I want to introduce our first speaker, Jamie Harris. Jamie's a pediatric nurse practitioner at Boston Children's Hospital. She's primarily with the EP team, but also sees general cardiology patients and cross-cover inpatient cardiac surgery. She will be presenting on a primer on congenital heart disease lesions. Thanks, Nate. Thanks, Brynn, for putting this session together and to HeartRhythm for having me. I have no disclosures. Okay. So, this is obviously a tough talk to give in 12 minutes, congenital heart disease in 12 minutes, but my goal is actually to tee you up for our next speaker. So, Melissa's going to talk about some of the arrhythmia implications of these lesions, and I am going to give you the anatomy that underlies that talk. So, our objectives for today are to describe three congenital heart disease lesions with a significant arrhythmia burden in children and adults, to get the basic anatomy of those lesions, and to identify from sort of a big global picture our two pathways for single ventricle palliation, which would be much too complicated to go into in a lot of detail today. Mostly, I want you to walk away understanding the impact of both the lesions themselves and the surgical repairs that we do on arrhythmia for these patients. This is our roadmap, so you know where we're going. And this is my background slide, or the why should I care slide, as I like to call it. So, congenital heart disease affects 1% of all births in the U.S. So, even if you're not working in this space, you probably know somebody affected by congenital heart disease. And one in four babies with congenital heart disease has a critical congenital heart defect, meaning they need life-saving surgery in the first year of life, or they will not survive. And from a global perspective, 90% of babies born with critical congenital heart disease will not have access to that life-saving care. So, we're incredibly privileged in the United States that 81% of those children and 93% of the ones who survive the first year will live to be adults. In the U.S., the number of adults living with congenital heart disease now exceeds the number of children living with congenital heart disease, both due to the surgical repairs getting better and also due to prenatal care getting better and the number of live births going down. Fewer than 10% of the adults who have congenital heart disease, though, will actually have access to a specialty center. So, adult congenital cardiology is absolutely a specialty, but you may not have that in your area. And so, in the years and decades to come, I expect that you will be seeing these patients if you haven't already, turning up in your ER. My patients like to have AFib on vacation, so maybe you've met them already, whether this is your practice area or not. We have some common themes that will keep coming up in the slides. So, we love to say this in congenital cardiology, no flow, no grow. So, the classic example would be mitral atresia. There is no flow into that left ventricle. It becomes hypoplastic and so does the aortic arch. Everything sort of downstream is undernourished in utero and doesn't grow. Similar to our adults with cardiomyopathy, if there's stretch and strain or ventricular dysfunction, we can expect to see arrhythmia. And importantly, for our congenital patients, scar promotes arrhythmia. So, sometimes we've actually done this to them with the repairs that we do in the operating room. So, we have to keep that in mind. What are the implications, not only of the lesion, but of the repairs that they've had? And understanding the different repairs through history and what your patient actually has for physiology now. So, the first lesion we're going to talk about definitely has significant impacts, a lot of arrhythmia burden, and that's Tetralogy of Fallot. So, classically, this is described as four defects, but it's really the result of one, all of the subsequent results of one defect, which is the anterior deviation of the conal septum. So, you end up with subpulmonary stenosis, a malalignment VSD with an overriding aorta, and then right ventricular hypertrophy. And incidentally, 25% of these patients will have a right aortic arch. Many of these patients are also syndromic, commonly have DeGeorge syndrome. And then down here, just a footnote for the sake of time, there are a lot of variations on a theme with all of these lesions, but with Tetralogy of Fallot, you can also have pulmonary atresia, which is palliated differently. It's more of a central single ventricle pathway, and you can have an absent pulmonary valve. So, the repairs that we commonly see for sort of traditional Tetralogy of Fallot would be the old style transannular patch. So, these are some of your older patients that you'll see this in with patch augmentation of the main pulmonary artery, closure of the VSD, and separation of the aorta and the pulmonary artery so that you have a biventricular circulation. You have no cyanosis, but what you do not have is a pulmonary valve. So, these patients will have free PR and almost categorically eventually require pulmonary valve replacement. We typically do that transcatheter now, and the arrhythmia burden from that dilated right ventricle is quite high. So, they often have VT that we will try to ablate beforehand. And the next lesion that we are going to talk about is Ebstein anomaly, which is the downward displacement of the tricuspid valve. So, if the valve is seated lower and it does not co-act well. And so, these patients have what we call atrialization of the right ventricle, so enlarged right atrium and then tricuspid regurgitation, which results in dilation of the right atrium and the right ventricle. And they should, you know, based on that physiology, they will have a right ventricular conduction delay. So, my mentor is Ed Walsh, and he always says if you see a patient with Ebstein who does not have a right bundle branch block, you should be suspicious that they actually have WPW. And there is a strong association with WPW and other accessory pathways. Historically, the only thing really in our toolbox for Ebstein was a tricuspid valve replacement, which is not a great procedure for children to go through. Or a single ventricle type palliation where they would actually patch over the tricuspid valve, functionally creating tricuspid atresia and then palliate them down that pathway. That's called the Starnes procedure. It's not done very often now unless the neonate is really sick and they don't have access to other procedures like the cone. But at most large volume centers, we are going to try to get patients to a cone procedure. So this is using the patient's native tricuspid leaflets. They are mobilized and then reshaped into a cone-length valve that co-ops more appropriately