Dr Bob Underwood (Host): When babies are born with congenital heart disease, like hypoplastic left heart syndrome or HLHS, the heart is unable to deliver blood to the body. To repair HLHS and other heart defects, Children's Health performs a series of three surgeries. The first one is the Norwood procedure, which is performed a few days after birth. Norwood is one of the most risky and complex pediatric heart surgeries, making it challenging to perform.

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. Bob Underwood. Today, we have with us Dr. Nicholas Anderson, pediatric cardiothoracic surgeon, Director of the Complex Biventricular Repair Program, Surgical Director of the Cardiovascular Intensive Care Unit at Children's Health, and Associate Professor at UT Southwestern. And he's here to discuss the Norwood Procedure and he shares with us the groundbreaking technique used to reduce surgical complications and increase survival rates. Welcome, Dr. Anderson, and thanks for being on today.

Dr Nicholas Anderson: Of course. Good to be here. Thank you.

Host: So, traditionally, the Norwood procedure is performed by stopping the heart and cooling the organs to slow down the blood flow. You use a new approach that keeps the heart beating during surgery. Can you share more about how this is applied?

Dr Nicholas Anderson: Yeah. The Norwood procedure is one of the most challenging and highest risk procedures for newborn heart surgery. And it has really evolved over the last 30 years. Traditionally, for this operation, the only way to do the repair was to actually use a technique called circulatory arrest, which sounds pretty dramatic, but basically what you're doing is you're cooling the baby down to an incredibly low temperature and then stopping all blood flow to the body for a short period of time. And that technique can be used successfully, but it causes significant challenges in the recovery phase, as there has been a period of time with no blood flow to any of the body, including the brain, kidneys, and the heart.

And so over time, people have found ways to perfuse or to provide blood flow to different parts of the body for this reconstruction, but there was always a need to still stop the blood flow to the heart in order to do the repair on the heart. I actually spent the last five years working to develop a new technique called the beating heart Norwood operation where we can actually do the entire operation without even stopping blood flow to the heart. And so, it's a very complicated cardiac surgical perfusion technique, but it allows for continuous blood flow to the brain, the lower body, the kidneys, and even the heart itself throughout the entire procedure. And it's really kind of a new thing that probably only a very, very small number of people in the world, are able to do or have attempted to do that.

Host: Yeah, absolutely. And it is an incredibly complex procedure in the first place. And to be able to do that with the blood flow still continuing is really just kind of amazing, if you understand the procedure itself. So, the Norwood procedure being complex and risky, can you tell us how this approach actually helps reduce the surgical risks?

Dr Nicholas Anderson: So, that's a good question, it's kind of why take something that's already complicated and make it more complicated. And the answer really is in trying to improve the outcome, so trying to find ways to take this scary, dangerous operation and actually make it safer for patients, what we found is that if we can successfully perform the operation with the heart beating, children actually have a much smoother recovery. And when they come out of surgery, when they leave the operating room, they're actually much more stable and much healthier. And so, we found that this actually can provide for a much more smooth postoperative recovery, a shorter postoperative recovery, and ultimately improved survival.

Typically, at the national level, the survival rate for the Norwood procedure is around 85%, meaning 15% of kids will not survive the operation. Using this new perfusion technique, I've been able to get that survival number up to around 95%, meaning that only about 5% of children in my hands have not survived the Norwood procedure. So, I do think there's a real benefit to having a systematic approach to the Norwood operation that includes use of these kind of sophisticated perfusion techniques.

Host: Yeah, absolutely. So, the previous, it's almost an induced hypoxic state that you're putting the patient in during the procedure itself and therefore, recovery, if you're continuing that perfusion, certainly is going to make a big difference. And you already talked kind of about what the survival rates under this new approach is. You said 95%. So, how does this help prepare these patients for the next surgery? Because it's a series of three surgeries.

