Specialty Spotlight with Siva: Straight from the Heart

Dr. Kade Goepferd: This is Talking Pediatrics, a clinical podcast by Children’s Minnesota, home to the Kid Experts, where the complex is our every day. Each episode, 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 kids.

Welcome to Talking Pediatrics. I’m your host, Dr. Kade Goepferd. Today’s segment is Specialty Spotlight with Siva where we delve into the complexities of pediatric medical and surgical subspecialties with pediatric otolaryngologist and facial plastic surgeon, Dr. Siva Chinnadurai.

Dr. Siva Chinnadurai: Welcome to Specialty Spotlight with Siva. This week my guest is Dr. Lisa Howley. She’s a pediatric and fetal cardiologist with 14 years of experience. She specializes both in pediatric and fetal ultra sonography and she is the director of the Fetal Cardiology program at the Midwest Fetal Care Center and Children’s Minnesota. Welcome to the podcast, Dr. Howley. It’s great to have you.

Dr. Lisa Howley: Thanks so much. Thanks for the invitation to join you today.

Dr. Siva Chinnadurai: Congenital heart disease is such a hot topic in our world of pediatric medicine and it’s a really growing and burgeoning field, and I think the increased ability to manage fetal heart disease has a big impact on a lot of other medical specialties. As we see children who are growing older with more complex diseases, there’s a huge desire in the pediatric community learn more about these children and how to best care for them and to understand a little insight into their journey and the teams that help generate the awesome outcomes we’re seeing. So that’s what I’d like to dive into with you today. We’ll start at the beginning. How common is congenital heart disease?

Dr. Lisa Howley: Actually, congenital heart disease is very common. It is the most common birth defect of any kind and occurs in about one in a hundred babies that are live born. Now, I say live born because I live in the world of fetal cardiology where actually more babies have congenital heart disease than this number here, but some babies don’t make it to a live birth, which leaves us with an incidence of a 1% or one in a hundred babies. According to the American Heart Association, about 40,000 children every year are born in the United States with congenital heart disease, and about 25% of them are going to have a critical form of congenital heart disease, meaning that they’re going to need some sort of intervention, either surgical or cardiac cath to be able to survive long-term after they’re born.

Dr. Siva Chinnadurai: Sounds like when you have a diagnosis of prenatal heart disease, sometimes this is a really serious problem as you mentioned, but sometimes it may not be quite as devastating. Is it always a serious problem when you receive a diagnosis of fetal heart disease?

Dr. Lisa Howley: In the world of fetal cardiology, I see families every single day around the metro examining babies’ hearts before birth, and there’s really a spectrum of heart disease out there ranging from very minor forms of heart disease to clearly very major forms of heart disease. So having a diagnosis of congenital heart disease does not always mean it’s going to be severe. Looking at the literature, they’ll say that if you have a fetal diagnosis of congenital heart disease, about half of those children are going to need some sort of intervention. Some of the neonatal periods, some a little bit later on in childhood, but clearly there is a population that will not require intervention after birth.

Dr. Siva Chinnadurai: Are there known risk factors or modifiable risk factors for expectant parents or people who are hoping to become parents to reduce the risk of heart disease?

Dr. Lisa Howley: So there are risk factors. I don’t know how many of them really I would consider modifiable. When I give presentations, I talk about how there’s kind of three different categories of risk factors, one being kind of maternal risk factors, then familial risk factors, and then things really specific to that fetus. I think big categories to think about is going to be a family history of congenital heart disease and specifically we’re talking about first degree family members of the fetus, so that would be mom, dad, or a sibling of the baby, and kind of a general number is that overall there’s about a three time incidence of increased risk for congenital heart disease if a first degree relative has congenital heart disease. So we do know that it runs in families. We don’t really quite understand the specifics of that, but that’s a kind of a number one risk factor.

Other things we think about are fetuses that are identified to have genetic differences in the pregnancy, and about 20% of babies with congenital heart disease will have some sort of associated genetic finding. Other major ones that we think about, there’s a lot of pregnant moms out there with diabetes, so moms with diabetes. That will increase the babies risk of having a congenital heart defect and warrant an evaluation with our team. Early exposures in pregnancy, be it either certain viruses that mom might have or a medication that she’s exposed to early when the heart is forming drugs and alcohol can increase congenital heart disease. And then lastly, something that’s very commonly talked about is assisted reproductive technologies. We’re talking about IVF and other forms of technology that we use to assist a pregnancy creation. Those also will increase the risk for a heart problem.

