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As Pediatric Congenital Heart Patients Age Out, Where do They Go?

With the growing number of pediatric congenital heart patients reaching adulthood thanks to ever improving medical care, Dr. Pilgrim takes us inside one of the few programs in the nation that bridges the gap to adult care without missing a beat.

Meet the speaker

Dr. Scott Pilgrim

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Host: Hello and welcome to Cook Children's Doc Talk. Today we're talking with Dr. Scott Pilgrim, who is the medical director of the Adult Congenital Heart Disease program here at Cook Children's. Dr. Pilgrim is board-certified in pediatrics, internal medicine, pediatric cardiology and adult congenital cardiology. Prior to coming to Cook Children's, Dr. Pilgrim founded the adult congenital heart disease program at the Steven and Alexandra Cohen Children's Medical Center in Long Island. Today, under his leadership, the Adult Congenital Heart Disease program at Cook Children's Medical Center has shown tremendous growth with over 500 adult visits every year. Welcome Dr. Pilgrim.


Dr. Pilgrim: Thank you.


Host: So growing up with a sister who had heart surgery as a child had a major influence in your life, and ultimately led you to your role here at Cook Children's as medical director of the ACHD program, compared to many other cardiac specialties, adult congenital as a specialty is relatively new, can you give us a little history.


Dr. Pilgrim: So, as you mentioned, my sister was born with a congenital heart disease. And so growing up with this in my family, it became of interest to me at a very young age. But as a field, congenital heart disease is something that has been around obviously, for several decades, this field of adult congenital heart disease has grown primarily because of the success stories that we've had in pediatric cardiology, namely, the surgical outcomes and the longevity of patients related to improved surgical care, improved surgical technique, you know, recognizing disease, early on fetal diagnosis, and so on and so forth. And so the incidence of congenital heart disease really hasn't changed. But the lifespan of individuals growing up with congenital heart disease has tremendously improved. And so when we think about the growth of adult congenital heart disease in the country, it really has grown out of necessity, because these kids are now growing up and becoming adults. In fact, the number of adults with congenital heart disease now far outweighs the number of children with congenital heart disease in the United States, almost to a 60-40 split. That care gap that has then been created, because of the increase in the number of adults with congenital heart disease has put a lot of strain on adult and pediatric services, namely, because adult providers may not be familiar with the congenital heart disease and pediatric providers may not be familiar with adult onset disease. And when you have adult onset disease in the context of an underlying congenital heart problem, it certainly can lend itself to some unique challenges. And that's where the field has essentially been born out of. Over the last decade or two, there has been a real push for subspecialty care in this field, so that individuals are actually seeking out care with adult congenital cardiologists rather than adult cardiologists or pediatric cardiologists so that we can standardize care, and hopefully even further improve outcomes for these individuals. So the first board exam was actually offered in 2015, for this field. So it's a very new subspecialty that's been offered now every other year for the last six years. So 15, 17 and 19. And so there is a growing body of board-certified adult congenital cardiologists in the country, but it still needs many, many more providers in order to fill the care gap that exists.


Host: Awesome. So when did you begin the program here at Cook Children's?


Dr. Pilgrim: I would say that the program really began before I got here. And because there was a think tank moving forward with regards to how we were going to manage the number of individuals growing up with heart disease here in the pediatric facility at Cook Children's, obviously, when I got here, the program began in name, there was a fair amount of infrastructure and discussion with the administration and within our group about how that was going to take place. So I think to answer your question more pointedly 2014, February 2014.


Host: So how prevalent is congenital heart disease?


Dr. Pilgrim: That's an excellent question. Congenital heart disease is the most common birth defect in children. And it has been for some time. The incidence, as I stated before, has really not changed over the last 80 or 90 years. We think that that incidence is somewhere around eight in 1,000 live births. Just to make the math easy, we tend to quote a 1% incidence of congenital heart disease. And so that 1%, obviously, is made up of a whole spectrum of different types of disease. And when we break down the complexities, if you will, of congenital heart defects, it really breaks down into about 50% of all defects being simple, another 30 to 35% being moderate complexity, and then the very complex types of congenital heart disease account for about 10 to 15% of the remainder, but the incidence has remained the same but because again, of surgical outcomes and improved lifespan of these individuals, they're now becoming adults.


