February 24, 2023
The world watched in shock as Damar Hamlin, a safety for the Buffalo Bills, collapsed on the field during a Monday night football game against the Cincinnati Bengals on Jan. 2, 2023. If parents and pediatricians weren’t already nervous about pre-sport physicals and sudden cardiac death, that scene certainly reinforced everyone’s worst fears. But could screening of any kind have prevented that incident? And what is really most important about cardiovascular risk screening in the primary care office, for athletes or for any of our young patients? Joining us in this week’s episode is cardiovascular Kid Expert, Dr. Chris Carter, pediatric cardiologist and co-medical director of the electrophysiology program at the Children’s Heart Clinic at Children’s Minnesota.
Transcript
Dr. Angela Kade Goepferd: This is Talking Pediatrics, a clinical podcast by Children’s Minnesota, home to the kid experts where the complex is our every day. Each week we bring you intriguing stories and relevant pediatric healthcare information as we partner with you in the care of your patients. Our guests, data, ideas and practical tips will surprise, challenge, and perhaps change how you care for kids. Welcome to Talking Pediatrics. I’m your host, Dr. Angela Kade Goepferd.
The world watched in shock as Damar Hamlin, a safety for the Buffalo Bills, collapsed on the field during a Monday night football game against the Cincinnati Bengals on January 2nd, 2023. If parents and pediatricians weren’t already nervous about pre-sport physicals and sudden cardiac death, that scene certainly reinforced everyone’s worst fears.
But could screening of any kind have prevented that incident? What is really most important about cardiovascular risk screening in the primary care office for athletes or for any of our young patients? Here to talk to us about all things heart health and cardiac screening is our cardiovascular kid expert, Dr. Chris Carter, pediatric cardiologist and co-medical director of the Electrophysiology program at the Children’s Heart Clinic at Children’s Minnesota. Chris, thanks for joining me today.
Dr. Chris Carter: Thank you for having me today.
Dr. Angela Kade Goepferd: The Damar Hamlin situation certainly represents our worst nightmare. I don’t know of a single person who watched that and didn’t get struck with fear. But really, how common are scenes like that or sudden cardiac death in student athletes?
Dr. Chris Carter: As a big picture, they’re very, very rare events, probably about 2,000 events in the United States per year in people under the age of 25. While that seems normal, that’s a population of about 99.5 million people.
Dr. Angela Kade Goepferd: Wow.
Dr. Chris Carter: So they actually end up being quite rare events. Obviously, when they do happen however, they’re very traumatic events and usually have a out-sized impact on community. Certainly for families and friends, they’re obviously life-changing events. So while they’re rare and fortunately we don’t see them very often, when they do happen, they obviously have a huge impact on the community.
Dr. Angela Kade Goepferd: Right. No, it’s like a plane crash. They rarely if ever happen, but when they do, it’s a big deal and everyone feels scared about it even though the chances are minuscule.
Dr. Chris Carter: The other thing to know too is we certainly see events like that in non-athlete populations as well, but certainly the risk is higher in athletes. It’s probably about two and a half times higher. Most of the events we see like that do happen in athletes, but we unfortunately also see them in kids that aren’t participating in sport under different circumstances as well.
Dr. Angela Kade Goepferd: There was a lot of back and forth about Damar Hamlin and what caused his event on the field and was it commotio cordis? Was it other things? But for us in pediatrics, what are the things that we worry about causing a sudden cardiac arrest or an event in a student athlete?
Dr. Chris Carter: Certainly by far the most common reason for a sudden arrest like that on the field is going to be a cardiac reason. If you look at all student athlete arrests, about 70% are cardiac. So certainly that’s the primary concern. Of the kids with cardiac causes for their arrest, about 40%, 45% are due to hypertrophic cardiomyopathy. That’s a genetic abnormality, causes abnormal thickening of the heart muscle. But if you look at the heart muscle under the microscope, it’s also a very disorganized heart muscle. That puts kids at risk for ventricular arrhythmias, so arrhythmias from the bottom chamber of the heart, that are really the main things that we worry about in student athletes and sudden death.
