February 4, 2022
It’s National Heart Health Awareness Month! Congenital heart defects are the most common birth defect among the kids that we care for, and perhaps contribute to some of the most fear inducing moments in the newborn nursery. The good news is that many congenital heart lesions are treatable with very good outcomes, if detected and treated early by cardiac specialists.
Join our Kid Expert Dr. Christine Hills, pediatric cardiologist, as she discusses our ability to detect congenital heart defects on fetal ultrasound, and how we can detect them postnatally through pulse oximetry screening if they are not picked up prenatally. She also shares with us some practical strategies for initial management of newborns with heart disease in the first few days and weeks while they await cardiology intervention or follow up.
Transcript
Dr. Angela Kade Goepferd: This is Talking Pediatrics, a clinical podcast by Children’s Minnesota, where the complex is our every day. Each week we bring you intriguing stories and relevant pediatric health care information, as we partner with you in the care of your patients. Our guests, data, ideas, and practical tips will surprise, challenge, and perhaps change how you care for the most amazing people on Earth, kids.
Welcome to Talking Pediatrics, I’m your host, Dr. Angela Kade Goepferd. February is heart health awareness month, and in the spirit of promoting awareness and education about heart disease, we have invited one of our favorite pediatric cardiologists, Dr. Christine Hills to join us on the podcast today. Dr. Hills has multiple areas of expertise and interests in cardiology, including the genetic basis of cardiovascular disease, as well as fetal echocardiography, complex single ventricle disease and management and treatment of lipid disorders. Today I’ve invited her to talk to us about heart disease and newborns, and the role for screening and early intervention. Dr. Hills, thanks for joining me today.
Dr. Christine Hills: Thank you, it’s lovely to be here.
Dr. Angela Kade Goepferd: I didn’t mention this in the introduction, but we’ve actually known each other for quite a long time, we went to medical school together. I won’t say how long ago, it was a long time ago. Then we also did our pediatric residency training together. So what led you down the path to pediatric cardiology?
Dr. Christine Hills: When I was an undergraduate with a major in journalism and advertising and publishing in social anthropology, I did not anticipate that I would eventually end up in medical school. However, I was working at a nonprofit program that was tracking outcomes for pediatric cardiology, and ended up learning the lingo ended up getting interested in the topic, ultimately was convinced by Dr. Mueller to apply to medical school. They accepted me and ultimately decided to do pediatric cardiology, I think because I’d already generated an interest in the area. I also really liked genetics and considered that, and I also really liked OB. Ultimately, I have been able to marry all of those interests into one field by doing fetal echocardiography, because we oftentimes see children with genetic abnormalities as well in that field.
Dr. Angela Kade Goepferd: That’s amazing. It’s great when that all comes together, all of your interests collide into one spot. I always love it when people end up in medicine who didn’t intend to end up in medicine, I feel like there’s a genuine draw to the field in those cases. You have a lot of areas of expertise that we could have you talk to us about, but I invited you here today specifically to talk about detecting congenital heart disease in newborns. The reason that I wanted to talk about this was twofold. One, congenital heart defects are the most common birth defects in kids, so it’s something that all of us encounter.
Also it’s very scary to those of us who encounter it, either knowing that a kid with congenital heart disease is going to be born, or being in the newborn nursery and really being afraid that we’re going to miss a kid who might have a congenital heart lesion, particularly a critical congenital heart lesion. Luckily, there’s pretty good technology these days for detecting things prenatally, so we have a little bit of an idea of what’s going on. Given your expertise in fetal cardiology, can you tell us a little bit about how good we are at picking up congenital heart disease these days?
Dr. Christine Hills: So as you say, we attended medical school a long time ago. I would say 40 years ago we did not have a field known as fetal echocardiography. In fact, the people that were starting to study that were being told there was not a possibility, there was not going to be the technology available to look at baby hearts during a pregnancy. Here we are now, we’re looking at baby’s hearts when they’re 12, 13, 14 weeks pregnant, and being able to detect complex heart disease with decent accuracy. There’s a lot of variability. If you look throughout the country, the reported rates of fetal detection of congenital heart disease ranges anywhere from 20% to 60%, which means that we’re still missing a lot of babies who have complex heart disease that’s going to be detected after they’re born. What we have found is that by training sonographers who are doing the routine ultrasounds of babies during pregnancies on how obtain good heart views, that we’re increasing and improving our accuracy and our detection rate.