Dr. Siva Chinnadurai: Do you get asked by your existing families or your existing patients’ parents who may have had a child with heart disease or may have other relatives with heart disease and are thinking about their own family planning about advice or family planning counseling around the risk factors congenital heart disease?

Dr. Lisa Howley: There are many, many families that have these identified risk factors and really even before they’re getting pregnant, they’re wondering those sorts of questions of what are the risks for my pregnancy? So many families will choose to meet with a maternal fetal medicine physician even prior to pregnancy really to understand those risk factors and if there are ways to mediate them to make sure that they’re approaching that, and in some families they elect for genetic counseling even prior to a pregnancy if they know that there is a risk factor that exists that potentially could affect the fetus.

Dr. Siva Chinnadurai: It’s great that we have those resources and I assume those are also resources that our pediatricians can maybe reach out to or connect their patients families too, should that question come up in their practice.

Dr. Lisa Howley: We do have a lot of services here through Midwest Fetal Care Center. It’s a multi-disciplinary clinic that’s really a joint effort between Children’s Minnesota and Allina Health, but that fetal center is a multi-disciplinary center that has and houses lots of different providers that all can see the family in one kind of joint visit, and that includes maternal fetal medicine. I mean, clearly fetal cardiology is there, which is why I’m so familiar. But we also have genetic counseling and the NICU team, we have specialized programs for moms with congenital heart disease and with diabetes. So there’s a lot of opportunity for families to get more specialized care through this program.

Dr. Siva Chinnadurai: Congenital heart disease is oftentimes picked up on just routine ultrasound. Can you tell us a little bit more about that journey? If somebody picks that up on a routine ultrasound, what does that look like for the families and for the care team?

Dr. Lisa Howley: The journey can come in different forms for different families. I would say given the scenario that you described, this would be a family that otherwise would be low risk. They did not have an identified risk factor, but came in for their typical mid-trimester scan, which is an ultrasound performed that’s examining the whole fetal anatomy. Part of that is that there are set fetal cardiac views that must be obtained and deemed to be normal. So in certain cases, those views are not able to be obtained or do not look normal, which then kind of prompts a referral from that primary OB or family practice provider to a higher level of care for a more detailed examination of the fetus and the fetal heart, and that’s often called a level two exam, which is through a maternal fetal medicine provider and then a fetal echocardiogram that is performed by our team, so fetal cardiologists. So typically when that concern is raised in the pregnancy at the time of mid trimester is when they’ll make that referral. So many times we’ll see families around 20, 18 to 22 weeks probably typically is the most common time, and we get those referrals really from all over the upper Midwest.

Dr. Siva Chinnadurai: By the time these conditions are picked up on prenatal ultrasound, that heart anatomy is developed, it’s set, but there’s a big role for continuing to follow these families prenatally to visit with them, keep track of things as pregnancy progresses. What do you do in those visits? What’s the role for those continued visits after the diagnosis is made?

Dr. Lisa Howley: So when I diagnose families at their first visit, I tell these families that the heart is fully formed by 10 weeks of pregnancy, so the things that we are seeing are permanent findings and that these are not going to be subject to change. We don’t expect that the heart could normalize at this point. However, there’s a lot of information really to convey to these families, which part of it is going to be having these serial examinations to monitor the fetal hemodynamic stability look to see if there are further parts of the heart that we can refine in better detail when baby may be in a better position, and to make sure that there is no decline in their hemodynamic status. But I think beyond just the imaging, a lot of it is going to be educational for families. So we really try to take the time to have families understand what’s going on with their baby because this is their baby and we want to make sure that they’re part of the team and can be informed and involved in the decision making regarding their baby.