Second most common thing that we worry about in kids would be coronary artery anomalies, specifically a left coronary artery coming off from the right sinus of Valsalva. It’s a congenital heart defect that oftentimes is asymptomatic until something like this happens. An exam and an EKG would be very, very normal in those kids outside of a sudden event.
Those two things by far are the most common things that we see. So the coronary anomalies probably make up another 15%, 20% of these kids. Then there’s a whole variety of other, both heart muscle, heart structure, and electrical abnormalities that can cause events as well.
Dr. Angela Kade Goepferd: I’m curious, when you mentioned that anomalies of the left coronary artery, you’d have a normal physical exam, you’d have a normal EKG, how useful are pre-sport history and physicals in picking up things that might lead to an event?
Dr. Chris Carter: I will say they are better than nothing, but the difficulty is we are talking about very rare problems. Certainly for the majority of things that are going to cause sudden cardiac events, a lot of those have a genetic basis. For example, again, hypertrophic cardiomyopathy is a genetic disease. So very commonly in patients with hypertrophic cardiomyopathy, there will be a positive family history and then that’s part of the screening. A good history and physical exam are really the best thing that we have, and they are useful to identify athletes with risk. Any time we’re talking about a very rare set of problems, even with the best tests in the world, the chance of having a good positive predictive value on a test is still going to stay very low.
Dr. Angela Kade Goepferd: You mentioned family history, particularly with hypertrophic cardiomyopathy, what other things should we really be attuned to looking for in a family history that would make us want to raise our eyebrows when it comes to a pre-sport history and physical?
Dr. Chris Carter: Anybody that takes histories will recognize that family histories can change. It’s highly dependent upon the people that you’re asking. But certainly I find the most useful screening question in a family history is any sudden unexplained death under the age of 50. I will keep that relatively broad and even give examples. Single car accidents, unexplained drownings, any death in a relatively young person without any obvious explanation is useful. A lot of families will describe that as heart attacks, which is a very vague term, but I often find that as a general, open-ended question, that is probably one of the more productive ones that we have.
On the flip side of that, I will always also ask specifically about the more common things from a cardiac point of view we concerned about. So I will mention hypertrophic cardiomyopathy, coronary abnormalities, long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, which is a mouthful-
Dr. Angela Kade Goepferd: Yeah, say that five times fast.
Dr. Chris Carter: …and Brugada. I will say surprising that people will say no to a general screening question, but specifically naming, at least from a cardiac point of view, the more common ones we worry about, sometimes that will trigger a light, and someone in the room will go, “Hold on, didn’t cousin so-and-so have something like that?” So there is utility in specifically naming those as well.
Dr. Angela Kade Goepferd: You’re an electrophysiologist, and one of the more common questions that I get from families when I’m doing these screenings is about arrhythmias in the family. Arrhythmias are not entirely uncommon for people to have here and there. Many of them are benign, but are there any particular arrhythmias that, you mentioned long QT, beside that that would cause us to take some extra precaution or do some extra testing?
Dr. Chris Carter: I think the vast majority of arrhythmias, fortunately, that we’re going to run across in young people are benign. They can be uncomfortable, they can be annoying, but they are not dangerous. The ones that we really worry about being dangerous are ventricular arrhythmias. Even then, not all ventricular arrhythmias are dangerous. Certainly the most common ventricular arrhythmia we see in young people is what we call idiopathic PBCs or idiopathic ventricular tachycardia, and that actually usually goes away when you’re up and active.
Specifically what we’re really worried about are either structural problems or electrical problems that set you up for ventricular fibrillation or what we call polymorphic ventricular tachycardia. Those are things like long QT syndrome or short QT syndrome, a CPVT, which is the easier way to say catecholaminergic polymorphic ventricular tachycardia and Brugada are the ones there.
