Helping the Youngest Patients with Congenital Heart Disease with Dr. Tess Saarel

Podcast Transcript

Nada Youssef: Hi. Thank you for joining us. I'm your heart, Nada Youssef, and today we're taking your questions regarding congenital heart problems, so please send us any questions you may have in the comment section below. Today our featured expert is Dr. Tess Saarel, chair of the pediatric cardiology here at Cleveland Clinic Children's, and we're very happy to have you. Thank you so much for coming.

As always, keep in mind this is for informational purposes only, and it's not intended to replace your own physician's advice. Thank you so much for being here. If you want to take just a few minutes to introduce yourself to our viewers?

Tess Saarel: Sure. As you said, my name's Tess Saarel. I'm a pediatric cardiologist here at Cleveland Clinic, and I happen to specialize in heart rhythm disorders, so electrophysiology, but I see kids from birth, and I also adults who were born with congenital heart disease, so we really take care of kids from before birth, all the way throughout their life, who were born with heart problems.

Nada Youssef: Great. Excellent. Now, congenital heart disease, also known as CHD, is the most common birth defect in the United States, and occurs in one in every 110 births. Now, nearly 5000 of those affected are also considered complex defects, also called CCHD. But before we get into the disease and explaining everything, I would like to know: What's a normal blood flow of the heart look like? Can you just kind of explain that?

Tess Saarel: Sure. The heart has four chambers, so half the heart, which is called the right side of the heart, takes back the blue blood from the body. The blue blood goes from your legs and your arms and your head, back to the right atrium, which is the upper right chamber. It passes through a valve into the lower right chamber, which is a pumping chamber that pumps your blood to your lungs. When the blood goes to the lungs, it gets oxygen, so it turns from blue to red, comes back to the upper left chamber, which collects the red blood from the lungs, goes down through a valve to the left ventricle, which is the pumping chamber of your body. That's sort of the workhorse of the heart, the left ventricle. And then the left ventricle pumps the blood out a valve to your body, brings blood and oxygen to your brain, to your other organs, to your muscles, and your body uses the oxygen. The blood turns blue, and then it comes back to the right heart. So it's four chambers that work together with the lungs to really bring oxygen to your body.

Nada Youssef: Sure, very good. Very good explanation. Thank you for that. So now knowing what a normal, healthy looks like, what is a congenital heart disease? What are those?

Tess Saarel: There's a huge range. The heart is complex, with the four chambers and all the blood vessels that have to come back to the heart, so you could imagine that it's easy for something to go wrong. Defects range from very minor, so it could be that one blood vessel didn't make it to the right spot; to serious, where you're actually missing a full half your heart. It can be a combination of different defects, so it involves blood vessels that are either veins or arteries. It involves the chambers themselves, the chambers that receive blood, the holding chambers in the top, or the lower chambers that pump. Any of the valves in between them can be affected, or the walls between the chambers can have holes in them, and that can also be a defect. It's probably the most common congenital problem because the heart is so complex.

Nada Youssef: Yeah, a lot can go wrong.

Tess Saarel: Yeah. There are a lot of things that have to go right to make a perfect heart. It's actually kind of amazing that it only happens at about 1 in 10, you know?

Nada Youssef: Yeah, that's true. That's very interesting. Well, I read there's 18 different types, correct, of CHD?

Tess Saarel: At least.

Nada Youssef: At least. There may be more?

Tess Saarel: Yeah. Since there's so many different things that could happen, we do try to put things in categories. Those are categories of different types of defects you can have, and then within those 18, there's kind of subtle differences with different types of heart disease.

Nada Youssef: That makes sense. Now, are there most common types that maybe we can talk about a little bit?

Tess Saarel: Sure, yeah. Luckily, about half the defects are relatively minor, so may not need surgery, or a child can do well all the way to adulthood without having problems. Those might be, for example, a hole in the heart, in the lower chambers. The wall between the lower chambers has to grow together, and it's happening throughout fetal life, so sometimes it just doesn't quite grow all the way together before birth, so you might have a small hole when you're born, but that will often close on its own, for example. That would be a minor type of heart disease that we just follow along, and most of the time it cures themselves, and then more serious things need surgery early on.