and can be seated more appropriately in where the annulus should be. And if there's a lot of atrial-lateral ventricle, that will be placated as well. There's also a large arrhythmia burden, both pre- and post-op for these patients. And so we look often with a pre-op EP study to see if we can get rid of some of those arrhythmias, both to make their post-op course better, but also because the last thing you want to do once you've made this tricuspid valve perfect is put a catheter across it. It's complicated by atrial arrhythmias and AV block. And ostensibly, you know, you could have worsening of the tricuspid valve function and RV function. But as you can see on the right, it is better than TBR for these patients in terms of RV function. And here's just a picture of that, kind of rotating that new tricuspid valve into the proper annulus. So transposition of the great arteries. This is not one lesion. This is many, many lesions, especially when you start to involve the patients with heterotaxy and, you know, complex congenital heart disease, including transposition. But just to simplify things, you know, D-transposition is a cyanotic lesion. And L-transposition is often called congenitally corrected. So those patients do have a, you know, appropriate systemic circulation and appropriate pulmonary circulation that is not cyanotic. But they will eventually get RV dysfunction from having a systemic RV. So they're often picked up actually later in life. And they may appear to you that way. But for today, we'll focus on D-transposition. So if you're into segmental anatomy, which I wish we could cover today, but we can't, this is your SDD. So your atria and your ventricles are in the expected positions. Your aorta and your pulmonary artery are essentially switched. The aorta is rightward and anterior, overlies the RV, often a VSD, which is good. They need that mixing so that they're not entirely cyanotic. And there may also be pulmonary stenosis. Traditionally, so in your older patients, middle age and above, this was repaired with a mustard or setting procedure. This is the same procedure with two different materials. The systemic venous return is baffled. So your vena cava are baffled across to the left atrium so that now you have a sub-pulmonary LV pumping to the lungs and a systemic RV pumping to the body. And it works. It's a great way to make these patients not cyanotic. But over time, that RV was not meant to be a systemic ventricle. And so they all have some degree of systemic RV dysfunction. There's problems with baffle obstruction, baffle leaks. And because we've done so much extensive work in the atria, they're very prone to sinus node dysfunction and atrial tachycardiasis. So the modern procedure, more modern procedure for this is the arterial switch, where the aorta and the pulmonary artery are sort of severed above the semilunar valves and transposed. And so now we have more of a traditional anatomy for these patients, where the correct arteries are attached to the correct ventricles. But the problem is that the semilunar valves are now switched. So a pulmonary valve is now a neo-aorta and was not meant to serve that role. So they do have long-term aortic insufficiency as a major complication, although that's getting better over time. AV block is a huge concern because you're right in that area with the operation. And then if you have to translocate the coronaries, the concern is for both short-term and long-term coronary ischemia. So our single ventricle lesions, there are a lot of different permutations. But classically, I think we talk most about hypoplastic left heart syndrome. That's the only one I ever saw in nursing school. And it's pictured here. We'll kind of use that as our example, just to show you the two different pathways. So you can see there's a no flow, no grow situation here. That left ventricle is underdeveloped. We can assume that that patient probably has mitral stenosis. There's some flow going through there, but not a lot. And then the aorta is subsequently underdeveloped. This was universally fatal until the Fontan. So this is a staged palliation. There are three surgeries. And those vary a little bit center to center. But the ultimate culmination is the Fontan. So you have a single systemic outflow tract. And pulmonary blood flow is passive. So that is a marked improvement on the, you know, non-survival of these patients. But there are long-term complications that we're trying to figure out now. So for adults with Fontans, we see hepatic congestion leading to Fontan-associated liver disease. And that can actually turn into liver cancer. We see a lot of lymphatic complications, like protein-losing enteropathy and plastic bronchitis. Formation of collaterals, which then become desaturated. And then, again, we are kind of operating in the area of the sinus node. And so sinus node dysfunction, to some extent, is expected. For our classic style Fontans, they have a very large right atrium. And we have a lot of atrial tachycardia. And then we still have one ventricle. So their functional capacity is almost never completely normal. So enter the bimetricular repair. We've been doing this now for quite a few years. And there are many different variations. It's very individual, based on the patient's anatomy. But, again, requires a staged recruitment prior. So for some patients with single ventricle disease, you can recruit the hypoplastic ventricle through a series of procedures that essentially ask it to work harder. And then it may or may not be up for the task of formal bimetricular circulation. So what's shown here is the reverse double switch. And my brain likes to think of this, that we are taking someone with hypoplastic left heart and giving them transposition physiology so we can then do a neutral switch. So it's old techniques applied to new problems. That's what Dr. Amani says, who does this procedure. Again, not everyone's a candidate. But it's just good to know this is coming down the pike. And the outcomes show us that patients with a bimetricular conversion appropriately selected patients for bimetricular conversion have better outcomes in terms of liver disease, PLE, plastic bronchitis, and arrhythmia. So I did it in 12 minutes, which was my goal. I really can't believe it. But we got through all these things. And hopefully we met our objectives. I think we're going to take questions at the end. But I'm also just going to put my QR code up there if someone wants to grab my contact information. Thanks. Thank you so much. We were so excited to start this session that we forgot to do a little housekeeping. And so the QR code, not this one, this one, is where you can scan to submit your questions if you want to do Q&A there and not come up to a microphone later. As Jamie said, we're