Cori Cross, MD (Host): This is Pediatric Insights, Advances and Innovations with Children's Health, where we explore the latest in pediatric care and research. I'm your host, Dr. Cori Cross, and today we'll discuss the Complex Heart Valve Program at Children's Health. The Complex Heart Valve Program at Children's Health provides specialty care to children who have structural issues impacting their heart valves. And to delve into that today with us is Dr. Karl Reyes, Pediatric Cardiothoracic Surgeon at Children's Health and Associate Professor at UT Southwestern. Dr. Reyes, thank you for joining us today.

Karl Reyes, MD (Guest): Thank you, Dr. Cross, for inviting me to talk today.

Host: So tell us about the Complex Heart Valve Program. What services does the program provide and who are the patients it serves?

Karl Reyes, MD (Guest): Yeah, so the complex congenital heart valve program is a program that addresses one of the more complex or challenging subset of patients who have congenital heart disease. These are the ones who are born with abnormal heart valves. Oftentimes when the valves are too small, there aren't really good options for replacing these valves, so, because of that, there is a very big effort to try to repair these valves or try to make them work by using repair techniques or adding tissue and everything like that which basically is in this effort to avoid replacement of the valve.

Other things that are important when you're talking about patients with congenital heart valve conditions, is that if you place a regular valve or if you replace the valve with a mechanical valve, then these patients will be on blood thinners lifelong. However, if you're able to repair these valves, then you can avoid blood thinners hopefully forever but if not for a very long time.

So to answer your second question, who are the patients who can benefit from this? It's really any patient who has a heart valve condition. These could be patients of several ages. They could be infants born with bad leaky valves and they need to be fixed right away or they could be patients in their teenage life whose valves just continue to deteriorate over time and then suddenly it becomes untenable or just, you know, the valve is just leaking too much and it's causing the heart's function to deteriorate. And that's when it's time to intervene for those patients.

Host: When you're able to repair a valve, are you able to then do that as a lifelong process or is that something you have to go in and sort of touch up along the way?

Karl Reyes, MD (Guest): Some patients, it can be a one and done, or, you know, it's just a one time procedure, but I would say that these patients are oftentimes more challenging than that, and they would often need a second operation or a third operation. And oftentimes we're able to offer, in our program, a second or a third repair, which is still more beneficial, I think, for these patients than to do a mechanical valve replacement at such a young age, just for the attendant reasons that I mentioned, with regards to having to be on blood thinners.

Host: And mechanical valves don't last a lifetime either. Isn't that right?

Karl Reyes, MD (Guest): Correct. So mechanical valves, number one, well, they can last a lifetime in adult patients. However, in young patients, because the valves don't grow, it will be, you know, there's such a thing as what we call patient prosthesis mismatch, meaning that the patient is bigger than the size of the valve opening. And so in that situation, the valve still needs to be replaced.

Host: Right. Because what we know as pediatricians is children grow, something that adult patients don't have to worry about. it sounds like there are a lot of experts involved. Can you share a bit about your team?

Karl Reyes, MD (Guest): you know, Children's Medical Center is a big hospital with a lot of physicians. The Heart Center is a big team, as you can imagine. And we have pretty much a specialist or a subspecialist for all the subspecialties of congenital heart disease.

So there are the surgeons, there are the cardiologists who sub specialize as well in the imaging realm. So these are the ones who do the echocardiograms and do the MRIs and the CTs, and these are cardiologists who are very good at advanced imaging, meaning they can, we are now able to look at the valves in 3D.

We are able to look at the valves by creating models of the heart. So there are many ways, nowadays to be able to look at valves using the expertise of the specialists we have. There are also the interventional cardiologists who help us with dilating valves so that we don't have to go to the operating room all the time to replace or repair valves again because they can actually do things in the cath lab that can help with temporizing or delaying any, delaying a future valve operation. And then, of course, there are our cardiac intensivists, our anesthesiologists, and the rest of the team, you know, the ones who work with the heart lung machine, our nursing team, our advanced practice. There's, it's a big team.

Host: I can imagine, and I'm sure there's a lot of follow up with these patients as well.

Karl Reyes, MD (Guest): Yes, correct. Yes, absolutely.

Host: And then are you following these patients for years afterwards, or do they eventually transfer to an adult program?

Karl Reyes, MD (Guest): So, I do like to think of congenital heart disease as something that requires lifelong care, even in the circumstances where everything is appears to be or is actually completely normal. Things can happen along the way and it's very good for you to be in the care of physician, specifically a cardiologist.

But yes, I think, once patients reach adult life, they have to transition to the adult care to adult congenital heart disease programs and so on and so forth. But, most certainly, these are patients who need lifelong care.