Nada Youssef: Sure, sure. Now, all these develop while the woman's pregnant? This is happening ...

Tess Saarel: Yes.

Nada Youssef: Okay, so this is all pre-birth?

Tess Saarel: Yes.

Nada Youssef: Okay, very good. Now, I wanted to talk about the tetralogy of Fallot, since awareness of this disease got a big bump when Jimmy Kimmel shared the story on air in front of everybody about his son and the open-heart surgery. So if you can talk about that, because I've heard that it's the most common complex defect, correct? And it has like, four defects?

Tess Saarel: Yes. What happens in tetralogy of Fallot is the wall between the lower two chambers, so the two pumping chambers on the right and the left ... again, we talked about having to grow from the bottom to the top together? Well, what happens is, the upper wall misses. So it's actually, it moves towards the right side of the heart, so you end up with different defects, what we call defects. The four things would be: a hole between the lower chambers where there's a gap; an overriding aorta, so the blood vessel to the body is moved rightward, so it's above the hole; then you get narrowing of the blood vessel to the lungs, the pulmonary artery. So that's the combination of defects that you can get, and it can be lower, below the valve. The valve itself tends to be small to the lungs. So that's tetralogy of Fallot.

There's a range of how the blood vessels to the lungs look. Sometimes they're tiny, but they're connected to the heart. Sometimes that valve is so small that it becomes completely closed, and we call atresia, and then the blood vessels are not actually connected to the heart anymore, the ones that go to the lungs. That's called pulmonary atresia with tetralogy of Fallot. That's the most serious form. This ranges sort of mild problem to serious, depending on how far that wall misses. If it misses only a little, it's not as serious, but if it misses by a lot, it's very serious. Jimmy Kimmel's son had the most serious form.

Nada Youssef: Oh, he did? Wow.

Tess Saarel: Yeah, so his son needed surgery early on, because otherwise he wouldn't survive. He wouldn't have any red blood to go to his body.

Nada Youssef: Now, this is nothing that could be preventable.

Tess Saarel: That's a great question. There are some forms of heart defects that are related to toxins like alcohol. There's fetal alcohol syndrome, some babies have this problem with heart defects. Some medications can actually affect ... They're called "teratogenic," meaning they can affect the development of the heart. But most cases, it's not preventable. We're trying to figure out the genetics of the heart disease. In some cases it's inherited, but most cases it's actually not directly inherited from your family either. It's a big area of research. We're trying to figure out exactly what causes these defects, because then we can try to help correct them in utero before the baby's born, or prevent them.

Nada Youssef: Wow, very interesting. Now, can you have this disease for the rest of your life if it's not harmful? I know we talked a little bit before the live about the Olympian winner, and he's an adult?

Tess Saarel: Yeah, that's another range of tetralogy of Fallot. Sean White also has tetralogy of Fallot. Look at him. He's ...

Nada Youssef: Amazing.

Tess Saarel: How many gold medals does he have? And did you see him at ...

Nada Youssef: I watched some of it, yeah.

Tess Saarel: His snowboarding was amazing.

Nada Youssef: Yeah, definitely.

Tess Saarel: But he's actually had three or four heart surgeries.

Nada Youssef: Wow.

Tess Saarel: He had one as a young child. He had tetralogy of Fallot as well, same as Jimmy Kimmel's son, but his was not quite as severe. He had a surgery when he was young to correct the problem, did well, obviously, trained in snowboarding. The pulmonary valve needed to be replaced again when he was older, and once or twice. So, look at him. Kids can actually do quite well, but it is something that has to be ... They need checkouts throughout their life, because sometimes you need a little tune-up.

Nada Youssef: I see. So it's not something that goes away. You always have to kind check on it in your adult years.

