Most children with congenital heart disease grow up to live active, healthy lives. It is important for these children to receive appropriate follow-up care throughout childhood to watch for signs of developmental issues.
Christopher D. Smyser, MD and Caroline Lee, MD, discuss the Cardiac Neurodevelopmental Clinic at the St. Louis Children’s and Washington University Heart Center, and how our heart specialists evaluate and treat infants and children to ensure that they reach their highest potential.
Cardiac Neurodevelopmental Clinic for Congenital Heart Disease
Featured Speakers:
Christopher D. Smyser, MD | Caroline Lee, MD
Christopher D. Smyser, MD works in Saint Louis, MO and specializes in Child Neurology. Smyser is affiliated with Saint Louis Children's Hospital.
Learn more about Christopher D. Smyser, MD
Caroline Lee is a practicing Pediatric Cardiology doctor in Saint Louis, MO.
Learn more about Caroline Lee, MD
Transcription:
Cardiac Neurodevelopmental Clinic for Congenital Heart Disease
Melanie Cole (Host): Children with congenital heart disease can grow up to live active, healthy lives, however, it’s very important for these children to receive appropriate follow up care throughout childhood to watch for signs of developmental issues. My guests today in this panel discussion are Dr. Caroline Lee. She’s a Washington University Pediatric Cardiologist, Director of the Fetal Heart Center and Medical Director of the Cardiac Neurodevelopmental Clinic at St. Louis Children’s Hospital. And Dr. Christopher Smyser. He’s a Washington University Pediatric Neurologist at St. Louis Children’s Hospital. So, Dr. Lee, I would like to start with you to explain a little bit about congenital heart disease. What are the different types and speak a little bit about the prevalence?
Caroline Lee, MD (Guest): The prevalence of congenital heart disease which is the most common birth defect is in about one percent of live births and they can range from simple heart defects that require minimal follow up or no follow up and may resolve on their own to really complex heart lesions that require medications, multiple surgical and cath procedures. So, there really is a wide range.
Melanie: Dr. Smyser how important is early diagnosis as being crucial to improve outcome predictions? Speak about how you would know if a child has congenital heart disease.
Christopher D. Smyser, MD (Guest): So, increasingly we are able to identify congenital heart disease lesions even before children are born through the improvements in imaging technology, through ultrasound and even MRI. So, we have become aware of the deficits that these children face even before delivery. And then that has led to additional improvements in terms of our ability to optimize care for these children in the immediate post-delivery period through admission to a center such as ours and optimization of both medical and surgical management. So, early recognition is one of the most important steps in terms of allowing us to meet children early in their course and then maximize the types of therapies that we are able to provide and get those introduced as early as possible to again, optimize and improve outcomes.
Melanie: Dr. Lee speak about kind of go along with what Dr. Smyser was saying about optimizing outcomes and treatments. Speak a little bit about why patients are more at risk for developmental delays, what factors will increase those delays and how they affect a child’s behavior.
Dr. Lee: We are learning more and more over time about the significantly increased risk in children with congenital heart disease for developmental delays and disabilities. And there are multiple factors beginning even before birth that we are learning that contribute to the risk for these delays. So, as Dr. Smyser discussed about we can diagnose heart defects in utero and we are having studies that show that there is abnormal brain development as well in these babies such as smaller brain volumes, abnormal brain metabolism, delayed development, different flow patterns to the brain of a baby with complex heart disease and the normal blood flow of a brain – blood flow to the brain of a normal child. So, even in utero, there’s evidence of just delays or immaturity in development of the brain structurally and functionally. And then for those children who require neonatal or infant surgeries; there are different hospital factors, physiologic factors, intraoperative factors that can also contribute to the potential for either delays in development or brain injuries.