going to do questions at the end. And so we will launch into our next one. We have Melissa Olin, who is an EP nurse practitioner at Nicholas Children's Hospital in Miami, Florida. And she's also the director of the South Florida Project Adam. And so her talk today is titled Common Arrhythmia Substrates in Congenital Heart Disease. Good afternoon, everyone. Thank you. Thank you to the organizing committee for the invitation to be here. Thank you to all of you guys for choosing this session and hanging around for the afternoon like this. I have no disclosures. The objectives for today is to discuss common substrates present in repaired and unrepaired congenital heart disease that increase the risk for arrhythmias. We will talk about review, we'll review the known surgical repairs that increase the risk for acquiring various arrhythmia substrates. And lastly, what are other contributing factors to consider that can increase the burden of arrhythmias in congenital heart disease? Now, thank you, Jamie, for the deep review of anatomy and setting the stage for me to talk about arrhythmias. I think that arrhythmias in congenital heart disease can be so multifactorial as to why that arrhythmia in that lesion in that patient. And so, for that reason, I'd like to honor this great poet that said to take a complicated skill and make it simple. And so, I have broken things down into categories, therefore consider the following. You can have arrhythmias from the congenital intrinsic conduction system itself. You can have arrhythmias from residual or postoperative sequelae, from abnormal hemodynamics, from hypoxic tissue injury, primary myocardial disease, genetic influences, and then, of course, they can be largely surgeon-specific, the way that their technique is, the way that they suture, the way that however long they have them on bypass. All of those things can contribute. They're also all going to be very highly variable. And so, therefore, we will focus on the bulk of our business, which are these first three categories. Now, let's first start with recognizing that there are some congenital lesions that come with some predictable arrhythmias substrates associated with them. So, pre-surgically, we can have some issues where the sinus node was formed. We can have issues with the way that the AV node was formed, displaced, abnormal function, twin AV nodes, things like that. We can also have congenital lesions that have a higher incidence of accessory pathways associated with this. All of this to say that having this, you know, knowing these lesions and having this awareness of these in the pre-surgical state can really help guide your management, maybe perhaps the need for an elective diagnostic or interventional EP study in the pre-surgical patient. And I think perhaps the greatest example of this, although not very common, is our heterotaxy family. This can be broadly broken down to patients with right isomerism, your asplenics, and left isomerism, your polysplenics. And maybe perhaps the greatest example would be the most severe kind where you're having twin sinus nodes with twin AV nodes. And seen here in this one continuous rhythm strip, you have a patient going from one conduction system to the complete other conduction system with right isomerism. And although it may seem nice to have a backup conduction system, those twin AV nodes can actually serve as a source of SVT as shown here. So this is yet another example of something that you may want to handle in the lab prior to that extracardiac Fontan for the obvious reasons. Now keeping with the same theme, with congenital lesions and the associated substrates associated with that, in Epstein's anomaly, the most common for having the presence of accessory pathways as high as 30%, most commonly located in the posterior and posterior septal areas of the tricuspid valve. And it's not uncommon in this lesion to have multiple accessory pathways associated with it. Now yes, they do have other arrhythmias like focal atrial tach, ventricular arrhythmias, your atrial tachycardias, and those rhythms that can degenerate to sudden cardiac arrest and sudden cardiac death. But those would be far more common subsequent to the postoperative sequelae or abnormal hemodynamics. Now let's pivot to postoperative arrhythmias substrates. And we know that with all surgical repairs, as Jamie well said, come with the surgical palliation comes with the consequences of all of these effects. And it's, you know, I mean sometimes it's a combination of these things, whatever, but longstanding AV valve regurgitation, the effects of scar, all of those sort of things that will set up for some effect of abnormal endocardial tissue, areas of slow conduction, that all end up being a substrate and a risk for arrhythmias. So by the time these patients with these lesions are reaching adulthood, about 50% of them are going to have documented episodes of intraatrial reentry tachycardia. And then of course ventricular arrhythmias and sudden death are not excluded from this. So let's talk about our transposition family. As well articulated from Jamie in going through all of the anatomy, I do think that it is important to reinforce what is meant when we say a systemic RV. That is defined as the morphological right ventricle that is supporting systemic circulation. And when you add that with the other things like abnormal congenital intrinsic conduction system, the effects of postoperative sequelae, all of these patients are going to have some element and effect of systolic RV dysfunction. And we think that it is that of why this lesion specifically has the highest risk for sudden cardiac death associated with it. And on autopsy, over 80% of these sudden deaths were during exercise, likely due to the increase in myocardial oxygen demand in this systemic RV. So what specifically are these arrhythmia substrates for sudden cardiac death? Well let's first start out with what these guys have in common, besides their name and the systemic RV. Both of them, ventricular arrhythmias, would obviously be a high risk for sudden death. The atrial switch population, fast atrial tachycardias are also going to be a risk factor for them. And then specifically going back to the early part of this talk, where we're talking about lesions in the presurgical state, that they can have a higher incidence of, we know that congenitally corrected transposition has a risk of having AV block associated with it, just from its anatomy itself. And then of course, the effects of this, of acquired pacemaker ventricular dyssynchrony in this congenital lesion, puts these groups at much higher risk. So let's talk about our Fontan patients. This is a horizontal bar graph of the distribution of arrhythmia burden types. And by far, your atrial arrhythmias