Host: So, it sounds like there are multiple treatment options and that you'd always prefer to repair a valve if possible. Tell us a little bit about how you determine which treatment is best for each patient.

Karl Reyes, MD (Guest): I think that if a valve repair is possible, then we would always almost certainly attempt that first. You know, If we can make that decision before going into the operating room or in the operating room itself, but we would definitely make that effort to repair a leaky or stenotic or small heart valve.

When we cannot repair the valve, you know, and sometimes, you've repaired the valve four times, five times. You know, and so on and so forth. And you really, you know that there are very limited options. Then you have to replace the valve. And the goal then would be to replace the valve with the biggest size valve possible so that if possible, your valve can reach adult life.

But it's not always possible. So, you know, there are situations where we don't repair the valve or don't replace the valve and we tolerate a little bit of leaking of the valve for several months just to get the patient older and that's really in the effort to avoid anticoagulation or having too small a valve inside a patient.

Host: And so when you have to place a mechanical valve, is there anything that's customizable about it? Like I know we have a lot of really cool things in other medical fields where we can really customize things. Is this something that's done with heart valves?

Karl Reyes, MD (Guest): In the current iteration of valves that we have when they are mechanical, they don't really have any form of customization. They are what they are. It's the size that they have. There are new metals that are being developed that are smoother, I guess, you know, they're made of carbon and, where blood does not really attach to it, so it doesn't clot as much but these are all, you know, in the process of development, I would say, and the ones that are in the market right now, while very good, obviously technology just keeps getting better, but to answer your question we're not able to customize the valve before putting it in. And so on and so forth.

Host: So, these carbon valves, if they are something that ends up coming to market, they may not require blood thinners then if the blood doesn't stick to them. Is that right?

Karl Reyes, MD (Guest): That is the goal. I mean, the valve will still not grow. But that is the goal. You know, the goal is to avoid blood thinners altogether. And that, this has been a process that's been, or this has been in scientific development for decades now. And it's gotten better and better, but we have not reached that panacea yet.

Host: Okay. And then while we're on that topic, is there any new research or treatment on the horizon that you'd like to share with us?

Karl Reyes, MD (Guest): There is always new technology out there. One thing that I, just this morning, I was on a call to look at uh, valve repair simulation, meaning that, if you're able to repair or simulate repairing the valve in different ways before you actually go to the operating room and repairing the valve; you might have a better result because there are many ways to repair a valve. And trying to figure out what is the best strategy for the valve that you have prior to being in the operating room might have some benefit. The other thing that we've been working on in our lab is what we call resorbable heart valves.

Meaning that, placing a scaffold as a valve leaflet tissue that will eventually disintegrate and be replaced by natural or native heart valve tissue. So that might be an option for patients, you know, who are young because this could be, a valve that will grow, so these are all in development right now. And so, I think, with enough interest and funding, we hopefully will be able to get there.

Host: That would be amazing. So as we wrap up, tell us, how do patients enroll in this program? Is it referral only?

Karl Reyes, MD (Guest): We have many ways for our patients to come over to see us. They can contact us directly, you know, and schedule a visit. A lot of our patients come from cardiologists who have had challenges with valves, you know, from other centers and getting an opinion to see if we can repair the valve.

So, yeah, we have a second opinion consult line as well. If you just want to get our insights on the valve that your patient or you have, we definitely have a referral system for this.

Host: So what is one takeaway you would like other physicians to know about the Complex Congenital Heart Valve Program?

Karl Reyes, MD (Guest): I think the one takeaway is that it is a difficult or a challenging disease process. There are many ways to be successful and some of it is with surgery sooner rather than later and some of it's waiting and some of it's waiting because you want to get, you know, the patient to a larger size and an older age that you can place a larger valve. The takeaway really is that there probably is no one size fits all, but having a team that focuses on this and really has a deliberate and intentional process to evaluate, analyze these valves and a team that repairs these valves, you know, in a dedicated way; most likely will lead to success rather than other ways, you know, and I think that that's probably the biggest takeaway here is that, this focus hopefully will lead to more success.

Host: That makes sense. I mean, the gold standard should really be to have a team that this is what they do and this is their specialty. Dr. Reyes, thank you so much for being here with us today and educating our listeners on such an important topic. It was such a pleasure to speak with you.

Karl Reyes, MD (Guest): Thank you as well. And I appreciate your time today.

Host: Thank you. To learn more, about the cardiology department at Children's Health and the exciting things going on there, please visit childrens.com/cardiology. Thank you again to Dr. Reyes and to our audience for listening to Pediatric Insights, Advances and Innovations with Children's Health, where we explore the latest in pediatric care and research.

You can find more information at childrens.com. And if you found this podcast helpful, please rate, review, and share this episode. And please follow Children's Health on your social channels.