Transcription:
An Overview of Congenital Heart Disease

Conan Gasque: Welcome to Inside Pediatrics Podcast, brought to you by Children's Hospital of Alabama in Birmingham. I'm Conan Gasque. February is American Heart Month. And with that in mind, we're talking about congenital heart disease. I'm joined by Dr. Camden Hebson, a pediatric cardiologist here at Children's of Alabama. Dr. Hebson, thanks so much for your time.

Camden Hebson: Thanks for having me here.

Conan Gasque: So again, we're talking about congenital heart disease. Tell me a little bit about, is this pretty common, not so common, how often does this happen?

Camden Hebson: Yes. So, congenital heart disease is the most common birth defect, congenital malformation that we as human beings have. It's 1% of all people will have some form of congenital heart disease. And then breaking that down further, about one in four patients will have what you can call critical congenital heart disease where they might need a surgery for their heart disease, you know, in the first year of life.

Conan Gasque: So, you were talking about 1% of all people have it. I guess that's probably a lot more common than maybe what the average person realizes.

Camden Hebson: Yes. So, it can be, again, things that needed to have surgery in the first year of life to forms of heart disease, maybe changes in valves that are mild, but really won't come up until you get to be a teenager or even an older adult, and then finally gets diagnosed.

Conan Gasque: So, there are critical forms and non-critical forms as they're categorized. Can you tell me a little bit about the critical forms, what some of those are and what that means exactly?

Camden Hebson: Sure. So, a couple of broad categories would be congenital heart disease that's significant enough that it's causing heart failure symptoms. And then babies, heart failure symptoms would be a baby that's breathing too hard and too fast, or is having difficulty with feeding, that's a lot of work, that's a baby's form of exercise, is to feed. So if that form of heart disease is causing heart failure for them, those things will come up significantly, even as an infant.

And then, the other version would be heart disease that would cause their oxygen levels to be low. That's cyanotic congenital heart disease. So instead of having normal oxygen saturations in the high 90s, it might be down in the 80s or 70s because of how the blood flow to the heart is going before a series of surgeries or palliations or fixes are done.

Conan Gasque: And I guess that's why it's called critical because these are types of congenital heart defects that need to be addressed urgently because the life of the baby is at stake.

Camden Hebson: Yes. And I think potentially the life of the baby or quality of life too would be what we can make better with addressing what is going on inside the heart with a surgery or a heart catheterization procedure to make that better.

Conan Gasque: What are some of the examples of these congenital heart defects that are considered critical?

Camden Hebson: So, the most common form of cyanotic or low oxygen saturation congenital heart disease is tetralogy of Fallot. There's cousins of tetralogy of Fallot that are similar, truncus arteriosis, double-outlet right ventricle. These are different names for these particular heart defects that have some common manifestations with the baby having low oxygen saturations as one of the things in particular that's happening.

Conan Gasque: You mentioned tetralogy of Fallot. What exactly is going on in the heart when a baby has that condition?

Camden Hebson: Sure. So, it's tetralogy, so it's four things. The big things are that there is a hole, there's a VSD inside the heart, between the left and the right sides of the heart. There's obstruction to blood flow going to the lungs, pulmonary stenosis. The right side of the heart gets thickened. And then the aorta, the main blood vessel of the heart going back out to the body sits over the top of that VSD. So, those four things sort of all kind of happened together because of how the heart's formed, but that particular heart defect can be fixed surgically so that the children really do very well after their procedure.

Conan Gasque: Is there anything that a parent needs to know if they find out that their child has been diagnosed with tetralogy of Fallot?

Camden Hebson: Well, I think, you know, one of the first things, from a parent standpoint, is that we have gotten better and better at picking all these different heart defects up. And one of the most straightforward ways that's being done is called congenital heart screening, it's pulse oximetry screening. It's using pulse ox to check the levels in the different limbs of the body. And that's now done, should be done, in newborn babies after they're born. And we've got an algorithm by which the nurseries operate to say these oxygen saturations are too low. It could be too low in the hands or too low down in the feet. And that then alerts the nursery to say this could be because of a congenital heart defect, and therefore we need to have a cardiology consult, an echocardiogram to see if this is a real congenital heart defect that's causing this or not.

So, the first thing that, I think, a parent or a family could do is just make that the nursery, the hospital they're delivering at, they do congenital heart screening. And if they do, you can then feel good about the fact that they're going to be the most equipped to pick up congenital heart defects early on.

And then, after a patient is diagnosed with one of these congenital heart defects, the good news is we've got people to help. So, you'll have a cardiologist. Eventually, you'll have a heart surgeon. This place has really good people to do that, and they're going to walk you through all the way on how this is going to be addressed. And with most congenital heart disease now, patients do really well long term. You know, for example, there are more adults who have congenital heart disease now than kids because kids live through their pediatric years and become adults and still have that same congenital heart disease. So, the expectation is these kids are going to do well over time. But getting your cardiologist, becoming informed, you know, asking your cardiologist questions so that they can give you feedback on it, that's how it goes, and it typically goes well.

Conan Gasque: So, I guess one of the earliest keys is making sure that it is picked up on early.