are the most common. We do know that the extracardiac Fontan has decreased the incidence of the atrial tachycardias, but certainly not eliminated it. And that is primarily the consequence of the longstanding effects of elevated right atrial pressures and the effects of scar in this right atrium, of why these are common lesions that we are palliating in the EP lab with CTI flutter ablations and things like that. Similar to your Fontan patients is the same effects that the Tetralogy patients are going to get with longstanding elevated RV pressures now. In addition to the effects of scars seen in them as well, where it's not uncommon for atrial macroreentrant, focal atrial tachycardia, and your typical flutters to be seen in this patient population. In addition to atrial arrhythmias, monomorphic ventricular tachycardia remains the dominant lethal arrhythmia in Tetralogy of Fallot. And this has really been well defined because of the frequency of EP studies that we're doing in order to risk stratify this patient population that has really enriched our understanding of the areas of anatomical regions of slow conduction, owning to really how narrow they are. And this is primarily in isthmus 3, which is located behind your VSD patch in the pulmonary annulus. And so, you know, I think that we do a really good job with having on our radar when we're seeing our adult Tetralogy patients, their risk level and all of those kind of things. And this slide was really to just not let our guard down because it affects our young patients too. So this was a documented 11-year-old with a fast cycle length VT with a right bundle branch block morphology and a very healthy looking baseline EKG. Here's an example of a 21-year-old with a fast cycle length also in a left bundle branch block morphology with, again, a healthy looking EKG. And then this one, a lot more concerning looking, presented with sudden cardiac arrest and polymorphic VT. All of these patients with very different presentations and VT morphologies brought to the lab and identified to be slow conducting anatomical isthmus 3. So I think at this point we have a lot of things to help guide and help predict. But sometimes you don't truly know until you have your intracardiac mapping to identify these areas and the risk factors. And so this VT is a real problem for this patient population. And don't let your guard down even in the young. So at this point, I think that I've hit on all of the major categories that can serve as arrhythmia substrates in congenital heart disease. In addition to these categories, things contributing factors to consider are your proarrhythmic medication, electrolyte imbalance, systemic illness and inflammation, cyanosis, low birth weight, and obesity. In summary, arrhythmia substrates in congenital heart disease are common and multifactorial. Arrhythmia substrates often vary in presentation even in the same anatomical lesion or surgical repair. And arrhythmias in congenital heart disease are multifactorial and more than likely a consequence from the congenital intrinsic conduction system, residual or postoperative sequelae, abnormal hemodynamics, and or a combination of all. I thank you for your time. Thank you, Melissa. I'd like to introduce our next speaker, Jacqueline Ryder. She's an EP nurse practitioner at Leary Children's Hospital in Chicago. She focuses on outpatient care and device and arrhythmia patients. She has a focus and passion in sudden cardiac death and prevention and is the program coordinator for Project ADAM. She will be presenting on Bridging the Gap Between Pediatric and Adult EP, Best Practices for Transition to Adulthood. Thank you. Perfect. And thank you to the Heart Rhythm Committee for allowing me this opportunity to talk to you all about best practices on transitioning our pediatric patients into adulthood. So for this talk, we'll go through the current status of the population. We'll highlight the difference between the words transfer and transition. We'll discuss some barriers and risks during this period of time, the guidelines and program structures that are available for you to implement into your clinical practice, also some billing opportunities and some other considerations. In patients with congenital heart disease, those of the transition age are estimated to be about 20% of that population. Lapses in care occurs about 30% to 60% of the time for CHD patients, and this time normally happens around the transition period. We know that lapses in care increases emergency room use and also associated with poor outcomes, and this is why a patient should go through a formal transition process when graduating to an adult provider. The goal of a health care transition is to optimize and maximize lifelong functioning through a developmentally appropriate health care system that empowers the patients as well as their caregivers. This process starts normally around mid-teens, mid-20s, but should be patient-dependent. It should be individualized based on the patient's maturity level, their ability, psychosocial status, and their stability of their disease. I want to take a moment to talk about the difference between the words transfer and transition. Transfer is moving something, moving from A to B, versus the word transition. It's the process. How do you get from point A to point B? In 2021, there's a systematic review that showed that the United States had the highest proportion of discontinuation of care at 43%, compared to some of our European colleagues, like in Sweden, at only 6.5%. We know that transition programs can help reduce gaps in care compared to usual care. Although the evidence on health care transition programs remain limited, there's been several evaluation studies that looks at the benefits of these programs. So I wanted to touch on the benefits of establishing a health care transition program in your clinic. We know that it improves health care-related quality of life, improves a patient's knowledge about their disease process, increases their transition-ready skills, and hopefully continues care. We also know there's adverse effects with lacking a program as well. There's patient dissatisfaction, medication complications, problems with treatments and adherence to their medications, higher emergency room use, which we know is associated with higher costs of care. And what we're hoping to avoid is discontinuation of care. There's no universal consensus on how transition care should be delivered and how the transition should be organized. But there's also slight variation in the measurements of the standards for health care transition program metrics. But I want to touch on the four overarching metrics through the different papers we'll go through in a moment. First the goal is to improve the patient's