So, for example, the most severe heart defects are those in which oxygen levels in these infants and children are lower than normal which we call cyanotic heart defects and so, chronic cyanosis; there is the risk of in the operating room for these children as well being on a cardiopulmonary bypass machine and what that can do in terms of activation of inflammatory pathways. There’s risk when we do circulatory arrest meaning stopping the heart and blood flow completely in order to perform complex heart repairs and then even in the postoperative period; in the ICU, where a child will be intubated for a prolonged amount of time receiving sedation, anesthesia; sometimes children are unstable with their blood pressures and blood flow to their brain. We even have children who unfortunately suffer cardiac arrest and require rescue with ECMO or heart lung support. So, all of these factors, not just one but multiple factors all along the course of a child with congenital heart disease is exposed to things like seizures and strokes in the postoperative period as well. So, there really are just a multitude of and cumulative contributing factors why a child may be at risk for delays.
Melanie: How important is a medical home for well child and chronic care visits? What is life like as a child grows into adolescence and even adulthood and how can a pediatrician help monitor these factors?
Dr. Smyser: So, a medical home is instrumental both in terms of the initial care that a child receives but as you alluded to, the longitudinal care as well. So, our program here begins with meeting families and affected children during the neonatal or infant period and then following those children long-term. The established relationships that we have as providers with families is instrumental in terms of again, providing continuity and maximizing the care. We, as part of this, and in addition to developments in the field have become increasingly cognizant of the long-term challenges that affected children face. So, these can affect all aspects of development, so motor, cognitive, language, and even social domains. So, we think that it’s instrumental that we are appropriately aggressive in terms of instituting therapy services early, the local pediatrician is instrumental in this process in terms of making sure that we are again offering all resources available to children in their community and also maintaining close collaboration in terms of the care of those children in the long term.
So, as you alluded to as well, these deficits evolve or change over the course of time. So, what an infant needs during the period immediately following discharge is often very different than what a child needs as they reach later childhood and even adolescence. So, close communication and collaboration with the pediatrician to stay on top of how the needs are changing and again to maintain awareness of the different resources that are available in each community are very important and can be invaluable in terms of again, making the families as comfortable as possible with the different and evolving medical conditions that they are learning about and helping to work through and with.
Melanie: Dr. Lee describe a little bit for us about the cardiac neurodevelopmental clinic at St. Louis Children’s and Washington University Heart Center and speak about your team of specialists that make up this multidisciplinary clinic. Who is involved?
Dr. Lee: Well the clinic was established in 2015 and really the impetus for this was that the shift in our focus in the field of pediatric cardiology is really changing. We have always been concerned and trying to improve survival and to mitigate morbidities, but there really has been a trend over the last decade really in shifting the focus not just from survival and cardiac outcomes, but into the quality of life for these children from neurological perspective, developmental and just overall family and quality of life. And so, it was for this reason that this multidisciplinary clinic was established knowing that as we discussed, these children have multiple risk factors for issues and we wanted to be really proactive about identifying them and providing the family with the support both medically and also within their community for therapies for their children.
So, it is a very multidisciplinary team. There is the cardiology side and I’m the physician and we have a nurse practitioner and a nurse coordinator, but really the cardiology part is just the way we get patients into the clinic, but our role there is very minimal. The primary providers for this clinic are pediatric neurologists like Dr. Smyser and his colleagues. We have newborn medicine specialists for the younger children under three years. We have a host of therapists that are key to working with our children and families and with neurology so those include physical, occupational, and speech therapists. We also have a dietician because feeding issues – we have a lot of infants with feeding tubes and delays in oral feeding, so we have a dietician and speech therapist to help us with that. But we also have a psychologist as well and in addition not directly in our clinic, but we have partnered with a couple other really important specialists that I think provide a lot of value to our heart children and those include a neuropsychologist who can perform neuropsychological testing and can make recommendations regarding accommodations for these children at school. And also, a psychiatrist as well who is able to prescribe medications for diagnoses such as attention deficit hyperactivity disorder and can help us with our children with autism spectrum disorder.
Melanie: Dr. Smyser explain a little bit about the referral process to the clinic and what a pediatrician can expect after referring a patient to this clinic.
Dr. Smyser: So, the referral process flows through our cardiology group. So, Caroline and her excellent team when a patient is diagnosed with a heart lesion that places them at high risk and then they will automatically qualify for our clinic. Often these referrals come through our inpatient heart center but certainly we have many patients who have been referred in from referring providers both cardiologists and pediatricians. So, when a patient is scheduled, they are schedule for all of the appointments by the specialists Caroline just ran through in a single day. So, we coordinate amongst our providers and allow this to essentially be one stop shopping so to speak for the families. At the end of the day, we provide the families with a summary of the recommendations across each of the providers that they have seen, and a set of goals and a return appointment time line as well. And then pediatricians will receive essentially a comparable document which outlines the assessment results, physical examination findings and equally importantly, the recommendations from each of the providers across the different specialties that the child has seen.