Host: So we know that around 10 to 15% of patients don't respond to traditional treatment or relapse from traditional treatment methods, can you discuss how candidacy is determined for CAR T-cell therapy recipients and why CAR T-cell therapy is a treatment option?


Dr. Pilgrim: CAR T-cell therapy is a treatment option for children with B-cell ALL who haven't responded the way that we've wanted to meaning that it's either refractory that it hasn't responded to the initial treatment or that it's relapse, meaning that it went away and then came back. So first of all, the patient has to meet that initial criteria of relapsed or refractory B-ALL and then they'll come to have a consultation with our cellular immunotherapy program, which includes me, and some other specialized doctors here at Cook Children's to help review the treatment and the process and determine that the patient and family are interested, and that this would be the best treatment for them.


Host: So studies show that there's been an increase in the number of adults diagnosed with congenital heart disease is this totally because of better diagnostics in children and adults, longer lifespans or people getting sicker as well?


Dr. Pilgrim: I think it's a combination of the three. Certainly we know that diagnostics have improved in pediatric cardiology, and our education in the pediatric population of making sure that individuals are aware that they need lifelong care has improved. That said, there is a fairly lengthy amount of time in our history, where kids who have undergone surgery for their congenital heart disease may not have been told or may not have known that they needed lifelong care. And so they're coming back into care later on, either because they heard about an adult congenital cardiologist, or in some circumstances they'd started to develop complications related to their congenital heart defect. So I think it's actually a combination of improved education in pediatrics, as well as adults who are coming back into care after being lost. I would also add that when we look at transitions, which we may talk about later, the transitions education program has certainly improved locally, our ability to make sure that these kids don't get lost to follow up. But in nationwide when we look at the attrition rate, if you will, of kids, there's a large number of kids over the age of 15, that fall out of care for various reasons. Some of it's because they moved, some of it's because they don't know, they may have limited resources, so on and so forth. But there is a fair attrition rates within the pediatric population that don't know that they're supposed to follow up in adulthood. And so they will also come back into care one way or another in adulthood?


Host: So what is the significance of having an adult congenital program at a children's hospital?


Dr. Pilgrim: Well, I think the significance is, is that we're successful at what we do. And I'm actually very proud of that, as a group, we've had very, very good success rates with our kids. It's a testament to the care that's been delivered by my partners and the surgery program here to even be able to talk about dealing with adults who have grown up with congenital heart disease, obviously, you have to start with kids, babies and young people to get to that age group. And so the significance of having an adult congenital program at Cook Children's is important, because I think it really establishes a footprint for the legacy that Cook Children's has created by doing congenital heart care for as many years as they've done it. And it also speaks to the Promise of Cook Children's that we did heart surgery as children, but we haven't neglected the fact that by virtue of us doing surgery as a child, now we have a growing population of adults with congenital heart disease that still need specialized care.


Host: So are there other children's hospitals that are doing this?


Dr. Pilgrim: There are. I would say that it's very variable as far as the individual institutions and how they tackle this problem, which again, speaks to the unique nature of the field. There are freestanding children's hospitals that do this, there are freestanding adult hospitals that do this, the majority of the time, it's a combination of the two where there are children's hospitals that may have been built within an adult hospital. And so there's lots of different ways to get the program taken care of it really is an institutional decision as far as how they're going to handle the care of adults.


Host: So is there going to be at some point in need to develop sort of a structure for care across the nation in terms of how this this care is?


Dr. Pilgrim: Oh, for sure. Yes. There are guidelines for the care of adults with congenital heart disease that came out in 2008. And then revised again through the American Heart Association in 2018, that outline basically the primary care of adults with congenital heart disease and various disease processes. That said, where it gets done is, again, institution specific, but as far as protocols, there is an accreditation process that we will be going through here at Cook Children's, hopefully over the next two to three years, where we will actually have an accredited program and recognized nationally as an ACHA accredited, adult congenital heart disease program. And part of that accreditation process is geared towards making sure that the necessary infrastructure is created and followed on a routine basis. Not to say that everybody is cookie cutter recipe based medicine, but it certainly is protocolized to where we know that this type of disease process should be handled at that institution.