knowledge about their heart disease, two, to increase their self-management and advocacy skills, three, to learn how to navigate complex medical systems, and finally, that coordination and integration into adult-centered care for both their primary care as well as their specialty care, like EP. In 2020-2021, there is a survey, the National Survey of Children's Health surveyed parents across the nation to see what percent of youth were meeting health care performance metrics, as in the slide we talked previously. And we found that youth between the ages of 12 to 17 years old that had special health care needs, only 16% of those children were actually meeting those metrics. And sadly, these estimates are actually lower than prior studies that were done. This just shows that we're not transitioning our patients well. We know that our teens with congenital heart disease are at increased risk of developing neurodevelopmental deficits. And transition experiences and outcomes are known to be poor in youth and young adults that have neurodevelopmental disorders. Adults with congenital heart disease have higher rates of unemployment, particularly among the black individuals. And patients' transition goals should reflect their unique abilities, strengths, and weaknesses. And the planning for decision-making support with either guardianship or custodialship should be best started around the age of 17. And if it's needed, make sure it's documented in electronic medical record for the transition. Additional factors play an impact in the ability to transition. They're listed here. I think it's important to point out that not only lack of insurance can be a barrier, but having insurance doesn't actually mean having access to the type of care that you need. One study estimates that approximately half of the US population lives over an hour from a reputable adult congenital heart disease center. And those that are uninsured, Hispanic, or lower socioeconomic status are most likely to be affected. I think it's important to note the lost opportunity cost that impacts the family. Missing a day of work, the cost it takes to hire child care for the siblings, parking and transportation, those all could be prohibitive in accessing the care. And finally, I think it's important to note as well, too, from a provider standpoint, we have been taking care of these patients on the pediatric side for years. We have built a relationship. We've seen them grow. It is difficult for us to let them go into the adult world. And the flip side as well, too, it's hard for the families who have built this trusting relationship to also say goodbye. So I'm going to briefly summarize a few structures and transition programs that you can apply to your practice, including the American Academy of Pediatrics, six core elements, the AHA's key transition curriculum components for adolescents with congenital heart disease and the European Society of Cardiology. So in 2020, the AAP created a transition program that's structured among these six core elements as noted in the yellow at the bottom. The transition process begins with building a transition policy. Second step is then tracking and monitoring of this transition, assessing transition readiness, the transition planning. Step five is the actual transfer. And then step six is the completion and ongoing care with the adult clinician. The timeline recommends starting around the age of 12, as you can see, and goes up to the age of 26 years old. And the green boxes are the key topics and tasks for the health care providers to be focusing on through that timeline. There's resources that are available, including templates to be able to build your own transition policy. There's also validated experience tools for teenagers, as well as for the caregivers. And all these resources are available for free on the GAP transition website. In 2022, the American Heart Association updated their statement on the transition to adult care with congenital heart disease. And within this statement, there is this image that shows the impact of social determinants of health and how it plays into the transition. And as we discussed previously, environmental, health care, community, education, and economic stability all play a role in the transition process. There's a large focus on having a family-centered approach, because we know that it doesn't just impact just the patient, it also impacts the entire family. And within this guideline or statements, there's also resources on a transition curriculum for providers, as well as curriculum task lists that you could then give your patients as well. And then finally, the European Society of Cardiology is based off of three phases, pre-transition, a transition, and then post-transition, that spans across the ages of about 12 to 24 years old. And their statement recognizes that care is provided across the world. And there is multiple different resources available, depending on where the cardiac care is provided. And these should be applicable to all places around the world. There's also a focus on patient empowerment, wanting to increase autonomy, patient participation, their psychosocial skills, because we know that increasing patient empowerment actually improves quality of life, well-being, and also clinical outcomes. And there's also a focus on doing assessments, to assess what needs the family or that patient needs during the transition period. For those with nursing backgrounds, if you remember the HEDS assessment, so looking at the home, education, activity, diet, drugs, depression, sex, and safety, and all those factors as we discuss those impact the social determinants in health in the transition period. They also recommend connecting patients with peers. Around the age of 16 years old, they recommend connecting someone who's gone through the process or is also going through the process to have that peer support. In addition, it's also recommended to have an active referral. So instead of just giving the family or your patient, this is the number to call to schedule and make your new appointment with the adult provider, interact with that provider. Have a conversation with them. Make sure they understand and have all the data that they need to understand the patient's history leading up to this visit. And finally, it's recommended that the pediatric center actually helps with the follow-up a little bit as well, too. So they recommend, after that initial visit, following back around with the patient family, making sure they have their next visit scheduled. Do they know that when the next visit should be scheduled? And make sure there hasn't been any other questions that may have arose. And it takes a village. And as you can see here, there's a list of key team members to consider including in