A lot of the time that we spend beyond just the ultrasound is really counseling families. It’s setting up visits with other folks on the team that are going to be taking care of baby after birth. We set up a lot of consultations, depending on the type of diagnosis that we deem for each child, so be that they may have consultations with the NICU team, the cardiac catheterization team if we think that their child’s going to need a cath or with one of our cardiac surgeons. We have three surgeons here at Children’s Minnesota, and so the families that we know will need cardiac surgery after birth, have the opportunity to sit down and meet one of them ahead of time, and we take care of families not only from the metro, but really as I mentioned from the whole upper Midwest. So we’re taking care of families from very different, more rural areas than here. A lot of it is just allowing them time to kind of familiarize themselves not only with our team, but with this area and with our program as well as have the opportunity to tour the space where their child’s going to be, because I think a lot of it is very scary until you’re able to see it and kind of connect and understand what your child’s journey’s going to be.

Dr. Siva Chinnadurai: When you meet a new family and pick up a prenatal heart condition are there other organ systems that are often involved? What are those and how do we prepare families for those challenges?

Dr. Lisa Howley: My mentor from Canada, where I trained for fetal cardiology, actually published on this and it was very eye-opening for me as I entered into pediatric and fetal cardiology, that many babies that we diagnose with congenital heart disease may have other organ systems involved. Publications will report that about 25 to 35% of babies with congenital heart disease will have extra cardiac abnormalities. Some of them will be genetic, but it may involve other organ systems. The most common ones that are talked about include urogenital anomalies, structural brain anomalies, as well as gastrointestinal anomalies. But really there’s a myriad of other conditions, which is why the fetal cardiology team really aims to always partner with a maternal fetal medicine team because jointly together we’re examining the entire fetal body carefully, and we can each kind of tap into each other for our different specialties.

Dr. Siva Chinnadurai: And as these children go throughout their life, I think from their cardiac standpoint, maybe from their other health challenges standpoint, this may be picked up as a fetal issue, but will continue to be a area that needs lifelong management. How do you help those families and those children as they grow into young adults transition out of the pediatric health care system and into the adult world managing their congenital heart diagnosis?

Dr. Lisa Howley: When we counsel them prenatally, we tell them about this, and we kind of set them on the beginning of that journey telling them that after their child is born, we set the expectations of what their child will need, including lifelong pediatric cardiology follow-up. The majority of children we diagnosed with congenital heart disease now move into the adult realm as you just mentioned. And so from our standpoint, we are very lucky in that our Children’s Minnesota pediatric cardiology team is now in a formal collaboration with the Mayo Clinic team, which allows for that lifelong care as they have a very strong adult congenital heart program down there, as well as there is one over in the Allina system. So when they have reached adulthood, we try and help create a seamless transition to an adult congenital heart team, which are cardiologists that are aimed and care for adults that are specialized in congenital forms of heart disease.

Dr. Siva Chinnadurai: Could you give us an overview of what ingredients go into making a high performing team or for people who are listening around the country and whose patients may be in need of a good cardiac team or a fetal team, what should they look for when they’re picking a team to help care for their growing family?

Dr. Lisa Howley: Just within the heart realm, the things that I can comment on is I think that there is an importance to having a depth and a breadth to the team. For us here at Children’s Minnesota, that means you want to have a number of providers with different specialties within the pediatric cardiology field. So where I am specialized in fetal and neonatal imaging, there are other team members that I have that are specialized in heart rhythm and specialized in cardiac cath and all the other forms of congenital heart disease that may need more specialized management. I also think it’s important to highlight the need for having more than one congenital heart surgeon. You need to have some bench depth so that if God forbid something happens, or a surgeon is out sick, that there was always somebody there to help take care of your child 24 7. But beyond that, I think it’s really important to be at a center that has lots of excellent pediatric subspecialists that can also contribute and help in the care of that child, especially with particularly those extra cardiac anomalies that also exist. So having a team that has pulmonologists and ENT and all those others that can help further round out the team as well as all those ancillary staff members that are so important, the dieticians, the respiratory therapists, everyone that can really support children that are medically fragile in an ICU setting are really critical for their successful outcome.