Host: So an unexpected an important service is the connection between pregnancy and heart disease. How did that come about? What is its significance? And where do you see this going??


Dr. Pilgrim: So pregnancy and heart disease is an interesting field. If you think about, let's say, 30 years ago, the number of women that were of childbearing age that had heart problems, the majority of the time was related to rheumatic heart disease. So more valvular heart disease, mitral and aortic valve disease, and with the increase in the number of adults with congenital heart disease now growing up and becoming childbearing age, both men and women, the idea of having children obviously, is something that you would expect with a young adult, they'd want to raise their own family and live a normal life. That's the whole goal of taking care of these patients. Obviously, half the population that I take care of are female, and they're certainly birth control and pregnancy considerations that factor in. But now congenital heart disease has become the most common reason for women of childbearing age to have a heart problem, which again, speaks to that care gap and who is going to take care of the woman with congenital heart disease, number one, just at baseline, but now you're Adding the pregnancy physiology, which is a different entity in and of itself on top of their congenital heart disease physiology and the interplay there certainly adds some potential challenges. As far as where it's going. It's getting very busy around here with our pregnant ladies. We actually have a very closely networked affiliation with our obstetrics colleagues and our maternal fetal specialists. We've also triangulated that care with our fetal cardiology program to where moms with congenital heart disease are getting their fetuses screened properly, with good fetal echoes through our program here, and they are also getting specialized Maternal Fetal Medicine care with high risk specialists here at the local adult hospitals. We meet on a monthly basis, we're able to essentially come up with a plan of care for each individual woman based off of their specific heart lesion. What the plan of care as far as their pregnancy is, how the delivery might go, anesthetic considerations, pain management considerations, delivery options, whether it should be a normal, spontaneous delivery, or if this is something that needs to be a planned cesarean section, so on and so forth. So those are each considerations that we give to each woman that presents with regards to pregnancy.


Host: So when you see an expectant mom, or have a patient with a congenital issue, who's considering getting pregnant, what do you discuss with them in terms of their own health and that of the baby?


Dr. Pilgrim: So that usually is along conversation ideally done before they're pregnant, so that they're understanding the risks that they're getting themselves into. Obviously, the discussions are different with each woman primarily, and I'll add the dads with congenital heart disease, there is a discussion that goes on there as well. But primarily with the women with congenital heart disease, we discuss what their unique physiology might be, what that looks like. Certainly, we want to deal with any human dynamic issues, valvular dysfunction, ventricular dysfunction, rhythm issues, we want to deal with those things, the best we can and get that situation tidied up so that they are in the best physical condition they can be before they take on the challenges of a pregnancy. That usually goes into a pre-pregnancy visit, which is a comprehensive visit looking at their own heart, looking at their echocardiogram, the structure, their function, a rhythm assessment, sometimes even an exercise stress test to see whether or not they have the aerobic capacity to handle the nine month marathon of pregnancy. We also discuss with them the unique challenges of having children with congenital heart disease. So we mentioned the incidence before 1%, or roughly eight in a 1,000 people in the general population being born with congenital heart defects. We know that men and women both with congenital heart disease carry a much higher risk of having children with defects themselves. So for men, we usually quote them a risk somewhere between three and 5%, which is a three to five times increased risk above the general population, prompting fetal screening for women, that risk is even a little bit higher, some literature has shown 4% to possibly even as high as 10% risk of having a child with congenital heart defect, that risk is assumed with each pregnancy. So that's not just for the first or for the fourth, it's for every intended pregnancy moving forward. Obviously, fetal screening becomes important to make sure that we're not missing something in the fetus. But it also is a challenge as far as the mom is concerned, because in some of our patients, there are longevity issues, we know that some of these patients certainly with complex congenital heart disease may not be expected to have the same lifespan as a normal person. And so there's a large ethical consideration and a discussion that you have to go into with them as well about, you know, your heart is not normal. And you're bringing a new life into the world, and what that might look like and what strain you might be putting on their partner. And so it gets to be a fairly heavy conversation. It takes a lengthy amount of time. And sometimes it's over the course of two or three visits, that we have those discussions. When you add the underlying current of potential genetic defects on top of their congenital heart disease that makes the risk potentially even higher. So some of our patients who have specific genetic problems, chromosomal problems, if you will, on top of their congenital heart disease, the risk of having a baby with a defect is that much higher.