We’ve asked sonographers when they’re doing those ultrasounds out in the clinics throughout the five-state area, if they have concerns because they’re not seeing heart views appropriately, or they have concerns about what they’re seeing, to either reach out to us and send us those images, or to refer the patient to us so that we can take a better look. Sometimes we find the heart is normal, we just weren’t able to get good views on it in those initial screens. Other times we find there is a problem and that can sometimes change where the baby is going to be born, such that we can have cardiology available and cares available to take care of that baby appropriately as soon as they’re born. We have of course other times where the babies are born someplace where they did not know the baby was going to have complex heart disease, and it’s something that’s detected after they’re born.
Dr. Angela Kade Goepferd: Now, when you say that sonographers are getting more comfortable with detecting heart disease, how would someone know if they were an expecting parent if the sonographer that they’re seeing, or if the OB practice that they’re affiliated with has someone who is going to be good at detecting that? Is there a certification, or how would someone know?
Dr. Christine Hills: There is actually a certification that sonographers can obtain that shows that they have received training in fetal echocardiography and taken tests that show that they are skilled at this particular type of sonography. We also when we have babies that are born and brought to Children’s with complex congenital heart disease who were not diagnosed prenatally, our fetal director, Dr. Howley reaches out to the place where they had their prenatal screening, and is happy to work with the sonographers to help them review those images and see if there was something that could have been perhaps picked up, or if she can offer them different views they could have obtained that might have been able to make the diagnosis prenatally.
Dr. Angela Kade Goepferd: Because I am in education here at Children’s I do have to plug that Dr. Howley has started a course specifically on fetal cardiology to try to teach that skill to folks to get better, specifically designed for sonographers. I know that’s a big goal of hers is to try to increase that. If we’re picking up 20 to 60% prenatally that means that there’s 40 to 80% that we’re not. Can you talk a little bit about the importance of screening and detection postnatally, and particularly maybe the pulse oximetry screening that we’re doing in the newborn nurseries?
Dr. Christine Hills: As you say, fetal echocardiography is not perfect. What we are trying to do with babies after they’re born is to prevent a catastrophic event from happening at home because of undetected heart disease. I would say that percentage of the babies that are referred to us postnatally are picked up in the immediate newborn period because the baby doesn’t pink up after being born, or struggles to breathe or appears to be in distress and just doesn’t transition in the normal newborn way. That causes the delivering providers and the NICU or the staff that’s there at the delivery to recognize something isn’t right, and to start the investigation to trying to determine if it’s heart, if it’s lungs, what exactly the problem is. We have another percentage of the babies that seem to be well after they’re born, and they are brought to the newborn nursery or they are being cared for in the mother’s room or the parent’s room.
Dr. Christine Hills: At 24 hours of age we do a routine pulse ox screening. Pulse oximetry screening is mandated in the state of Minnesota at every location where babies are delivered. What this does is it checks the baby’s oxygen saturation in their right hand, as well as one of their feet, and determines whether that’s normal. You would think this is a pretty simple test you’re not drawing blood, you’re not doing something that hurts the baby, it doesn’t require significant training to be able to obtain. But in five out of six cases it determines babies who have significant heart disease that have not otherwise been detected prenatally. The reason it does so is the majority of the complex heart disease results in mixing of the blood within the heart. That results in the baby not having normal saturations. We consider normal saturations to be 95% and above, so basically an A plus on a test. You can get a B plus on a test and still pass, and it depends on whether we’re seeing saturations that are significantly low or significantly different between the arm and the feet.
Places that deliver babies have algorithms that are available to them they’re on the Minnesota Department of Health. There’s flyers that they can print out that they can look at. There’s also videos they can watch that talk about the screening and how this babies can fail the screening because of poor equipment, or because the baby’s crying or because they’re under bili lights. All these different things that can affect the outcomes from that testing. When a baby doesn’t pass the test, they initially are allowed to try it two more times. An hour apart each time. If after three tries the baby doesn’t pass the test, then we recommend additional either screening or testing.