your transition program. Also consider including culturally similar peers, community health workers, their school or community centers they might be involved with as well, too. And I think it's important to note that there's no consensus on the minimum training or education requirements on who's best lead to families through this process. But APPs are trained to perform health interviews. We address psychosocial concerns. We offer education. And we have the organizational skills that are essential in this role. And we know that maximizing billing and productivity in this world, in today's health care system, is very important. Currently, there's no billable CPT code specifically for pediatric to adult transition. But the Gap Transition website has a recommended list of codes that can be used, including like extended time coding for your clinic visits. So during routine visits, you could add this education in there to start that transition process. If your organization has a social worker, consider bringing them into your transition program. They could do billable health-focused clinical interviews. They can assess behavioral. They could assess their understanding of their disease process and motivation and adherence to the medical therapy. And finally, you could also work with your hospital to develop a CPT Category 2 code. These codes are not billable codes. But what they do is it's a way to measure and track the use of the transition services. So it helps to show productivity and use of a transition program, which is definitely important. So finally, some other considerations. The guidelines, most of them recommend transition starting with their primary care provider. So going from a pediatrician to an internal medicine physician first. And then that person can also help them with connecting them with more adult-based resources. The patient's health should be stable. I don't recommend transitioning your patient, unless it's needed during an acute lead problem or their device needs replacement. It's best to wait until they're in a good place. And finally for our EP patients, don't forget about the remote transmissions. So coordinating with the new team who's going to be taking them on, I think it's important to figure out how that handoff is going to be because they have to request them. And then the patient also should know when that change happens and who's going to be following them. So in conclusion, 20% of our population is at the age of beginning transition process. There's resources available to build programs and validate assessment tools. And if you don't have a program, just start by finding those patients that may need the help. There's healthcare, there's services, these could be, sorry, these services could be billable opportunities for APPs while improving healthcare quality of life for our patients. So thank you. There's my email and there's also the QR codes to be able to get access to those, those papers we discussed. Thank you. Thank you. Sorry, I was just really interested in that. So our last speaker here is Bryn Decker Crooks. She is from the University of Michigan Medicine as a pediatric nurse practitioner in electrophysiology. She is also the manager of the advanced practice team for the Heart Center. And she will be talking to us on optimal device selection and programming of CIEDs in congenital heart disease. Great. Thank you everyone for sticking around today. I know it's been probably a long day for most of you and I'm so happy to be here with all of my wonderful PEDS EP friends up here. Just like Lisa said, we're going to talk about optimal programming of CIEDs in congenital heart disease. I'll start with a few objectives. We'll first talk about device selection for patients with congenital heart disease. Then we'll move on to optimal programming for devices in patients with congenital heart disease. And then lastly, we'll touch on future directions for pediatric devices. For those who may not know, in 2021 the Expert Consensus for Indication and Management of CIEDs in Pediatric Patients was released. It's a very helpful resource. So I recommend if you don't know about this white paper to review it and know it if you work with pediatric or congenital heart disease patients. Jackie, you'll recognize this young patient to the right. So when we first think about patients with congenital heart disease, we need to think about how to select the proper device for them. And there are some things that we need to think about. So one is the size of the device versus the size of the patient, what their diagnosis and venous anatomy is, whether it's going to be transvenous or epicardial, the type of arrhythmia that they have or may have in the future, because as we know, based on Melissa and Jamie's talk, that they will develop arrhythmias over time, what their activity level is, the lifetime of the therapy for them as they grow, and then obviously their growth. And so these two pictures, the one on the right is a friend of mine's child with an epicardial pacemaker, and he loves to draw pictures on it. And my picture on the left is a college-age diver that really wanted to pay attention to where her scar was. And so I think it's important to also work with your patient to know what type of device would work better for them. Moving on to how to program the devices, we'll start with heart block. So when you have a patient who has heart block, many of our patients with these types of lesions that Jamie spoke about earlier will need a pacemaker. So you want to think about optimizing battery life. And all of the companies have automatic threshold management, so make sure you turn it on and you use it. You also want to minimize ventricular pacing. As we all know, this has been proven in all of the adult studies. Again, all of the companies have a variation of it. They're all listed here, but make sure that you have that turned on. And then you also want to optimize your dual chamber settings. Kids' heart rates get up fast, so you want to have a higher upper tracking rate. You want to use rate-adaptive AV. That helps physiologically shorten their AV when they are exercising. And then also remember to program your upper rate less than the total, less than the TARP. And I think we all know this, but it's something that we have to think about very much in pediatrics. And the reason is this is a patient that I had taken care of who was exercising on the elliptical machine. And as you can tell in the bottom picture here, heart rate goes up, up, up, hits the upper tracking rate, and then drops in half. And as you can imagine, that would feel pretty crappy when you're a kid that's going 180 to 200 beats per minute. So again, make sure you know how to optimize your pacemaker for a child or a very active person. So