So, again, this is part of the partnership that we see this clinic as with the local pediatricians as well. And follow up to each appointment we talk to each family once they have gone home and make sure that all the services that we have recommended have been able to be established whether that is additional therapy or community services, something from a medical perspective. So, we close the loop in terms of communication with the families and then again link that back to the pediatricians as well following the appointments. And then again, in advance of each appointment in the future, we will touch base with the families and see what concerns they have and also make sure that any concerns raised by the pediatrician are addressed as part of each appointment. So, we try and touch base with the pediatricians at multiple points through the communication process with the families for each encounter.
Melanie: Dr. Smyser, sticking with you for a minute, you have the first last word here. Tell the listeners what you would like them to know about the cardiac neurodevelopmental clinic at the St. Louis Children’s and Washington University Heart Center and when you think it’s important that they refer.
Dr. Smyser: So, the later portion is certainly straight forward to address and that’s any time that there is any sort of concern or the possibility that we might be able to help any child and their family with a congenital heart lesion. So, we are happy for that to occur as early as the neonatal period or even now before children are born or from that perspective, any point after the child has been discharged from the hospital and or if there is concerns that arise down the road. So, we are always here and available to help and happy to help as early as possible.
So, the real strength of the clinic as we have alluded to on a few occasions from my perspective is the multidisciplinary nature. And we are able to help these families and their children in many ways through the different types of services that we are able to provide. We are very excited about the improvements in outcomes that these children are now experiencing and are very excited about the possibilities of continuing to see the services that we are able to provide grow in lock step with the improvements in outcomes that we are seeing. The way in which we are providing services from again that fetal period all the way through childhood is also something that’s very exciting to me as a provider. I get to see children grow up. I get to see all the new tricks that they can come back and show me each time in clinic. I get to know the families and establish strong relationships which is the most rewarding part of what I do. And again, I’m just very excited about all the progress that we have shown and also the ways in which we are able to continue to give back and learn new ways that we can help these children as they get older.
Melanie: And Dr. Lee last word to you. What would you like the listeners to take away from this segment about congenital heart disease and even what you see on the horizon for some promising therapies?
Dr. Lee: So, I’m just really excited as well with Dr. Smyser that our outcomes overall in congenital heart disease are improving and then to take the next step and focus on the quality of life for these children and so much of their home life and their social life has to do with their abilities to do well in school or to get along with others and to function physically and so, all of those things beyond the heart; we try and address in this clinic. And so, I think that’s something important for both parents and primary care providers to know about our focus and our aim that we are trying to achieve with this is to enhance the quality of life for these children.
So, I think we have done this now for three years. We keep growing and expanding. We have now increased our clinic from once to twice a month and we are expanding our age groups and over time, I’m hoping to expand our services not just for the focus on neurologic and developmental issues, but also on the psychological realm as well and that’s another area that we are learning more about for these children as they enter into adolescence and young adulthood for all that they have been through, that they have an increased risk for anxiety and depression and so I think that’s another horizon that I would love for our program to expand to.
But in the meantime, our focus is really on providing families no matter where they come from because we do get children from all over the state of Missouri and Illinois and so we try and work with the therapists in those communities as well. I think that’s an important point that although people make the trip to Children’s for this particular clinic; we ultimately will send them home hopefully with services in place in their local community and with their local provider. So, I see this clinic as a place where children and their families can check in if the child is doing great, developing normally, I think it’s very validating for these parents to hear that from the neurologist that their child looks really, really normal for all that they have been through. And then on the other end of the spectrum, for those children who have suffered neurological injury or sequelae from all that they have been through; that we are actively trying to address those to help them reach their full potential.