Commonly, and I think this is an area where especially for primary care doctors it’s a little more difficult situation, is WPW, Wolff-Parkinson-White. That’s an extra electrical connection in the heart. There are actually two completely separate reasons that we care about it. One is it is a setup for SVT, and SVT, even in WPW, is still a benign rhythm, meaning that we don’t typically worry about that causing harm or worse rhythms.
What we do worry about in WPW is if you get atrial fibrillation. If atrial fibrillation, that little extra connection doesn’t always protect the bottom of the heart like the normal conduction system will, and you get rapidly conducted atrial fibrillation. That’s where the risk of sudden death comes with with WPW. Fortunately, that’s rare. It’s 2% to 3% of sudden death is secondary to rapidly conducted atrial fibrillation. That’s an area where, especially when parents start reading, can become a little unclear to them. WPW under very specific circumstances can cause that as well.
Unfortunately, patients with WPW seem to be more prone to atrial fibrillation, so we still do see that. But in the 12 years I’ve been here, I’ve only seen three cases of that. So fortunately, that’s pretty rare, and we see a lot of WPW patients.
Dr. Angela Kade Goepferd: Yeah. The other thing that we do in a sports physical is there’s this big long page where families have to go through and mark all of these things about things that have happened to a child or things that haven’t happened to a child. What are you looking for as a cardiologist on that form that would trigger further investigation?
Dr. Chris Carter: This is true the Minnesota state form. Those are all based off of this 14-point history and physical from American Heart Association in association with PACES, which is the pediatric electrophysiology professional group. You’re right, it’s a fairly long form [inaudible 00:10:05] go through to figure it out. It asks a lot of questions, which I feel like are almost designed to have at least one positive somewhere.
The specific things in a history that would make me more concerned are really exertional symptoms. That’s exertional chest pain, meaning chest pain during activity, especially more atypical chest pain. In a kid, very commonly these sharp chest pains that are associated are musculoskeletal. But a chest pressure or chest discomfort that’s hard to put words to would be more concerning actually for me. Exertional syncope, so fainting during exercise is also, I would say, a fairly big red flag. Fainting after exercise, less so. That’s certainly more common to be vasovagal. But running down the field and going down is obviously more of a concerning thing.
Then dyspnea upon exertion. This isn’t just the kind of I feel I’m not able to keep up or I’m short of breath. If you look at the HA form, it’s really designed to be something that’s really out of proportion to what you would expect for an otherwise normal, healthy patient. That is something as well that would be concerning to me. So really those exertional symptoms are a big trigger and really what clue me in.
Also, I will say a history of previous cardiac evaluation or intervention, disturbing. The number of patients we’ll see in a cardiac clinic where they don’t report a family history and the kid takes off their shirt and there’s a sternotomy scar, and you’re like, “Oh, what’s that?” Then they have a history of a VSD repair or coarctation repair that the family just put out of their mind because they haven’t seen a cardiologist in 10 years. So that personal history is important as well.
Dr. Angela Kade Goepferd: If we come across a concerning family history feature or a concerning checkbox on that form, what’s your recommendation? This is going to be a little bit setting-dependent because different people have different access in terms of … In the primary care office, should we go ahead and be ordering that initial EKG and/or echo? Should we be just going ahead and sending them to cardiology for further evaluation? What’s your recommendation?
Dr. Chris Carter: I definitely think if any red flags come up, getting a baseline EKG is a very reasonable thing to do. If you’re in an office where your staff in particular is comfortable doing a pediatric EKG, it’s often very nice to have those even before I see a patient to know if there’s anything there that preps me for a specific conversation. So I certainly think doing an EKG in the office with staff accustomed to doing a pediatric EKG is useful.
Recognizing that a lot of EKG machines set in primary offices or family practice offices aren’t set to read pediatric EKGs, and so you might get a lot of scary things that come out on the automatic read that are actually normal for kids. If you’re in an office where you either don’t have an EKG machine or don’t have staff that are comfortable doing that, we are certainly always happy to do that when we see them as well.