Dr. Angela Kade Goepferd: That would involve probably an echo or consulting with a cardiologist or something along those lines I would imagine?
Dr. Christine Hills: Most of the times you’re right. When we first started this program, we did get some concern from places where echocardiography is not easily available. What are we going to do with these babies if they don’t pass the test? The answer is you are going to observe them further, you’re going to do further investigation. That might involve doing an arterial blood gas to see if the oxygen saturations really are as low as what the test is suggesting. It might suggest getting an X-ray to see if the problem is actually that there’s a problem with the lungs as opposed to with the heart.
If the baby doesn’t pass the pulse oximetry screening and we’re not finding another reason for the baby to be blue or hypoxic or cyanotic, then we typically would say please call a cardiologist. There’s a cardiologist at Children’s, there’s a cardiologist at the University of Minnesota, at Mayo Clinic, in Duluth, in St. Cloud, in Fargo. There are pediatric cardiologists available to people throughout the state of Minnesota that are delivering babies that can be consulted to determine whether an echocardiogram can be obtained locally, or whether we are recommending that the baby be transferred someplace where an echocardiogram can be obtained.
Dr. Angela Kade Goepferd: In the case that the screening doesn’t pick up a congenital heart lesion because five out of six still isn’t six out of six, what are some other things that you would commonly think of, or that we would see that might indicate that a baby has a congenital heart lesion?
Dr. Christine Hills: There is a convenient little pneumonic named HEART. How convenient it naturally matches what we’re talking about. That gives parents and providers a way to think about ways that babies will present if they do have a structural heart problem that hasn’t been picked up to that point. The H stands for heart rate. If you have a baby whose heart rate is too fast or too slow, that can indicate a problem or irregular. The E stands for energy, which means a baby that is either lethargic, not eating well because they’re too sleepy, or they’re just not waking up appropriately. Or a baby who’s very irritable and acts like they’re in pain or they’re otherwise in distress. The A stands for appearance. A baby that looks pale or blue in appearance is a baby that have underlying congenital heart disease. The R stands for respiratory, so a baby who’s breathing fast or appears to be struggling to breathe.
Babies who have complex congenital heart disease usually have holes in their heart, and whereas their lungs start to relax that can send too much blood flow to their lungs, makes their lungs wet. Their lungs get very heavy and they don’t exchange oxygen as well, and that results in babies who start to breathe fast and hard trying to counteract that. The T stands for temperature. A baby in Minnesota in the wintertime can feel cool to the touch, but if they’re wrapped in a blanket and they’re snuggled up warm and you unwrap them and they still feel cold, that can be a concern either for congenital heart disease or sepsis, other infections that babies can have. Both of which require pediatrician or a family practice doctor to take a look and see if they have concerns.
Dr. Angela Kade Goepferd: I mentioned that one of the reasons I wanted to talk about congenital heart disease is that I think there’s something that feels scary to us about it as pediatricians. Knowing that, what are some of the things that you’ve seen that are maybe common missteps that people make with newborns who either are discovered to have congenital heart disease, or we know they do prenatally and maybe it’s not handled as well as it could be in a hospital setting? What are some words of advice that you can give to those of us who are in newborn nurseries and meeting babies in those first few hours of life, for kids who are either discovered to have an abnormal screening or kids who we know going in are going to have a congenital heart defect?
Dr. Christine Hills: The first thing I would say is to have an open line of communication with your referring pediatric cardiologist or a pediatric cardiologist that you can reach out to if you have questions or concerns. They can help come up with a care plan for the babies. Some of the babies especially those who have holes in their hearts, their saturations are not going to be normal. Of course our instinct as pediatricians and as care providers for babies is to try to make their saturations normal. We will have babies who as cardiologists are perfectly happy having their saturations be 85%, because that means there’s an equal distribution of blood flow to the lungs and to the heart. But if we want their saturations to be higher, we might put them on oxygen to try and get their saturations to look better. But what that does is make the lungs relax, and it sends too much blood flow to the lungs, which ultimately makes the babies breathe harder and have more difficulty called congestive heart failure.