Host: So I'm glad you mentioned genetics. So what role do genetics play in congenital heart disease? And how does that affect the whole family?


Dr. Pilgrim: That is a moving target. Genetics and congenital heart disease has been a hot point of discussion among congenital cardiologists for a very long time. Unfortunately, we still don't know a lot about the genetics of specific lesions, we are learning more about specific genetic mutations that may predispose to certain types of heart disease, but unfortunately, we don't have pinpoint details as far as certain mutations that cause all of the defects that we handle. As mentioned just a minute ago, though, if we know that there is a specific genetic condition. For instance, some of our genetically inherited heart diseases like Long QT syndrome or hypertrophic cardiomyopathy, we do know the inheritance pattern of those types of diseases. And that certainly goes into the discussion as far as their risk of having a child those are both autosomal dominant conditions, which means that there's a 50% probability of having a child with that same genetic defect whether or not it manifests as actual disease will be determined as they grow. Because you can have the same defect in a father and a child and they may manifest differently with different severity levels. But genetics certainly does factor in tremendously. Unfortunately, we just don't know a lot about the specific genetic mutations with regard to isolated lesions.


Host: So when you have, say, a couple that comes in, and they bring their baby, and so you have you, that's the whole family, how do you sort of educate them? So let's say you realize that dad has had a heart issue, or maybe they weren't aware early on, and now they do, how do you educate them moving forward?


Dr. Pilgrim: With regards to the baby?


Host: With regards to the baby, or just in general, the family like does it like say a dad doesn't even know that he could pass that along. I think a lot of people aren't aware of that.


Dr. Pilgrim: Right? That is something that basically is an education issue, training the family to understand, I'm assuming you're talking about an adult with congenital heart disease and their risk of having a child with that same problem. So that goes back into making sure the patient is fully aware and educated with regards to their risks, and understanding what that may look like in the baby certain symptoms that may develop certain ways that babies behave with congenital heart disease, clinically what they may present with or if there's specific things the pediatrician may raise in a routine office visits that may be of concern. Those would all be indications to possibly even come in and visit with a pediatric cardiologist for the baby.


Host: In addition to educating families on their conditions, risks and long term outlooks, you've been responsible for developing the Transitions Education program, a truly unique and beneficial part of care for preteen and teen patients to help prepare them for transition to adult congenital care. Can you tell us a little about how it works and its benefits?


