment, visit LurieChildrens.org.

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