Tess Saarel: Yeah. It depends on how severe the original problem was. Some are one-and-done and you're pretty much okay. Some cure themselves. Other things, if it's more complex congenital heart disease where you needed a surgery, you usually have to be checked, and we're actually trying to learn ... Before, at my age, a lot of babies with these type of heart defects would not have survived. We've gotten so much better at heart surgery over the years, and we're really learning about the outcomes in adulthood and how to make our surgeries better when a baby's born, so they're great when they're 60. That's another area of research, we're trying to figure out the best approach and the best operation

Nada Youssef: That's great. Now, is treatment always surgery? Is there any other kinds of treatment?

Tess Saarel: That's a great question. The vast majority are still surgical. We can do more and more with a catheter now, so avoiding opening the chest, so avoiding incisions and stitches. More and more we can do through your veins or arteries. We can go in within a catheter and tweak a valve or close a hole with a device, or balloon a valve. So not 100% is surgical, but the vast majority is surgical.

Nada Youssef: Okay, that's great. Then actually, my youngest daughter, personally, was born with a heart murmur. So of course, it was terrifying to hear your daughter is born with something that you can't control. Can you talk a little bit about what heart murmurs are? Thank God on my end, it was nothing harmful.

Tess Saarel: Lucky, that is probably the low-severity end.

Nada Youssef: Yes, yeah, but they still sometimes say that they can hear it, so I'm just wondering what it is.

Tess Saarel: That's a great question, and this is something that moms and parents ask all the time. All a heart murmur is, is you're hearing the blood flow through the heart. If you listen carefully enough, you can hear the blood flow through the heart in everyone, or if you have a special stethoscope that makes it louder. Everybody here has a heart murmur. If you didn't, you wouldn't have your heart pumping, right? So that's not a good thing. But the question is, is it a normal murmur or abnormal? Normally it flows pretty smoothly. It's kind of like a hose that's flowing, and when it's wide open and flowing, you can't really hear much. You just hear a little ... we call it vibratory murmurs, or like water flowing through a brook without rocks in it. Smooth-sounding. If there's blockage to the blood flow or if it's under high pressure, it becomes turbulent. Like you say, you put your finger over the end of the hose and you can hear that spray? That's an abnormal heart murmur.

We hear that when we listen with our stethoscope for the tone, how the pitch ... Is it low pitch, high pitch? Is it at the wrong timing of the heart? We're supposed to hear blood flow when the heart squeezes and it pumps the blood, but when it relaxes, you're not supposed to hear a heart murmur. If we hear it at the wrong time, or the pitch of the murmur's wrong, then it's abnormal. It's actually a skill to diagnose abnormal heart murmurs, and cardiologists like me spend a lot of time practicing and getting taught, and sometimes you also hear abnormal heart sounds when the valves close or open. They're really subtle little clicks. We spend a lot of time doing that.

Nada Youssef: So a stethoscope is the only way you can find out about a heart murmur.

Tess Saarel: Yeah. If they're really loud, you can feel them.

Nada Youssef: Oh, wow. You can feel them?

Tess Saarel: Yes, because it's so loud that it transmits to the surface, so that would be the loudest ... Yeah, we grade them on how loud they are. 1 is really soft, where you can kind of barely hear it. 6 is the loudest for systolic, and that you can hear without your stethoscope.

Nada Youssef: Wow. And the louder, the more complex.

Tess Saarel: Yeah. The louder is usually, how severe the blockage is to blood flow. It's one of the things that cardiologists will do when they listen to heart murmurs, and pediatricians.

Nada Youssef: Sure, great. Thank you for that. Now, what I wanna ask is, how do you know what's best for your child? Open heart surgery or any kind of heart surgery is terrifying. Do you have anything to say to parents that just are looking at treatments, at surgery, what to expect?

Tess Saarel: That's a great question. There are different outcomes for results of surgeries. This is a very highly skilled field. Surgeons who can operate on a heart the size of a walnut, and fix the valves and fix the holes, and do it well, are not that common. The results do vary by surgeon and by center, so you need all the diagnosis correct. You need good surgery, you need good care to have the best outcomes. So actually there's a big push in the congenital heart field for transparency. There's a huge push, really driven by the parents and the patients, along with the caregivers like me and others, who are advocates, to release data. Share, be transparent, say what your outcomes are. I would encourage parents to actually look at that data. There is more and more on the internet available. We have large registries where we track outcomes over time in congenital heart disease. It's there and we're trying to push for even more transparency.