Melanie: Thank you so much both of you for joining us today and for explaining a little bit about congenital heart disease and the Cardiac Neurodevelopmental Clinic at St. Louis Children’s Hospital. Thank you again for being with us. A physician can refer a patient by calling the Children’s Direct Physician Access Line at 1-800-678-HELP. That’s 1-800-678-4357. You’re listening to Radio Rounds with St. Louis children’s Hospital. For more information on resources available at St. Louis Children’s Hospital you can go to www.stlouischildrens.org, that’s www.stlouischildrens.org. This is Melanie Cole. Thanks so much for listening.
Cardiac Neurodevelopmental Clinic for Congenital Heart Disease
Melanie Cole (Host): Children with congenital heart disease can grow up to live active, healthy lives, however, it’s very important for these children to receive appropriate follow up care throughout childhood to watch for signs of developmental issues. My guests today in this panel discussion are Dr. Caroline Lee. She’s a Washington University Pediatric Cardiologist, Director of the Fetal Heart Center and Medical Director of the Cardiac Neurodevelopmental Clinic at St. Louis Children’s Hospital. And Dr. Christopher Smyser. He’s a Washington University Pediatric Neurologist at St. Louis Children’s Hospital. So, Dr. Lee, I would like to start with you to explain a little bit about congenital heart disease. What are the different types and speak a little bit about the prevalence?
Caroline Lee, MD (Guest): The prevalence of congenital heart disease which is the most common birth defect is in about one percent of live births and they can range from simple heart defects that require minimal follow up or no follow up and may resolve on their own to really complex heart lesions that require medications, multiple surgical and cath procedures. So, there really is a wide range.
Melanie: Dr. Smyser how important is early diagnosis as being crucial to improve outcome predictions? Speak about how you would know if a child has congenital heart disease.
Christopher D. Smyser, MD (Guest): So, increasingly we are able to identify congenital heart disease lesions even before children are born through the improvements in imaging technology, through ultrasound and even MRI. So, we have become aware of the deficits that these children face even before delivery. And then that has led to additional improvements in terms of our ability to optimize care for these children in the immediate post-delivery period through admission to a center such as ours and optimization of both medical and surgical management. So, early recognition is one of the most important steps in terms of allowing us to meet children early in their course and then maximize the types of therapies that we are able to provide and get those introduced as early as possible to again, optimize and improve outcomes.
Melanie: Dr. Lee speak about kind of go along with what Dr. Smyser was saying about optimizing outcomes and treatments. Speak a little bit about why patients are more at risk for developmental delays, what factors will increase those delays and how they affect a child’s behavior.
Dr. Lee: We are learning more and more over time about the significantly increased risk in children with congenital heart disease for developmental delays and disabilities. And there are multiple factors beginning even before birth that we are learning that contribute to the risk for these delays. So, as Dr. Smyser discussed about we can diagnose heart defects in utero and we are having studies that show that there is abnormal brain development as well in these babies such as smaller brain volumes, abnormal brain metabolism, delayed development, different flow patterns to the brain of a baby with complex heart disease and the normal blood flow of a brain – blood flow to the brain of a normal child. So, even in utero, there’s evidence of just delays or immaturity in development of the brain structurally and functionally. And then for those children who require neonatal or infant surgeries; there are different hospital factors, physiologic factors, intraoperative factors that can also contribute to the potential for either delays in development or brain injuries.
So, for example, the most severe heart defects are those in which oxygen levels in these infants and children are lower than normal which we call cyanotic heart defects and so, chronic cyanosis; there is the risk of in the operating room for these children as well being on a cardiopulmonary bypass machine and what that can do in terms of activation of inflammatory pathways. There’s risk when we do circulatory arrest meaning stopping the heart and blood flow completely in order to perform complex heart repairs and then even in the postoperative period; in the ICU, where a child will be intubated for a prolonged amount of time receiving sedation, anesthesia; sometimes children are unstable with their blood pressures and blood flow to their brain. We even have children who unfortunately suffer cardiac arrest and require rescue with ECMO or heart lung support. So, all of these factors, not just one but multiple factors all along the course of a child with congenital heart disease is exposed to things like seizures and strokes in the postoperative period as well. So, there really are just a multitude of and cumulative contributing factors why a child may be at risk for delays.
