Pediatric Cardiology and Congenital Heart Defects

Patrick Flynn, M.D., discusses the important issues on pediatric cardiology and congenital heart defects. He shares the latest on treatment options available for children affected. He also highlights the importance of screening family members and how the care team provides treatment and counselling to those affected.
Pediatric Cardiology and Congenital Heart Defects
Featured Speaker:
Patrick Flynn, MD
Dr. Flynn is an Associate Professor of Clinical Pediatrics at Weill Cornell Medicine and Associate Attending Pediatrician at NewYork-Presbyterian Hospital/Weill Cornell Medical Center. 

learn more about Patrick Flynn, MD
Transcription:
Pediatric Cardiology and Congenital Heart Defects

Melanie Cole (Host):  There's no handbook for your child's health, but we do have a podcast, featuring world-class clinical and research physicians covering everything from your child's allergies to zinc levels. Welcome to Kids Health Cast by Weill Cornell Medicine. I'm Melanie Cole, and I invite you to listen as we discuss Pediatric Cardiology and congenital heart defects. Joining me is Dr. Patrick Flynn. He's an Associate Professor of Clinical Pediatrics at Weill Cornell Medicine. Dr. Flynn, it's a pleasure to have you join us today. For the listeners, give us a little working definition. What is congenital heart disease and how common is it?

Patrick Flynn, MD (Guest): Well, thank you, Melanie. It's a pleasure to be here and congenital heart disease would be any structural problem, that a child could be born with that is different than the normal structure that we know of the human heart. Fairly consistently, the prevalence of congenital heart disease is about eight per thousand. So, a little bit less than 1% of babies are born with something wrong with the heart. And that, makes congenital heart disease, the most common of the structural congenital heart problems that a child could be born with.

Host: So then Dr. Flynn, how's it identified? When does it become apparent that a child has some form of congenital heart disease, whether it's in utero or right after birth? When are these things found?

Dr. Flynn: It's a great question because in that, group of congenital heart disease, about a third of congenital heart disease is what we consider serious. Meaning that it's a heart problem that would require some type of intervention surgically or in the cath lab in the first year of life. And those, nowadays, in modern medicine, are usually identified prenatally, in the form of a fetal echocardiogram, which is an ultrasound like any other ultrasound. Not every pregnancy has a fetal echocardiogram.

So, there are certain risk factors that a pregnancy might have. Probably the most useful one though, is any sort of aberration seen on the regular level two obstetrical ultrasound that might identify the need to look at the heart. So, most of the heart disease that will either cause an infant to be sick, either cyanotic or blue or have trouble with cardiac output after birth, will be identified prenatally. We still have our certain ones that will come up postnatally. But the outcomes of babies with serious congenital heart disease have become so much better in the modern era because we don't have the surprise postnatal diagnoses.

Now, beyond that, what about the other two thirds? The other two thirds are usually picked up on evaluation, after the pediatrician notices something like a heart murmur or some other problem, during infancy or even during childhood.

Host: So, you just mentioned heart murmur. Are they a problem? Is this something that parents need to be afraid of?

Dr. Flynn: That's a great question. Heart murmurs, depending on the age, are very, very common. And a heart murmur is really just the noise coming out of the heart. And the heart is actually making noises all the time. So, we always do like to remind people and if a parent of an infant or a child is being referred to the Pediatric Cardiologist for a heart murmur, we want them to know from the very beginning that they're very likely, when they're bringing their healthy child or infant in for that evaluation, at the end of the visit they're probably going home with just as healthy a child as they came in with. I always like to say that if you bring me a 100 four-year-olds, I'll hear a heart murmur on 70 of them. And obviously when the incidence of congenital heart disease is less than 1%, that 70% of children that have heart murmurs have to, the vast majority of them be normal. The most common of the murmurs is something that we call a Still's murmur, which is actually a musical note that the heart is making because one of the valves is held together by little strings. And when the heart squeezes, the valve closes and the strings twine, just like a guitar string or a piano.

So, we’ll always say that we hear a heart murmur on 70% of four-year-olds in a quiet enough room, if it's, as a Pediatric Cardiologist, it's the only skill that we have. So, I don't expect the pediatrician to hear that many heart murmurs. But again, in a child that's that age, if they're cooperative enough to lie flat on the table, the heart rate is low enough for us to hear these noises and their chest wall is still thin enough for us to have the perfect acoustics for that. So, that's the kind of preparedness that we like our parents to have when they come in. It's always a little frightening to come to a sub-specialist office. But some of those heart murmurs might indicate small holes between the pumping chambers of the heart or a narrowing or a leakiness of one of the valves, which needs to be identified because those need to be followed up through childhood and occasionally need intervention later on.

Host: Then let's talk about diagnosis and treatment. Tell us a little bit about fetal echocardiography and then what would you do once you determine something's going on?

Dr. Flynn: That's a great question. Fetal echocardiography is, a great modality. We can see the heart typically very well, right around the mid portion of pregnancy. We like to do fetal echocardiograms in the 18 to 20 week period of time. And there, we can see how the heart has developed in those first few weeks the pregnancy. We can't rule out all of the congenital heart lesions, especially some of the more common ones and that's because the circulation of the fetus is very different than it will be after birth, but the major heart lesions, at around that age, we should be able to identify. And when we do, it gives us plenty of time to discuss those problems, and what the treatment would be and what the prognosis is with those families.

They get the information and make the active decision whether to continue the pregnancy or not. Most people do, even in the situation of significant structural heart disease, because our outcomes are so good. And part of the reason that the outcomes are so good with open heart surgery, even in the newborn period, is the fact that we have the prenatal diagnosis. We have a plan before birth. We don't allow babies to be born and then have to get sick for somebody to identify that there might be heart disease. We do much less playing catch up than we did 30 years ago when I was training, when most babies were not diagnosed prenatally and we would have a two or three-year-old baby in shock and have to kind of unravel the pieces, and have to deal with the organ damage that might be happening.