If the pediatrician are told by the cardiologist that we’re okay with the saturations being 85%, I would not start to treat with oxygen trying to upset that apple cart and make it change that scenario. We also have times where I think people are afraid to reach out because they feel that we should be experts in every field because of our pediatric trainings. I’m in awe of our pediatricians and our family practice doctors and providers in terms of all that they’re expected to know. I just want them to be aware that we are more than happy to talk to them at any time of the day or night to review a case and help provide a care plan and guidance and what we expect to be seeing.
Dr. Angela Kade Goepferd: We have a kid who has been identified as having a congenital heart lesion, and potentially they’re going to be having a surgery in the first few months of life. We are the primary care provider for the family, and the family is understandably quite anxious about this and coming to us to say what can we expect? What should we be doing? One of the final questions I have for you is any advice that you have for primary care clinicians who are going to be with a child or a family who has particularly like a critical congenital heart lesion that’s going to be having some surgery and intervention?
Dr. Christine Hills: The patients that we have that are in what we call our single ventricle monitoring program, these are patients who are born with very complex heart disease, such as hypoplastic left heart syndrome. They are particularly vulnerable to events that could happen at home because they are dependent on blood flow to their lungs through shunts or somewhat vulnerable vessels. These patients are actually managed in a different way than our traditional patients that are just coming to clinic for visits here and there. These patients are sent home with pulse oximetry equipment, they’re sent home with a scale.
They have a number that they can call a nurse practitioner in our single monitoring program 24 hours a day if they have concerns about how the baby is looking, or if there’s a change in the saturations or in how the baby’s eating or growing or gaining weight. Such that if there are concerns we can act on it sooner than waiting until their next clinic appointment, or waiting until morning, or waiting until they can get to an emergency room. With that we have been, knock on wood, very successful in keeping our babies safe so that they are able to come for their next surgery without having untoward events at home.
Dr. Angela Kade Goepferd: I would imagine that the same thing applies in this case as in the newborn nursery, where if I have a patient who is at home with all this monitoring equipment and something doesn’t feel right, and they call me because I’m on call for the pediatric clinic and I’m unsure, that you and your colleagues are more than happy for us to give a call even if it’s in the middle of the night to find out what we should be doing.
Dr. Christine Hills: Absolutely. In fact, we would rather hear when there’s a concern so that we can help guide things that can be done locally. In terms of maybe we just ask that the baby be brought to a local emergency room so that we can get an X-ray or that we can have them check the saturations, or to get an accurate weight so that we know if the baby is indeed losing weight or not gaining weight and help troubleshoot it locally. Then if there are concerns then we can arrange for a hospital to hospital transfer if needed.
Dr. Angela Kade Goepferd: Well, thank you so much that’s been very helpful. Is there any other last words of wisdom or advice you’d like to impart to our listeners about congenital heart disease in the newborn period?
Dr. Christine Hills: I would let people know that we have become very successful in terms of our ability to take care of babies that have heart disease, even complex heart disease. If you looked a generation ago the babies were not as likely to survive early surgeries and/or do well at school as they got older, or to be able to grow up into adults who are fulfilling and enjoying their lives. At this point we actually expect that the majority of the children that we diagnose either prenatal or postnatally are going to do well, and that they’re going to continue to enjoy doing activities at home or within their schools, or to participate in sports.
We have camps for children that they can attend during the summertime that are with other children who have had heart surgery. It’s really a joyful place to get to work. We of course have times where we struggle, just as the same as everyone else with the patients and their families and of course feel pain when things don’t go the way we want them to go, and that’s hard for all of us. But the families and the children that do well are what carry us through and make this a enjoyable field.
Dr. Angela Kade Goepferd: Well, thank you so much for all of your advice and expertise today, we really are thankful for our cardiology kid experts here at Children’s Minnesota. I’m thankful to get the opportunity to chat with you today given our long history together, so thanks so much for coming in.
Dr. Christine Hills: Thank you.
Dr. Angela Kade Goepferd: Thank you for joining us for Talking Pediatrics. Come back each week for a new episode with our caregivers and experts in pediatric health. Our executive producer and showrunner is Ilze Vogel. Episodes are engineered, produced, and edited by Jake Beaver. Lexi Dingman is our marketing representative. For more information and additional episodes, visit us at childrensmn.org/talkingpediatrics, and to rate and review our show, please go to childrensmn.org/survey.