Melanie: How important is a medical home for well child and chronic care visits? What is life like as a child grows into adolescence and even adulthood and how can a pediatrician help monitor these factors?
Dr. Smyser: So, a medical home is instrumental both in terms of the initial care that a child receives but as you alluded to, the longitudinal care as well. So, our program here begins with meeting families and affected children during the neonatal or infant period and then following those children long-term. The established relationships that we have as providers with families is instrumental in terms of again, providing continuity and maximizing the care. We, as part of this, and in addition to developments in the field have become increasingly cognizant of the long-term challenges that affected children face. So, these can affect all aspects of development, so motor, cognitive, language, and even social domains. So, we think that it’s instrumental that we are appropriately aggressive in terms of instituting therapy services early, the local pediatrician is instrumental in this process in terms of making sure that we are again offering all resources available to children in their community and also maintaining close collaboration in terms of the care of those children in the long term.
So, as you alluded to as well, these deficits evolve or change over the course of time. So, what an infant needs during the period immediately following discharge is often very different than what a child needs as they reach later childhood and even adolescence. So, close communication and collaboration with the pediatrician to stay on top of how the needs are changing and again to maintain awareness of the different resources that are available in each community are very important and can be invaluable in terms of again, making the families as comfortable as possible with the different and evolving medical conditions that they are learning about and helping to work through and with.
Melanie: Dr. Lee describe a little bit for us about the cardiac neurodevelopmental clinic at St. Louis Children’s and Washington University Heart Center and speak about your team of specialists that make up this multidisciplinary clinic. Who is involved?
Dr. Lee: Well the clinic was established in 2015 and really the impetus for this was that the shift in our focus in the field of pediatric cardiology is really changing. We have always been concerned and trying to improve survival and to mitigate morbidities, but there really has been a trend over the last decade really in shifting the focus not just from survival and cardiac outcomes, but into the quality of life for these children from neurological perspective, developmental and just overall family and quality of life. And so, it was for this reason that this multidisciplinary clinic was established knowing that as we discussed, these children have multiple risk factors for issues and we wanted to be really proactive about identifying them and providing the family with the support both medically and also within their community for therapies for their children.
So, it is a very multidisciplinary team. There is the cardiology side and I’m the physician and we have a nurse practitioner and a nurse coordinator, but really the cardiology part is just the way we get patients into the clinic, but our role there is very minimal. The primary providers for this clinic are pediatric neurologists like Dr. Smyser and his colleagues. We have newborn medicine specialists for the younger children under three years. We have a host of therapists that are key to working with our children and families and with neurology so those include physical, occupational, and speech therapists. We also have a dietician because feeding issues – we have a lot of infants with feeding tubes and delays in oral feeding, so we have a dietician and speech therapist to help us with that. But we also have a psychologist as well and in addition not directly in our clinic, but we have partnered with a couple other really important specialists that I think provide a lot of value to our heart children and those include a neuropsychologist who can perform neuropsychological testing and can make recommendations regarding accommodations for these children at school. And also, a psychiatrist as well who is able to prescribe medications for diagnoses such as attention deficit hyperactivity disorder and can help us with our children with autism spectrum disorder.
Melanie: Dr. Smyser explain a little bit about the referral process to the clinic and what a pediatrician can expect after referring a patient to this clinic.
Dr. Smyser: So, the referral process flows through our cardiology group. So, Caroline and her excellent team when a patient is diagnosed with a heart lesion that places them at high risk and then they will automatically qualify for our clinic. Often these referrals come through our inpatient heart center but certainly we have many patients who have been referred in from referring providers both cardiologists and pediatricians. So, when a patient is scheduled, they are schedule for all of the appointments by the specialists Caroline just ran through in a single day. So, we coordinate amongst our providers and allow this to essentially be one stop shopping so to speak for the families. At the end of the day, we provide the families with a summary of the recommendations across each of the providers that they have seen, and a set of goals and a return appointment time line as well. And then pediatricians will receive essentially a comparable document which outlines the assessment results, physical examination findings and equally importantly, the recommendations from each of the providers across the different specialties that the child has seen.