I think this is part of the push towards regionalizing heart care. You don't want to have to go too far for care, but you also want to ... If you get a really good surgery when the baby's young, it pays off the rest of their life, whereas if you don't, it means more surgeries and maybe less quality of life. So I would push parents to look on the internet, look at outcomes, reach out to parent groups, word of mouth. It's not necessarily built into the medical system yet to triage that way, but someday I'm hoping it will be. So look at outcomes, be an advocate for your child. Do some research. It's common, but not that common, so your pediatrician or your doctor might not know all the details. So, yeah. Do a little homework and ask around.

Nada Youssef: Yeah. Definitely, definitely, and I'm sure every parent does that too when they find out about their child. Now, before I go into live questions, I wanted to talk about what we developed in 2016: a 3D-printed model which allowed us to examine the probability of whether a procedure could improve oxygen saturations. Can you please talk about that, and how that changed the approach of pediatric heart surgery?

Tess Saarel: Yeah, that's actually really exciting. We're always looking for ways to help our patients even more and get better outcomes, and this actually was a game-changer for patients who had the most severe form of heart disease. Patients, for example, who had vein problems and artery problems and chamber problems and valve problems, heart all mixed up. So we are always ... We do imagings, so we get pictures of the heart, with ultrasound or MRI or CT, and we're trying to put it together. What does the heart look like? How is it functioning? And explaining that to the surgeon who actually has to come up with the surgery to help correct it. The nice thing about these models is that actually, you actually make it in 3D, and you can see it better, and you can actually open the heart and look inside and say, "I can make this chamber go to that chamber and fix this valve." Or go in and look, say, "That's not gonna work," and come up with a new operation. So we've actually had ... In the last couple of years, we've had several kids that were deemed inoperable...

Nada Youssef: Until this came along?

Tess Saarel: Yeah, and it's exciting. It makes me really happy.

Nada Youssef: Yeah, so based on MRIs and CT scans, you can make a 3D model and-

Tess Saarel: Yup. You put them all together, and the team here is excellent at putting them together into these models, and then really, we've created brand new surgeries for them. They're unique. Their heart's unique. They have a unique surgery, but we had one little boy that went from being so blue that he couldn't ... Actually, he was in a wheelchair because he couldn't walk, because when your muscles don't get enough oxygen, it hurts. They get pain. So he went from being in a wheelchair all the time because it hurt to walk, to riding a scooter and running down the street because he got an operation, so that's really exciting. So the 3D heart models are a new way of imaging the heart, that has allowed us to ...

Nada Youssef: A hands-on experience.

Tess Saarel: Yeah, and the surgeons can actually practice and know where they're gonna make their incision, and so that makes the operation go better. And the novelty is really just putting together the images.

Nada Youssef: Wow, that's amazing.

Tess Saarel: Yeah, it's really cool.

Nada Youssef: It's a good time to be alive.

Tess Saarel: Yeah, it is.

Nada Youssef: Well, we're getting some live questions. I have Kristen, "If I have ..." I'm gonna butcher this one. Bicuspid aortic?

Tess Saarel: Yup. Bicuspid aortic valves and pulmonary valves.

Nada Youssef: "Should I have my children checked? Should I have my oldest checked?"

Tess Saarel: Yes, you should. Particularly with bicuspid aortic valves and other bicuspid valves, there's actually a 50% chance of inheriting that. So the recommendations are nationally to have all first-degree relatives checked for this, so that means your parents or your children should be screened with an echocardiogram at some part. That's a good question.

Nada Youssef: Great, yeah. Then we have Jamie, "Does a child always have a congenital heart disease at birth, or can it come at any age?"

Tess Saarel: That's also a great question. "Congenital," by definition, means it was present at birth, but sometimes the symptoms don't come on till later, and sometimes the exam can actually be normal until you're older. So it is possible to have congenital heart disease diagnosed when you're older, even though it was present when you're younger. I hope that helps. It doesn't really come on when you're older, but it doesn't show any symptoms or signs till you're older.