sinus node dysfunction is most common in heterotaxy and, again, lesions that lead to scarring in or near the sinus node. The best programming for those with sinus node dysfunction is rate response pacing. And some of the things that I looked at during this talk is some practical pearls. And when you program the activity threshold, these are the terms that will come up on your programmer. Low, this responds to most body activity. Medium to low responds to moderate or greater exertion. Medium to high is limited response to only moderate movement. And high is an only response to vigorous movements. So depending on your patient's activity level, you may choose one of these rate response modes. We typically use the out-of-box setting, which is medium to low. But you may need to adjust over time based on your own patient. This is what it would look like. This is a specific Medtronic device, but obviously there are other devices that could have this. And the key here is the ADL response. This alters the target of rate distribution in the ADL rate range. The exertion response alters the rate distribution in the exertion rate range. And these are the choices that you would get for your patient. Out-of-the-box settings is made for an adult. So the ADL rate is 100. Many of our patients are at 100 all the time or even higher. So you just need to make sure that your upper sensor rate and your ADL rate is programmed based on your patient. Moving on to ICD shocks, that's something that's very important in pediatrics, I'm sure, even in adults as well. But we definitely want to minimize as much as possible any ICD shock. So for VF detection, we program it high, over 200 beats per minute in many instances. We also program a longer detection time. We utilize ATP during and before charging. And then they're now coming out with additional types of ATP. So this one is called IATP, which is an individualized ATP. And it's, again, something that can help avoid a shock in patients. Also using multiple zones, so a fast VT zone or a VT zone can, again, prevent an ICD shock. And these are all appropriate shocks for your patients. Now we also want to minimize inappropriate shocks. And there are several algorithms within the devices that are automatically on or we can program on. The first one is T-Wave Oversensing Algorithm. We can use this in both a traditional ICD, so that's either an epicardial or a transvenous. Also the subcutaneous ICD has a similar feature that's called SmartPass. And the goal of these features is to differentiate the EGM between an R-Wave and a T-Wave. So the picture to the left on the top shows what it looks like before the T-Wave algorithm is utilized. And then the picture on the right shows a different size between the R and the T-Wave. And the same thing for SmartPass. They work generally the same. And this can help avoid double counting and can avoid an inappropriate shock. There's noise detection or lead integrity alerts. And these reduce over-sensing of rapid signals, also important to program for your patients. And then lastly, utilizing SVT to VT discrimination. There's a couple different kinds. I'll just kind of touch on a few of them. So if the onset is gradual, it's more likely to be SVT than VT. If it's a non-stable rhythm, it's more likely to be VT. And if the arrhythmia morphology does not match the normal sinus rhythm template, it's more likely to be VT. And these can help reduce inappropriate shocks in your patients. Now many of our patients that we're talking about today do have atrial arrhythmias. And we use ATP for our atrial arrhythmias. There's varied success. According to the studies, it's between 57% and 72%. Success of the ATP can be dependent on the type of repair. It's more successful for two ventricle repairs versus one. If they have documented IRT before implant, it's most likely to work. And it's least likely to work in patients with an LTGA. We have to remember that there's a small risk of degeneration to either AFib or ventricular fibrillation, which is shown in this patient. So the ATP is in the box. And then the patient turns into a disorganized rhythm and eventually comes out. So the ATP actually didn't terminate the rhythm. The disorganized rhythm ended up kind of spontaneously converting. And the patient did not need a shock at that time. So programming ATP, there's Burst Plus. This is programmed. It's a programmed number of pulses followed by two premature stimuli. That's shown in here in this bottom box. And there's also RAMP. And it's programmable number of pulses delivered at a faster cycle length, which is shown here. And all of these graphics come from Medtronic Academy. It's a great place to get information if anyone is looking for additional information. According to the study out of Iowa, there's no difference in success of atrial ATP between Burst Plus and RAMP. So I think you just kind of choose based on how you're feeling that day. Ventricular arrhythmias we touched on a little bit with Melissa's talk and Jamie's too. As we talked about, the risk of ventricular arrhythmias is highest in Tetralogy of Fallot patients. And there is a risk of sudden death in patients with congenital heart disease. And this is something that we think about all the time. This paper comes out of Jack. And the two, the Venn diagrams, the blue part is anatomical macro reentry. The red is the scar-related focal micro reentry. And then the risk of sudden death at the top is lower than the risk of sudden death at the bottom. So our biggest patients that we worry about are the atrial switch patients that are most likely to have some sort of risk for sudden death. And this paper is actually very helpful in describing the different types of risk for various congenital heart disease. Same thing with ventricular ATP. You can program that on for patients who may have a more stable ventricular tachycardia to avoid a shock. There's Burst, which pacing pulses are of equal cycle length. There's Ramp, which is a decremental interval. And then there's Ramp Plus, which is the first three pacing pulses at a programmable percentage of the detected VT. And then there's subsequent pulses at a percent of the third pulse. And I just want to touch on the benefit of remote monitoring. I think it's very important for our pediatric patients. Again, we had another guideline published in 2023 in HeartRhythm. That's the Expert Consensus Statement on the Practical Management of the Remote Device Clinic. This was an updated version from 2015, and there's been a lot of changes since then. There was a specific pediatric section in this guideline that stated there was significant benefit for pediatric and congenital heart disease patients to detect lead issues, battery depletion, and arrhythmias. It allows for early detection and