I do think it’s important that if you’ve gotten to that point that there’s level of concern, I do think it’s good to see a pediatric cardiologist, one, to help interpret that EKG in terms of whether it’s normal or not. Then two, to have those discussions about potential things that families either have questions about at the time of their visit or will go home and read about and have questions later. I never think it’s wrong for us to see those kids.
I would probably say in terms of an echo, I’d hold off until they see pediatric cardiology, just because a lot of times hearing the story, doing an exam, and seeing that EKG, an echo won’t necessarily be something that is needed for followup. But I certainly think in a primary care setting, if you’re comfortable getting an EKG, it’s a very reasonable thing to do if a red flag comes up.
Dr. Angela Kade Goepferd: In a kid who may have a family history of a sudden cardiac event, like you mentioned, their physical exam is relatively normal, the EKG is normal, but they still have this concerning history. At that point to complete the workup, would you recommend that they do see a cardiologist and get an echo in your office just to make sure?
Dr. Chris Carter: Yeah, and I think one of the reasons it’s good to see a pediatric cardiologist before doing the echo is depending on what the family history is, screening begins at different times. A good example is true genetic hypertrophic cardiomyopathy is almost never seen pre-adolescence and really is something that shows up during adolescence. So current recommendations, even with a parent with hypertrophic cardiomyopathy, we really start screening kids at age 10. So depending on what it is, age of screening is important.
On the flip side, long QT, if you have long QT, you can do an EKG in a two-year-old and see whether that’s present or not. Some of those screening tests are going to be a little bit age-dependent. Depending on what the concern is, even more advanced testing may be needed. There’s an entity called arrhythmogenic right ventricular cardiomyopathy or dysplasia [inaudible 00:14:46] an echo and an EKG can actually be looking pretty normal in those patients, but an MRI is actually what you need to do.
Dr. Angela Kade Goepferd: Got it.
Dr. Chris Carter: So screening isn’t always as straightforward as just EKG and echo. Something that’s also becoming more important in screening for a lot of these things is genetic testing. Being able to discuss that and direct them to genetics and get that appropriate testing done is very useful. Fortunately, the genetics team here, specifically Allison Berg, who does all of our cardiac screening, is a big part of a lot of that evaluation as well. Can certainly go beyond the EKG plus or minus echo in terms of what’s appropriate for a family and then helping them navigate what all that means down the line.
Dr. Angela Kade Goepferd: A little bit of a controversial question. Starting particularly more in some of our suburban teams and families is this concept of just screening everybody with an echo before they play sports. Families can pay for that if they want out of pocket to just go get an echo done on their child. What are your thoughts on that?
Dr. Chris Carter: One, it is contrary to current recommendations for some of the reasons we’ve discussed. An echo is not always going to rule out a problem. Having a expert in an evaluation of those particular problems is going to be important to be involved. At these screenings, it’s not necessarily a pediatric cardiologist. It is not a pediatric electrophysiologist. It’s sometimes an adult cardiologist or even maybe no advanced care practitioner. So there’s a little bit of, I think, difficulty in terms of counseling and I think could provide a false sense of security to families.
Those screening echoes really are pretty much focused on valves and heart muscle. They don’t really look at the coronaries. As we discussed, that’s the second most common structural abnormality that we worry about. We’ve certainly seen a lot of these kids that come from these screenings where they do pick up stuff, often atrial septal defects or well-functioning bicuspid aortic valves, so things that are good to identify and follow but aren’t necessarily a risk for sudden cardiac death. But they’re also are potentially missing things that a echo done at a pediatric center would be more focused on evaluating.
Echoes in general are also expensive tests. Unfortunately, cost is something we have to consider, especially … really in any country when evaluating how best to screen and where we’re using our resources. So my biggest concern is the cost that might not be giving the results we want, and I think it’s a false sense of security for some families, and you could still very well have something that’s just missed.
I do think those screening programs are set up with really good intentions, and I don’t think any of them, specifically Play for Patrick is probably the most common one out there. It’s not a money grab. It’s not done for some malicious intent. I think it really is well-intentioned, but appropriate screening with the appropriate tests with the appropriate people evaluating those tests, I think, is really important, and it’s just not always something that’s available at those.