Nada Youssef: Great, thank you. Then I have Wendy, "Is it true that a child with CHD won't be able to exercise or have a normal life as they grow up, and can you please explain?"

Tess Saarel: That's a great question as well. That is not true. Most of my patients who were born with congenital heart disease go on and exercise, play sports. You would never know that they've had a surgery unless you open their shirt and you see a scar. Sean White, for example. There's a perfect example of a child who's had three heart surgeries since baby, and no one can say he's not living a supernormal life, in terms of activity. So there are some forms of heart disease that are really serious, like for example, some kids are born with only two chambers of their heart, instead of four. So the surgery for that ... We can never fix the heart. We can never create a new chamber. Not yet. We're working on that. That's another ...

Nada Youssef: That's the research ... Maybe we can go into that.

Tess Saarel:  That would be great. But, those kids can be a little bit limited in what they can do. We try to allow them to do as much is safe and that they can do. So that's a good question. Really, kids can have great quality of life, even if born with congenital heart disease.

Nada Youssef: Great. Great to know. Omar's asking, "They closed my hole four months ago. Can I do a normal sport activity?" I don't know how old Omar is.

Tess Saarel: Yeah, that's another great question. Depends where the hole was, and what your heart looks like after it was closed. Most of the time, for example, that's where the residual lesions come in. If the hole was closed, the heart functions now normal, there's no residual leak, usually your doctor will let you go back to sports activities probably about now. The first three months, the sternum, the breastbone's still healing, so we usually recommend letting that completely heal before you go to contact sports, for example. But usually around now, if things look good in your heart, will be a time when you can start going back to activities, but that's something I would check with your doctor and get some personal advice.

Nada Youssef: Great, thank you. Lana's asking, "What should you do if your child has signs of AFib?"

Tess Saarel: Oh, that's a good question. AFib is quite rare in little kids. It's very common as we get older. In fact, some people consider AFib a normal aging process. It's extremely rare in newborns. It almost never happens, and I might say "never," although I'm not assuming ... never say never.

Nada Youssef: Never say never, sure.

Tess Saarel: But it almost never happens in newborns, and then by the time you're 80 or 90, way more than half the people have it. So AFib in kids would be unlikely, but if you're talking about palpitations or an irregular heartbeat, the thing to do would be talk to doctor. There's lots of great tests we can do now to check the heart rhythm and record it and then diagnose AFib, or maybe it's a normal heartbeat that ... otherwise the child's feeling an abnormal, feeling palpitations, but the rhythm's normal. That could be diagnosed by monitoring. So I would recommend, if you think your child has it, talk to your doctor, go get them checked out, and we can tell whether or not they have AFib.

Nada Youssef: Now, what does a daily routine look like for a child that just had heart surgery?

Tess Saarel: Okay, that's a good question too. It depends on the age, but usually you come in, you have your surgery. You spend a couple of days in the intensive care unit, because during surgery, you sometimes have to stop the heart. That's kind of a stress on your body. We put you on the breathing machine. You have to get your body used to coming off that support. That usually takes a couple days, then sometimes there's the fluid that builds up.

We have to wait for that to go away, so after the first couple days in the intensive care, you usually go to a regular hospital where we just monitor you for these fluid around your heart, and other things after surgery, and then you go home. And then, initially you'll be on some medications. Usually you need a little water pills for a little while, to get rid of that extra fluid that your body retains in surgery, and then slowly build up your activity, wean off those post-op medications, and then go back to school at some point and back to sports at some point.

Nada Youssef: So, does their meal plan change at all after surgery?

Tess Saarel: That's a great question. Not so much for kids. Most of the time we're just trying to get kids back to eating normally, and that will take a little while. First time, when you first come out, you want clear liquids, and then move onto things you can drink and then, usually they're picky for a little while. They don't want to eat as much, but then they ramp back up.

Nada Youssef: Sure, sure. Especially after such a ...

Tess Saarel: We should all be eating heart-healthy diets.

Nada Youssef: Of course, yeah. Mediterranean, you suggest?