therefore early intervention of any arrhythmias. In a study that we did at the University of Michigan, tachycardia of course is the most common abnormality found on remote monitoring in this population. So again, it's very important to have your remote monitoring working well and communication with the patient as well. So future directions, I'm going to have to put in a plug for HRX. Jamie has been there, Nate, Lisa, all of us. And you know, this is a place where we can go and actually speak with industry on needs that we need for pediatrics. Things that we should talk to our industry colleagues about are smaller devices. These devices are made for adults. They're not made for kids. We also should fight for pediatric and congenital heart disease programming. We're most of the time trying to fit our patients into their programming, and that can be a big challenge. We do have some option to do remote programming. That's little things like turning on and off alerts. But just like Jackie said, many of our patients are over an hour away. And if you have to make a simple change, they have to drive to us. And that can be a big challenge for our patients who have low resources as it is. Anyway, HRX is in September in Atlanta. If you have more questions about it, there's a lot of information here at HRS this week. So our take-home points today, one size does not fit all for device selection or programming. Programming the pacer for the long game. We know these patients will have these devices for their lifetime. Atrial and ventricular arrhythmias are common in congenital heart disease, so make sure you utilize ATP as much as possible. You want to focus on reduction by utilizing shock reduction features. And remote monitoring is key for arrhythmia detection and management. And I do not have a cool QR code, but I'm sure anyone can find me if they need me. Thank you very much. »» We'll go ahead and open it up for questions. If anyone has one, please come up to the microphone. I want to start off with our congenital patients. They don't have a pacing indication. Are you guys seeing EVICDs placed in these patients that are at risk for sudden cardiac death? »» I mean, not yet in our institution, but I think that's something that we discuss all the time. Wondering what Jamie says from Boston. »» I have seen a couple, but it's definitely very carefully selected patients. »» What about you, Nate? »» We haven't done any congenital heart disease patients, but we've done one on a structuring normal heart. »» So I think in pediatrics, it's slow, just like everything, takes a long time to take off. But we'll eventually find its place in how to implant it. I think we have to get our surgical colleagues obviously involved too in that. That is a whole other follow-up, Dr. LePage is in the back here, and I know he had started some beginning conversations with our pediatric surgeons on that. »» I have a question here for Jackie. What has become my recent project is to find all of the devices I'm still following that belong to anybody over the age of 21. And I have a decent amount. And so do you, what I'm finding is that I can send them a letter, I can recommend that they see an adult EP, but do you, then the adult EP side doesn't, they give one phone call and if they don't answer, they're done trying, which is deeply different than the pediatric side. So do you have any tips or tricks to getting adult colleagues to try harder or how I can amend my expectations to make that successful? »» Yeah. At our institution, we are blessed with an amazing device nurse coordinator who recently under-tasked a similar project as well. And she did the similar method of just persistent follow-up with a patient, see where they're at and what are they doing. And I was actually surprised. There was a small amount of patients that actually already had transitioned and they just didn't know we had their remote monitoring and just established a new account for them. Which I thought was interesting. A very small amount. But I think persistence is key. I think also having a conversation, if you get ahold of that patient, of where they want to go to be able to nail down something that's close to home and accessible for them. Sometimes our neighbors next door, the adult institution may not be the best fit for them. So thinking outside the box and trying to bring the care as close to home as you can for them. »» Yeah, I've definitely done that. I've launched it to where do all of our patients live in Illinois and Missouri and trying to figure out someone in each region. »» You had mentioned using peer support. How do you go about getting other patients to be able to talk with patients that are making that transition? »» Yeah. We, I feel like we struggle in regards to connecting with peer support. We do have an annual event at our hospital that we do invite all of our device patients come in September. So that's a great way for kind of peers to peers to kind of more naturally establish like that peer support. And so we have like lectures for parents and then we also have like a teen room as well too. And then for our little kids we have like other pacemaker safe activities for them. So that's like a really good event. It's a lot of work. We rely heavily on our volunteers to pull it off. But it definitely has grown to develop great peer support at multiple different ages as well. But we also try to connect on the individual level if a family reaches out in between as well. »» So I think that these kids these days, it sounds so old. I think that they are so innovative and really cute. This just happened last week. One of the patients, I took care of both of them as newborns. One is a fontan now. And the other one was a very sick truncus who was on support for a handful of days. And they created their own podcast. And it has like, I mean, created so many roots in the congenital heart population of our area that they're having like all these interviews of people telling their story. And so I think that, you know, the world is so young in that all of what they're branching into with podcasts and social media platforms, that it's really helping that community in some sense. So we can do all of our reunions and stuff, but it doesn't quite connect and have that reach. »» Yeah. »» Yeah, that's great. Will you share that with us so we can send it to our patients to listen to? That's awesome. Any other questions from out there? All right. »» Give you back a little time. »» Yeah. We'll call it a day. Thank you so much, everybody. This was a great session. Thank you.

congenital heart disease

CHD management

arrhythmia development

pediatric to adult care

cardiac implantable devices

healthcare transition

surgical interventions

personalized device programming

remote monitoring