Dr. Pilgrim: Sure. So the Transitions Education program is something that's near and dear to me, because I take care of the adults with congenital heart disease. But I have found that really knowledge is power. And if the young person coming to see me for the first time already has an idea of what it is they're dealing with, they know what their heart lesion is, they know what the potential long term outcome might be, potential complications might be, it makes my job that much more rewarding, but it also makes it even a little bit easier. So the Transitions Education program has been created here at Cook Children's for our heart center, in an effort to make sure that our young people ages 12 to 18, are receiving a proper and uniform education about their own heart problem. If you will, when a baby is born with a heart defect, obviously, it's usually the parents that are dealing with the medical care of that infant. As the infant grows into a child, the child may or may not understand varying degrees and varying levels of complexity of their heart lesion. When they get to be an age of understanding in adolescence, let's say, they are given more and more autonomy in other parts of their life and making decisions about going to college, for instance, or whether or not they're going to be involved in a sport. But many times in health care that gets forgotten. And so as a pediatric cardiologist, I am often challenged with giving enough information to the parent to make parental decisions, but also making sure that the adolescent is receiving enough information so that they're aware that when they become an adult, it's going to be their responsibility to make those decisions as well. So in getting back to the Transitions Education program, that is an education program that we've created here at Cook Children's that is specifically designed to introducing them to their anatomy initially, first of all, to give parents the understanding that we've thought about it. With many parents, they get nervous when their kid starts to get into those teenage years, like what's going to happen to my child when he becomes an adult? Who's going to care for him? Certainly with our more complex heart problems, there's some anxiety about transitioning them into an adult medical arena, perhaps with physicians that may or may not understand what they have. And so first of all, just letting them know that we've thought about this as a program, and that over the course of the five or six years of them transitioning into adulthood that we will introduce them to their anatomy, their physiology, potential complications, these are the names of your medicines, these are the doses, this is why you take them, these are the potential complications of these medicines, potential side effects. This is who to call in the case of an emergency. If you're not feeling well, what ER do you go to? You have other medical conditions, this is how those other medical conditions might interplay with your congenital heart disease. Certainly, we've talked before about birth control and pregnancy. Those are all reproductive health issues in both men and women, the likelihood of having a child with congenital heart defect. These are all parts of the Transitions Education program, in order to have that well rounded individual graduating from our pediatric program and ready to face the medical arena on the adult side, namely with me in the adult congenital heart disease program, but potentially even with their primary care provider. And it gives them a sense of empowerment. Let's say they end up in a urgent care center or in an emergency room somewhere where they may not have have adult congenital services they know to say, my heart lesion is XYZ. My doctor's name is let's say, Dr. Pilgrim. Here's his number, if you have concerns about my heart, or how my heart might interplay with what I'm here with, you can call him. And that happens routinely where I'm getting phone calls from all over the place for these patients, so that we can have discussions with providers about the appropriate way to manage whatever they're presenting with. So it really gives the patient a sense of empowerment, when they reach adulthood to be their own advocate, and to know what to look for, to know what to ask for and to know when they don't know. And to call me or the group to answer those questions that they may not know. Again, knowledge is power. That's my mantra for a lot of this for transitions education, that the more you know, the more likely it is you're going to handle it properly, or at least get it into the hands of somebody who can.


Host: So with all the different medical and support needs of your patients, can you talk a little about the key disciplines that may be involved in what a patient team looks like?


Dr. Pilgrim: Sure. So for adult congenital heart disease, it looks very much like what they did as children, they come in and they see the cardiologist, they get some of the same testing with EKGs and echocardiograms. They may or may not undergo exercise stress testing. So a lot of the testing from a cardiovascular care standpoint is very much the same as what they're used to growing up. When you're talking about the overall team though, we're looking at some of our patients who may have long term gi issues or liver damage or lung disease. And so then you're adding in adult providers in those sub specialties that may not be familiar with congenital heart disease, but can speak to their individual organ system, and the effects that that physiology may have on it. And so we are in process of developing a lot of networking with adult physicians and subspecialists in the region to be able to care for these individuals from a neurologic standpoint, let's say, in some of our patients with developmental delay, making sure they're getting appropriate services. Having a social worker, which we're very blessed to have here that can help with legal guardianship issues from full blown, Mom and Dad make all the decisions to maybe an assisted decision making capacity for individuals who may be able to make some of their decisions but need assistance in others. So the feeding programs, for instance, some of our adults still grow up and they may still be on feeding tubes or gastrostomies. So we have nutritionists that are involved there. Physical therapy, occupational therapy, certainly noncardiac surgery is a big issue, dental care. Those are all things that you may not think about straight up as far as caring for somebody with a heart lesion. But those are all questions that we get almost every day, Am I safe to have this procedure performed as an outpatient? I've even had cosmetic surgeons call me about some of my patients, whether or not certain types of procedures can be performed in an office setting. And so it really runs the gamut as far as the different ways that these patients come to care and how we interact with the adult medical arena. But as far as our comprehensive care here, all of their congenital heart disease issues, save for some specific complex pulmonary hypertensives, and adult heart failure therapy, so namely transplant, all the rest of it is dealt with here at the Heart Center here at Cook Children's.


Host: So for a primary care physician, particularly, what do they need to be aware of, and when do they need to know to refer a patient or to work with their adult congenital cardiologists?