Dr Nicholas Anderson: Exactly. So, children with these single heart defects, you know, they do require multiple surgeries, and it's important that each one goes as well as possible so that they're as healthy as possible when they're going into that next operation and they haven't accumulated a lot of injury or morbidity along the way. And so, the things that we're really seeing, in addition to improved survival is, again, kids are having less injury as a result of the surgery. So, their kidneys are healthier, their lungs are healthier. And then, one thing I'm really interested in also is getting kids home as quickly as possible so that they're spending less time in the hospital.

One thing I'm really worried about is if we have children with complex medical conditions who need multiple surgeries, that can lead to a long period of time spent in the hospital during their childhood, which can be detrimental to sort of neurodevelopment, learning how to be a kid, learning to walk and read and do all the normal things that kids do.

And so, I also think it's really important just to have these surgeries, really kind of be as routine and, light lifting as possible so that we can get kids homes to be normal kids in between these staged operations. And so, that all boils down to a metric called hospital length of stay, essentially keeping track of how many days kids are in the hospital after surgery. And I've also seen very good reductions in hospital length of stay using these new techniques.

Host: Yes. That's absolutely phenomenal. And then, we talked about the fact that they need more surgery after the Norwood. So, can you share a little bit about what surgeries follow the Norwood procedure?

Dr Nicholas Anderson: Most often, children require two more surgeries. The first one is called the Glenn operation, which is performed around six months of age. And then, the last one is called the Fontan operation, and that's performed around three years of age. Both of those operations are a lot simpler and a lot safer than the Norwood procedure. So, those, again, are, oftentimes viewed as a little bit more routine. Kids are also older and older kids are easier to operate on and typically bounce back from surgery a lot faster. So, we like to think of those as smaller surgeries that are a little bit more predictable. But as you mentioned, it's really important that we have kids in the best health possible going into those surgeries so that those operations later in life can be as smooth as possible.

And then, there's another subset of children who have what we call borderline ventricle heart disease, who may be able to actually undergo two-ventricle repair. And those children typically do require additional surgeries, but the timing of when those happen might be a little bit different.

Host: So, what is your hope for the future of pediatric heart surgery at Children's Health?

Dr Nicholas Anderson: So, the program is growing very rapidly, in part due to a real clear focus at the hospital level on elevating cardiac care here in Dallas, and also as a result of just the growing population of the city itself, which is growing rapidly. So, the program is growing very fast. What I'd like to see is that we really focus on tackling challenging cases and being a center in Texas and in America where people can send their hardest cases and their most difficult problems.

I'd also like to see, like we've talked about with the Norwood procedure, I'd really like to see us try to take things that are historically challenging and high risk and almost simplify them and make them not so intimidating and turn it into something that's more routine and predictable so that we can confidently offer surgery to high risk patients and be able to confidently tell people that we can expect to have good outcomes and we can expect to have a short length of stay in the hospital and demystify these challenging operations. And I think that would be another big service for the field in general, is to take some of the really hard things and simplify them so that we can offer them to more patients and make them safer and easier surgeries to undertake.

Host: Yeah, absolutely. It's a great vision, great aspiration, especially to help patients in what would be a difficult time for their family. So, anything else you'd like to add?

Dr Nicholas Anderson: Maybe the only other thing to mention is that we're taking an approach to streamline and organize our care for all single-ventricle heart patients into a very holistic organized approach. And so, we're actually starting a single-ventricle heart center. And patients with single-ventricle heart defects have some of the most challenging heart defects of all children with congenital heart abnormalities. And we really feel that it's important that we are able to offer a holistic service from the day kids are born until adulthood with all the required specialists and different expertise involved. And so, I do think that will ultimately be a tremendous benefit to children in the state of Texas with single-ventricle heart defects to have really a dedicated center that's designed specifically for them and the unique problems that they have.

Host: That's fantastic. It really is. Dr. Anderson, thanks for helping us understand more about this new approach to the Norwood procedure, more about hypoplastic left heart syndrome. It's been great talking to you.

Dr Nicholas Anderson: Of course. Thank you.

Host: And thank you 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/heart. And if you found this podcast helpful, please rate and review or share the episode and please follow Children's Health on your social channels.