Dr. Angela Kade Goepferd: One other question that I was wondering. We’ve really honed in on cardiac conditions, but there are other things that kids can have and that can affect them that could put them at risk on the sports field even for a cardiac event that’s from a non-cardiac origin. I do a lot of care of adolescents, and so eating disorders or uncontrolled eating disorders come to mind for me. But what are some of the things that come to mind for you that we should really be paying close attention to because they could have an impact on a sudden event with an athlete, but not because they have a coronary primary cardiac condition?
Dr. Chris Carter: Trauma. Trauma is, outside of cardiac, the most common reason for sudden death in athletes. Just being certain that the league or the schools are following appropriate guidelines in terms of safety equipment and contact and concussion restrictions and all of those things is very important to be sure that we’re playing safely is a big one.
Certainly another thing we’ve seen is heat stroke, being very cognizant, especially for summer sports or sports that begin training in the summer, especially those with equipment. So football, I feel, is the one we usually will hear about with this kind of concern. So being aware of the signs and symptoms of heat stroke, heat exhaustion as an earlier sign are very important to pay attention to, and they can be very dangerous if they’re ignored. So having an option or a place for kids to go down and cool off, maintaining really good hydration, and listening to kids when they’re telling you that they’re not feeling good I think are all really important things for coaches and schools, again, to have in the back of their mind.
Certainly other things that are on that list, drugs. In addition to drugs, I will throw supplements out there. A lot of supplements are very popular with athletes and student athletes. These are unregulated substances, and we don’t always know exactly what they do, and we don’t always exactly know what concentrations are safe. Because they’re supplements, they’re unregulated and unstudied. So there’s certainly things out there that cause some weird things to happen and I think need to be used with an eye towards not using them, especially if they’re making you feel not good.
Then there are certainly other things out there in terms of substance use that we see. Synthetic marijuanas have been associated with some really weird cardiac and respiratory issues. Again, anything that’s unregulated that affects the body in ways you don’t understand could potentially be a risk.
Dr. Angela Kade Goepferd: If someone listening has a student athlete or really anyone in their practice that even if they’re not an athlete, because as you mentioned, it’s not just athletes that have sudden cardiac events, where they’re worried that they need more evaluation from a pediatric cardiologist, what’s the best way for them to get in to see you and your team? What’s the wait like for someone who wants to get an athlete seen?
Dr. Chris Carter: Certainly you could always call the heart clinic, shameless plug. If you call us during daytime hours, you’ll get our triage nurses usually, and they can help facilitate that. If you call after hours, you’ll get one of us, and we can help facilitate that as well. We save spots in our schedule for urgent add-ons. So as soon as the next day or even same day depending, we can get someone in and evaluate them. If it’s really a situation where we think it’s important for them to be evaluated, we’ll make space for them to get evaluated and get the testing they need so they can get back to playing.
I will also say that a couple years ago, AAP actually put out a recommendation that beyond athlete screening, every two to three years, just a very short general screening for kids is not necessarily a bad idea. They put out a four-question easy thing that’s a little bit of a modified 14-point, mostly focused on family history.
Dr. Angela Kade Goepferd: Well, thank you so much for joining us again on the podcast. Learned a lot as always, and appreciate your help in caring for kids and making sure we can keep everyone healthy and on the field safely.
Dr. Chris Carter: Yeah, and that’s what we’re here to do. So thank you for having me.
Dr. Angela Kade Goepferd: Thank you for joining us for Talking Pediatrics. Come back each week for a new episode with our caregivers and experts in pediatric health. Our executive producer and showrunner is Ilze Vogel. Episodes are engineered, produced, and edited by Jake Beaver. Amie Juba is our marketing representative. For more information and additional episodes, visit us at childrensmn.org/talkingpediatrics, and to rate and review our show, please go to childrensmn.org/survey.