Dr. Siva Chinnadurai: You hit on another point in some of these really sub-specialized fields, your field, my field, many others. As we grow and learn more and take advantage of developments in technology and developments in science and get excited about new things, we always try to keep our feet grounded in making sure that these great advancements we’re making in science and clinical care remain accessible to everyone and that we are keeping an eye towards equity and not helping promote health care disparities, but rather combat them. I know this is a field that you’ve been active in, both in your practice and in publication. You’ve been involved in some studies looking at telecardiology outreach and other ways to combat health disparities in the growing field of fetal cardiology. Can you talk a little bit about those efforts and how we keep this great growing field that you’re talking about accessible to all of our patients?

Dr. Lisa Howley: If you look historically at the rate of prenatal diagnosis of congenital heart disease, we are unfortunately quite poor around the country in terms of prenatal diagnosis. We are getting better as we’re getting more people comfortable looking at hearts prior to birth and as our technology improves. But there are clearly factors that can impact the prenatal diagnosis and cardiac care of children, not only in the upper Midwest, but really around the country. I was part of a large multicenter study that came out a few years ago in the Journal of Circulation that was led by my good friend, Dr. Anita Krishnan, who’s out of Children’s National, and that study really demonstrated that lower socioeconomic status and Hispanic ethnicity and rural residents were all associated with decreased prenatal detection of two major forms of congenital heart disease, that very quickly after birth, without recognition, those children could unfortunately pass away.

I think that this, trying to better understand these social determinants of health is really important. I’m going to highlight one of my partners, Dr. Cathleen Pruitt, who has joined me here at Children’s Minnesota from University of Utah, has really started a research area of work here at Children’s Minnesota studying the social determinants of health here in our population and in Minnesota to try and improve and address those disparities both in the fetal and the neonatal population. So that’s currently ongoing here and there’s much more to do. I think personally, one of my greatest goals since coming to the great state of Minnesota is trying to reach into these more rural areas, either by partnering with pediatric cardiologists that are in other parts of the upper Midwest, as well as through education and doing sonographer in-service trainings to really help those sonographers recognize abnormal hearts as they’re scanning them to try and improve recognition of congenital heart disease and also make them more comfortable reaching out when they have concerns by reducing those barriers to communication with a tertiary center.

Dr. Siva Chinnadurai: As we wrap up, is there anything new developing the hottest thing in fetal cardiology that has you really excited about new developments in the field or new things to come?

Dr. Lisa Howley: I would say the hot things, and I don’t know if they’re hot to everybody, but to me personally is that when I started in this field, there was very little research that was being done, and the research that was done was all very small single center studies that really didn’t give you a whole lot of data to stand on to know what should be best practices for families as we’re diagnosing and caring for them. With the advent of the Fetal Heart Society, which is a large multi-institutional group that we are a part of and actively participate in, we’re now involved in a lot of very large multicenter studies that are ongoing, looking at different types of fetal cardiac diagnoses and really creating research that is much more robust and that we’re able to use to better help guide our practices, not only here, but really around the country. So I’m hopeful in the next couple of years that we’re going to have some really great publications that come out that are going to help us better care for families.

Dr. Siva Chinnadurai: Well, thanks for joining us today, Lisa. Today we learned that the field of fetal cardiology is growing, and not all prenatal diagnoses are critical, but about 50% are, about 25% will need surgery. And for children who are in those higher risk groups, it’s really important to pick a good team, which is characterized by a lot of specialists who can work together with deep depth in terms of their specialty and their bench, and are not only able to work with each other, but there’s a lot of learning going on in the field between centers. That’s what defines a good team, and for those listening who might need resources, including families who might want some prenatal counseling or even pre-pregnancy counseling, can reach out to your maternal fetal team to get some resources before or early in pregnancy. My guest today has been Lisa Howley, the director of Fetal Cardiology at the Midwest Fetal Care Center. Thank you so much, Dr. Howley.

Dr. Lisa Howley: Thank you so much for letting me join you today.

Dr. Kade Goepferd: Thank you for listening to Talking Pediatrics. Come back next time for a new episode with our caregivers and experts in pediatric health. Our showrunner is Cora Nelson. Episodes are produced, engineered and edited by Jake Beaver and Patrick Bixler. Our marketing representatives are Amie Juba and Krithika Devanathan. For information and additional episodes, check us out on your favorite podcast platform or go to childrensmn.org/talkingpediatrics.