Dr. Pilgrim: Well, my hope would be that by the time they're an adult with congenital heart disease, that the primary care provider is already aware that they have a congenital heart defect. So that would be my ideal to where they're not having to make the decision to refer. Now if it's a new diagnosis, which happens, then to look for specific symptoms that may be a tip off a new murmur on exam symptoms of passing out or rhythm problems or something along those lines that would bring them to care. Usually, those patients if they're already in adulthood, the usual track for that would be through an adult cardiologists office where then the adult cardiologists would see them and then recognize that this is perhaps a congenital issue, and then it would come to me or through the program for further care. But from a primary care standpoint, my hope really would be that we're educating our patients enough in pediatrics. So the primary pediatricians are obviously getting notes and updates from us routinely throughout their childhood, but by the time they are transitioning to an adult provider, that they are already in hand, given a summary of care that this is what we have done to your heart, and so that they're aware and know how to contact us.


Host: And so for a primary care physician who may have a patient that maybe it's a new patient, and they come to them and they know they have a heart issue already or they suspect. At what point do they talk to them about not just going to a cardiology program, but to transition to let them know or let them know that they'll be there to work with them through that.


Dr. Pilgrim: So the, the awareness of the adult congenital heart disease program, I think, is perhaps the most important thing that we exist, that we're there to handle this care gap of these individuals who may fall in the cracks between pediatric and adult care. And while they may not completely understand, I hope they do after this conversation, that we exist and that we're able to take care of these patients, at least to know that this is something that requires a specialist, and then usually it follows the normal pattern, they would end up with a congenital heart disease specialist. And those items can be ironed out.


Host: Great. And similar, then for ob/gyns out there that may have a patient with a heart condition or suspect a fetal heart problem, when should they refer the patient to an adult congenital program? And what's the advantage?


Dr. Pilgrim: In my opinion, the earlier the better. Like we spoke about previously, my ideal scenario would be that I see the young lady before she's pregnant. So we can go through the full pre pregnancy assessment, actually understand risk profiles, and so on and so forth. Obviously, if they're already pregnant, the physiology of pregnancy changes as you get deeper into a pregnancy, you know, blood levels change, and there's hemodynamics that change throughout the pregnancy. And so the earlier I get my hands on a patient who may be pregnant with congenital heart disease, the more likely it is that we can diagnose problems, we can come up with a scheduled delivery plan, speak to the the high risk doctors, make sure that their baby gets proper screening, and be prepared for that aspect of their care as well. And really dictate whether or not they are safe to deliver in their outside wherever they were planning to deliver. Or if they need to be delivered more locally here for advanced care during and after delivery.


Host: Let's say that the fetus has a heart condition, mom has never known about having one does she need to be checked just in case or ...


Dr. Pilgrim: Usually no. So again, with the incidents being 1%, and that doesn't sound like a lot. But when you do the math on that there's a fair number of babies being born with heart problems, the majority of the time mom doesn't have a problem. I have had several instances since I've been here where moms have actually been diagnosed with a congenital heart problem during a pregnancy. That's where it actually manifested symptomatically. And then we've had to manage mom with her newly diagnosed congenital heart problem, screen baby for said heart problem, and manage them both and then tackle mom's problem when she's no longer pregnant, either surgically or interventionally, or perhaps medically, if necessary.


Host: So after everything that we've talked about, where do you see the future of adult congenital medicine going particularly within the realm of pediatrics overall, and especially here at Cook Children's.


Dr. Pilgrim: So I think the future of adult congenital heart disease is bright. It is a growing field that is now recognized as a separate subspecialty within cardiology, both from the pediatric and adult cardiology realms. The number of kids growing up with heart problems is not going away, that's going to continue to grow as our surgical techniques and the types of care and the amount of care these kids are getting further improve from where we already are, is just going to further increase the number of adults with congenital heart disease. And I think that the future is very bright with regard to the overall nationwide aspect of adult congenital heart care and the networking that's currently going on, but also locally here, where we are now establishing a very firm footprint in the community in Fort Worth, and the surrounding Metroplex for care with adults with congenital heart disease.


Host: Dr. Pilgrim, thank you so much for taking time out of what we know is an extremely busy schedule to talk with us today and to share the work that you do here in the adult congenital program.


Dr. Pilgrim:Thank you so much. It's my honor.

Host: We're so glad you could join us today. If you'd like to learn more about this program or any program at children's please visit us at Cook Children’s dot org.