So, again, this is part of the partnership that we see this clinic as with the local pediatricians as well. And follow up to each appointment we talk to each family once they have gone home and make sure that all the services that we have recommended have been able to be established whether that is additional therapy or community services, something from a medical perspective. So, we close the loop in terms of communication with the families and then again link that back to the pediatricians as well following the appointments. And then again, in advance of each appointment in the future, we will touch base with the families and see what concerns they have and also make sure that any concerns raised by the pediatrician are addressed as part of each appointment. So, we try and touch base with the pediatricians at multiple points through the communication process with the families for each encounter.
Melanie: Dr. Smyser, sticking with you for a minute, you have the first last word here. Tell the listeners what you would like them to know about the cardiac neurodevelopmental clinic at the St. Louis Children’s and Washington University Heart Center and when you think it’s important that they refer.
Dr. Smyser: So, the later portion is certainly straight forward to address and that’s any time that there is any sort of concern or the possibility that we might be able to help any child and their family with a congenital heart lesion. So, we are happy for that to occur as early as the neonatal period or even now before children are born or from that perspective, any point after the child has been discharged from the hospital and or if there is concerns that arise down the road. So, we are always here and available to help and happy to help as early as possible.
So, the real strength of the clinic as we have alluded to on a few occasions from my perspective is the multidisciplinary nature. And we are able to help these families and their children in many ways through the different types of services that we are able to provide. We are very excited about the improvements in outcomes that these children are now experiencing and are very excited about the possibilities of continuing to see the services that we are able to provide grow in lock step with the improvements in outcomes that we are seeing. The way in which we are providing services from again that fetal period all the way through childhood is also something that’s very exciting to me as a provider. I get to see children grow up. I get to see all the new tricks that they can come back and show me each time in clinic. I get to know the families and establish strong relationships which is the most rewarding part of what I do. And again, I’m just very excited about all the progress that we have shown and also the ways in which we are able to continue to give back and learn new ways that we can help these children as they get older.
Melanie: And Dr. Lee last word to you. What would you like the listeners to take away from this segment about congenital heart disease and even what you see on the horizon for some promising therapies?
Dr. Lee: So, I’m just really excited as well with Dr. Smyser that our outcomes overall in congenital heart disease are improving and then to take the next step and focus on the quality of life for these children and so much of their home life and their social life has to do with their abilities to do well in school or to get along with others and to function physically and so, all of those things beyond the heart; we try and address in this clinic. And so, I think that’s something important for both parents and primary care providers to know about our focus and our aim that we are trying to achieve with this is to enhance the quality of life for these children.
So, I think we have done this now for three years. We keep growing and expanding. We have now increased our clinic from once to twice a month and we are expanding our age groups and over time, I’m hoping to expand our services not just for the focus on neurologic and developmental issues, but also on the psychological realm as well and that’s another area that we are learning more about for these children as they enter into adolescence and young adulthood for all that they have been through, that they have an increased risk for anxiety and depression and so I think that’s another horizon that I would love for our program to expand to.
But in the meantime, our focus is really on providing families no matter where they come from because we do get children from all over the state of Missouri and Illinois and so we try and work with the therapists in those communities as well. I think that’s an important point that although people make the trip to Children’s for this particular clinic; we ultimately will send them home hopefully with services in place in their local community and with their local provider. So, I see this clinic as a place where children and their families can check in if the child is doing great, developing normally, I think it’s very validating for these parents to hear that from the neurologist that their child looks really, really normal for all that they have been through. And then on the other end of the spectrum, for those children who have suffered neurological injury or sequelae from all that they have been through; that we are actively trying to address those to help them reach their full potential.
Melanie: Thank you so much both of you for joining us today and for explaining a little bit about congenital heart disease and the Cardiac Neurodevelopmental Clinic at St. Louis Children’s Hospital. Thank you again for being with us. A physician can refer a patient by calling the Children’s Direct Physician Access Line at 1-800-678-HELP. That’s 1-800-678-4357. You’re listening to Radio Rounds with St. Louis children’s Hospital. For more information on resources available at St. Louis Children’s Hospital you can go to www.stlouischildrens.org, that’s www.stlouischildrens.org. This is Melanie Cole. Thanks so much for listening.