Tess Saarel: Yes, there you go.

Nada Youssef: Okay. That's what every heart doctor's been saying.

Nada Youssef: Now, I have, Dan is asking about the Fontan procedure? Am I saying that right?

Tess Saarel: Mm-hmm (affirmative).

Nada Youssef: And if you can just talk a little bit about what that is.

Tess Saarel: Yup. A Fontan palliation is a treatment for the most severe form of heart disease. That's when you're born with really only half a heart, so one chamber that can pump to the body only. That chamber has to do the work pumping blood to the lungs and the body at the same time.

Nada Youssef: Wow. Double the work.

Tess Saarel: Yeah, so what the Fontan is, you actually bring the blue blood from your head, your upper body and your lower body, and you actually bring it directly to the lungs without going through the heart. It bypasses the heart, so it's actually a passive flow, and it goes through the lungs, and then on the left side which is the pumping chamber of your body, the blood is actually pulled out of the lungs when the heart relaxes. It actually actively relaxes, blood fills the heart, so it sucks it out of the lungs, and then pumps it to the body. So it's doing both the work of bringing blood through the lungs and to the body. It's pretty cool. Actually, this was another game-changer. When you're born with half a heart, when I was training early on, those babies unfortunately didn't survive.

Nada Youssef: Didn't make it.

Tess Saarel: No, and we ... That's so hard with the families, and we kept them comfortable and supported the families, but they didn't make it past a few months.

Nada Youssef: That's very sad.

Tess Saarel: Yeah, so this is actually a real step forward for these patients.

Nada Youssef: That's amazing. Now, we only have a few minutes, but do you want to talk about research that's always being done for better outcomes? I'm guessing we're always, always doing research.

Tess Saarel: We are. Things like ... Research brought us the 3D model, for example, so that's allowed us to really improve care, but there are other areas where we have to figure out a lot in congenital heart, so not only better treatments like surgeries or other catheter interventions, but also prevention. So we're trying to figure out what exactly causes the heart disease. Some of its genetic, but not all of it, so we're trying to figure out environmental factors. What can do we in utero, so when Mom's pregnant, to prevent these defects? We need research there. There are also some congenital heart problems that, as someone asked about, don't show up till you're older, and could actually present with sudden death. So you seem completely healthy and then you die suddenly, and that's an area that I'm interested in trying to figure out, how do we detect that before the worst happens?

Nada Youssef: Sure. So right now there's nothing you can do, treatment-wise, while the woman's pregnant?

Tess Saarel: Well, for the severe forms of heart disease, we actually do some in utero treatments.

Nada Youssef: Oh, okay. Okay.

Tess Saarel: Yeah, but not early on, so not really preventative. Not like, at the first trimester. Later on, if we don't think a baby's actually gonna make it to term, sometimes we'll do in utero, so while Mom's pregnant, we do surgery on the baby through the abdomen.

Nada Youssef: Wow, amazing.

Tess Saarel: Our fetal care program here is ramping up to do fetal surgeries, there's a lot of exciting things on the horizon to help make sure that all babies with congenital heart disease make it, or that we can prevent it.

Nada Youssef: Sure, and that's why research is very, very important.

Tess Saarel: Yeah.

Nada Youssef: Well, that's all the time that we have for today, but is there anything that you want to tell our viewers before I let you go? Or did we touch on everything?

Tess Saarel: I think that's just about everything. I mean, the message is, it's the most common birth defect, but it's treatable, and these kids do well, and can go on and live a great life and great quality of life, through support and advocacy ... making sure we have insurance coverage for these patients is a big deal, and also, supporting research would be terrific.

Nada Youssef: Thank you. Thank you, it's been a pleasure having you here.

Tess Saarel: Yeah, thank you.

Nada Youssef: And make sure you guys join us next Tuesday on Facebook here, we're gonna have dietician Kate Patton, and we're gonna talk about heart-healthy eating. For more health tips and information, please follow us on Facebook, Twitter, Instagram, and Snapchat @clevelandclinic, one word. Thank you, I'll see you again next time.