Camden Hebson: Right. It's a decently simple thing to just apply the pulse oxes to the babies to pick it up. But, you know, sometimes telling that the baby's oxygen levels are low can be kind of subtle. So, this is just formally doing it with pulse ox and the different forms of cyanotic or the low oxygen saturation congenital heart disease come up. You can see it, the pulse ox is low. So, I think it's been a great intervention to pick those defects up. And then because of that, those babies get treatment started right away, rather than, you know, waiting until finally somebody notices it as an outpatient.

Conan Gasque: And I guess so many advances have been made over time, that that's part of the reason that many of these children are surviving well into adulthood.

Camden Hebson: Yeah, the surgeries have come a long way. The surgeons here at Children's of Alabama are excellent. The teams around them are excellent. The ICU team is excellent, and that's nationwide too. But here in Alabama in particular, we've got a really good team. So first up, picking up the diagnosis, making sure that we got it right, and then getting the right people involved to make sure that that child gets the right care to get them to do well over time.

Conan Gasque: And I know people hear about heart defects and they think open heart surgery. But here at Children's of Alabama and across the nation, there are a lot of more less invasive procedures as well.

Camden Hebson: Yeah, that's exactly right. So, it keeps becoming less and less invasive over time. You know, how extensive the surgery needs to be done, even if it is, but addressing things in the cardiac catheterization lab has really come a long way as well. You can put in new valves that 20 years ago, you can never do. You can put that new valve in with a heart catheterization. That doesn't require any type of open heart surgery to do that.

If you have a hole in your heart, you have an atrial septal defect, you have a hole between the left and the right sides of the heart, you can plug that hole with a device to prevent the blood flow from abnormally going through that hole anymore. And you can do that in the cardiac catheterization lab. So, many things can be addressed without open heart surgery now, and that's a really good thing.

Conan Gasque: You mentioned the atrial septal defect. I know that falls into the category of non-critical congenital heart defects. Can you talk about what some of the other non-critical congenital heart defects are and what that means?

Camden Hebson: Sure. Two really common ones would be VSDs and ASDs, ventricular septal defects and atrial septal defects. They're both holes. Atrial septal defects is a hole between the left and the right sides of the heart, on the top part of the heart, the receiving chambers. And ventricular septal defect is a hole between the left and the right side of the heart between the two pumping chambers.

ASDs and VSDs, for that matter, they come in all sizes. They can be very large, they can be really small to where the heart really doesn't even know it's there. Technically, it's there, but the heart operates just as if it wasn't there at all. And so, little small VSDs or ASDs really oftentimes we do nothing for other than say, "Hey, your child looks awesome. I'll see you again in a year or two. There's nothing we need to do." Big ones tend to cause more symptoms. So, a big VSD, for example, would be one of the things that could cause heart failure type symptoms for a small infant and then need surgery early on in life. And that would be an open heart surgery. But even those children typically have their one surgery and then that's all they ever need, and you'll see that same child when they're four or five years old running around the T-ball field no different from anybody else.

Conan Gasque: Wow. So even in the most severe cases, there are great ways to treat it that ensures that the kid lives a normal life.

Camden Hebson: Yeah. For things like VSDs, that's definitely our mindset to say that that's how this should go over time.

Conan Gasque: And the good news here in Alabama is that the Children's of Alabama Heart Center, we can treat congenital heart defects in every way that's available. How special is the heart center in terms of the things that we can do here?

Camden Hebson: So, I've been here for five years and it's a really great place. It's a place based on teamwork. I think that starts with our leadership. We've got great people in charge of the cardiologists. We've got great people. The people who are the surgeons, the ICU doctors, we all work together really well and it's collaborative and it is sort of making sure that everybody's held accountable doing things the right way too. So, you know, there's tough cases too. And on those tough cases, people come together and really try to hash it out to make sure that that child gets the best possible care plan together, and that can be a hard thing if a team has dysfunction within it. But again, I've been here five years and I've been so impressed by the people that are here and the leadership sort of making sure that this happens the right way. And when that happens, even in the really hard cases, things work out the best they can and you feel really good about that.

Conan Gasque: Tough cases, but a lot of success stories here as well.

Camden Hebson: Yeah, that's for sure. It's a really nice thing. You'll have patients that really a whole team has worked with for months or a year to really get them to a certain point, and then it'll be a nice thing. You'll get an email and it'll be from one of the social workers showing, you know, that a family shared a picture of their child. And you remember that child as this little baby that they were in the ICU. They were on a lot of support. They were having some struggles. But then, there's that child and they're sitting on Santa's lap or they're playing, the Olan Mills picture, you know, and they just look great. And you're like, you know, "That's awesome. I can't believe that that child looks that way now here these couple years later." So, it's a really redeeming and nice thing about working here.

Conan Gasque: Well, it's great to hear more about these conditions and things that parents need to know and the ways that we are treating them here at the Heart Center at Children's of Alabama. Once again, Dr. Cam Hebson, with a look at congenital heart defects. Dr. Hebson, thanks for your time.

Camden Hebson: Thank you very much. Thanks for listening to Inside Pediatrics. You can find more podcasts like this one at childrensal.org/insidepediatrics.