Now we're able to intervene right away. We usually, in those situations where there is structural heart disease, we'll have the parents have the opportunity to meet and consult with our Pediatric Cardiothoracic Surgeons prenatally. So that when everything happens after birth, it's a much more controlled experience and environment, that's certainly much better for the baby and for the families themselves.

Host: What great points that you made. So, as you're telling us about services that are offered at Weill Cornell Medicine in Pediatric Cardiology, tell us about the other specialists that are involved and how important a multidisciplinary approach is for these patients and their families.

Dr. Flynn: Well, that's a great question because I think it's really one of the most rewarding things about Pediatric Cardiology is the collaborative nature of the entire field. Starting again with our Pediatric Cardiac Surgeons. I think one of the interesting things in Pediatric Cardiology is that the relationship between the Pediatric Cardiologist and the Pediatric Cardiothoracic Surgeons is not really like anything else that exists in any other disciplines.

If you walk into a major medical center that has pediatric heart surgery, and you find your way to the cardiothoracic surgery department and asked to meet the pediatric guy, they probably won't know where he or she is. In that situation, the Pediatric Cardiothoracic Surgeons are generally located in Pediatric Cardiology because we work so closely together. And we're probably one of the only medical specialties that is routinely found in the operating room because a Pediatric Cardiologist for open heart surgery will be present at the beginning and the end of every heart surgery to perform an echocardiogram through the throat, to look at the anatomy before the operation, and then to actually review the repair at the end.

So, we work very closely, not only with the Pediatric Cardiac Surgeons, but with the Pediatric Cardiac Anesthesiologists in the operating room, because, the three of us are all in there participating in the care of the patient from start to finish. Then of course we have great relationships and great collaboration with our Pediatric Critical Care Specialists who take care of the patients afterwards, and it's a true teamwork. In our institution, the Pediatric Critical Care people and the Pediatric Cardiologists formally round together at the beginning of the day, and participate with the parents at the bedside, in the care of the patients on an ongoing basis.

This is also true for the babies that are in our Neonatal Intensive Care Unit. And then beyond that we have relationships, not only with the general pediatricians that are referring us our patients, and then also participating in the care after discharge for surgical patients, but also the other pediatric subspecialties and most notably our pediatric geneticists who are so important in the overall management of our children with congenital heart disease and their families. So, having all of those specialists around and having them in an environment where we really do have the opportunity to collaborate together and to work as a team, is not only effective for our patients, but it's also really, probably one of the most rewarding aspects of our job.

Host: Which leads me perfectly into our last question. As you summarize for us, for parents, this is very scary, Dr. Flynn, for any parent. And as one who experienced this myself, I can say that when I was pregnant, it was probably the most stress I'd ever felt in my life. And you Pediatric Cardiologists are what got me through and helped me to feel less stressed and understand what was going on.

As you're summarizing this for parents and giving them hope about all these exciting things that you can do for them, tell us also how rewarding this is for you as a Pediatric Cardiologist to do what you do and help families to come out on the other end and see the sunshine.

Dr. Flynn: Well, it's a great field, especially for that. And I think one of the most rewarding parts is first of all, that we do get to take care of a lot of patients who come and go. Most of the patients that I'll see this afternoon whether they're coming for a heart murmur or chest pain or palpitations or fainting, most of those children are not going to have a serious heart problem. That's very rewarding. But our babies that do, these are relationships that we keep forever. I have patients, I've been at this long enough that I have patients that I've diagnosed in utero., I’ve taken care of them through multiple surgeries. And now we've had to pass them on to probably one of the most important collaborators that I didn't mention earlier. And a great field of medicine that we have now that we didn't have then, which is our Adult Congenital Heart Disease Specialist.

So, when we have those situations of people with significant heart disease, that we now get to see them, take them through sometimes multiple surgeries, watch them grow into teenagers, and then young adults, it's incredibly rewarding. I actually have one patient who I've taken care of since she was three days old, who's now graduated from medical school and is a pediatric resident, with an eye toward becoming a Pediatric Cardiologist.

And when that day comes, I'll be happy to just kind of hand the keys over and let her take over for me. But that's the thing to remember. And we know that when we talk to parents, the last thing anybody wants to hear is that there's any need for us beyond that one day. But when we do have to be involved because there's congenital heart disease, we have to have them understand it in the moment and understand the things that we will need to be doing as we go forward. But at the same time, we have to give them that broader view toward the future. That we're very, very hopeful. And hopeful for good reason that we're going to be able to get their baby through the surgery and through the infancy and then childhood, and then annual visits where we pretty much get to high-five each other on the terrific outcome that they've had.

That's the kind of view that we want the parents to have, as we know, they're focusing so much on the present. But it really is not a Pollyannish view of our field. I think it's the realistic view of Pediatric Cardiology and Pediatric Cardiac Surgery that we really have extraordinarily more satisfying and happy days than we do days of difficulty.

Host: Very rewarding. Thank you so much, Dr. Flynn for joining us today and telling us about your field and letting parents know that there are a lot of exciting things you can do for their children and why there's hope and what you can do for them at Weill Cornell Medicine. And Weill Cornell Medicine continues to see our patients in person as well as through video visits. And you can be confident of the safety of your appointments at Weill Cornell Medicine. I want to thank our listeners and that concludes today's episode of Kids Health Cast. Please remember to subscribe, rate and review this podcast and all the other Weill Cornell Medicine podcasts.   For more health tips and updates like these, please follow us on your social channels. I'm Melanie Cole.