Chapters Transcript Video Cardiomyopathy & Heart Failure in Children Zachary Hutchinson, MD discusses cardiomyopathy & heart failure in children. Right. Thanks for having me. Appreciate it. Um, so I did my cardiology fellowship at UCSF and uh went away last year to Seattle for a 4th year in heart failure transplant and then started back in UCSF in October. So nice to virtually meet everyone and I hope, uh, this is useful for everyone. And I wanna thank my colleague Othman Al-Jihani for, um, graciously giving me these slides. Um, so the objectives of this presentation are that, uh, providers will be able to define heart failure in children, identify risk factors, recognize clinical presentations, initiate workup, understand the etiology of iron deficiency in children with heart failure, heart failure, and formulate an approach when suspecting heart failure in a child. Um, I hope one day I might have a financial conflict of interest, but I currently don't have any financial or commercial interest to disclose. Uh, so this is just a reminder that this topic does show up on pediatrics and pediatric emergency medicine boards. Um, while there's some overlap in how cardiomyopathy and myocarditis patients present compared to other forms of congenital heart disease, I'll be focusing on heart failure related to cardiomyopathy and myocarditis. Um, so lucky for you or not, I will not be covering, uh, single ventricle physiology in detail today. Um, this is a slide just showing that there's this, uh, Children's Cardiomyopathy Foundation that's an excellent resource, uh, in particular for families to learn more about these conditions, and there's another, um. Foundation called the Action Learning Network, uh, that's a great resource for families, um, and in particular, there's a lot of education material for providers as well. And, uh, when we think about pediatric heart failure in general, uh, we can divide it up into four main categories. So, cardiomyopathy is, um, heart muscle disease that can be either genetic or secondary to other diseases. Cardiotoxicity is heart injury, um, is heart muscle injury that's caused by medications or treatments, and the classic example of that would be anthracycline induced cardiomyopathy. Myocarditis is heart muscle injury due to infection, and in pediatrics, this is most commonly a viral infection, um, or idiopathic. Um, we don't find an identified cause. And then congenital heart disease, um, is not included in this talk, um, but all of those lesions can certainly present with heart failure. And most of the time nowadays, um, these conditions are diagnosed via fetal echo. Um, but not all the time, and, uh, VSDs would be the most common cause of heart failure due to congenital heart disease. So put most simply, heart failure is defined as a clinical syndrome that occurs when cardiac output is not sufficient to meet the metabolic demands of the body. And then the second definition is a more cardiology-ish definition, um, and that incorporates more congenital issues, and it is failure of the heart to supply blood to either systemic or or pulmonary circulation at an appropriate rate of flow, or to receive venous return at an appropriate filling pressure, resulting in adverse effects on the heart, the circulation, and the patient. For the purposes of this talk, the term heart failure is limited to children with signs and symptoms secondary to ventricular dysfunction, um, which can be either impaired ejection or impaired filling of the ventricles. These are some other terms that you may hear and just so you know what we're trying to communicate, um, acute decompensated heart failure is heart failure severe enough to require hospitalization. And then advanced heart failure is, uh, heart failure that is refractory to conventional heart failure medications, which I'll cover at the end of the talk, um, and may require additional therapies such as, um, inotropes or Millerone ventricular assist devices or transplant. And I have a brief couple of slides on pediatric heart failure staging and functional class systems uh that I think are quite useful in communicating uh the patient's overall heart failure status and how affected they are by their heart failure. So the most common one is probably this ACC AHA heart failure staging system, and it has stages A to D. and I just remember that A is the mildest, and these patients are essentially at risk for heart failure, um, but still with no symptoms and a normal echo. So this would be, for example, um, someone who underwent, uh, chemotherapy with anthracyclines, but their echo is still normal and they're asymptomatic. And then I just remember stage D is the worst, um, i.e. refractory heart failure that will essentially land patients in the ICU for advanced therapies, um, like I mentioned, things like Mil Renone and ventricular cyst devices. Uh, then B and C are, of course, then in the middle. Uh, stage B has echo changes, but, uh, still no symptoms. So this might be that same patient who was exposed to anthracyclines and has, um, just some mild dysfunction, but it's not causing symptoms yet. And then stage C, um, has echo changes and symptoms, but they're overall, um, decently well compensated and, um. Can still for the most part, um, stay out of the ICU and out of the hospital. Um, but we usually initiate heart failure medications at Stage B, um, just so we can do everything we can to prevent progression. And if there are more symptoms, um, which we'll go over, um, in detail, then that's when we typically add diuretics and other things we consider salt and fluid restrictions. The second heart failure classification system is called the modified ROSS, and it's quite useful in pediatrics world, um, where we of course can ask the babies about their exercise intolerance, and it's again class 1 through 4 with 1 being asymptomatic, um, 2 is mild tachyney or or sweating with feeds. Class 3 has a worse tachypnia with feeds, prolonged feeding time, and failure to thrive, and then class 4 is symptoms at rest and still with failure to thrive. So this one is nice because it includes uh failure to thrive, and if you have that, it automatically puts you at a class 3 or a Class 4. Unlike adults who present with shortness of breath and edema, presenting symptoms in children can be non-specific and misleading with GI symptoms and respiratory symptoms being the most common. So my biggest plea is to not forget about the heart if you have a patient with failure to thrive, and if a patient has both GI symptoms. Especially hepatomegaly and feeding intolerance and respiratory symptoms, um, in particular, tachypnea and work of breathing with feeds, um, in the absence of a clear, um, URI, um, then I think I would just think of it as a cardiac issue until proven otherwise. Um, so you can see in this chart here that GI and respiratory symptoms for all ages are the most common presenting symptoms, and things like chest pain and frank fluid overload are rare, and um, it's pretty advanced heart failure if they get to those points. We can crudely assess a patient's hemodynamics based on their presentation using this uh 2 by 2 chart of wet versus dry and cold versus warm. Um, so the wet versus dry, um, assesses the patient's fluid state and then the, um, warm versus cold assesses their profusion. So to assess for someone's uh profusion, i.e., warm or cold, we would look for things like hypotension, cool extremities, and possible decreased level of consciousness if it's um severely decreased perfusion. And then to assess a patient's fluid status and look for wetness, um, we look for hepatomegaly, tachypnea, orthopnea in older kids and edema, in older kids, teenagers. Um, and then sweating with feeds, and these are all signs of, um, a high fluid state and likely elevated ventricular filling pressures. So, the holy grail, if you will, of heart failure is to be warm and dry, which means that you are well compensated. Warm and wet means that you are in a fluid overloaded state, and the treatment should include diuretics. Cold and dry may present with hypotension, poor pulses, cool extremities, and treatment in these cases should include um otropes and vasodilators such as uh Merone or epinephrine infusions. And then cold and wet um is the worst place to be, and that requires a combination of inotropes as well as diuretics, um, and also potential for advanced therapies like we'd be considering ventricular cyst devices. And I, uh, one other point to add here is that, um, this assessment can be tricky in pediatrics sometimes, in particular if, um, you know, babies are crying and upset and maybe they haven't been wrapped up, so, um, I would just say it's, it's best done if a baby's, you know, been swaddled because if they're cold just because of environmental reasons and they warm up, then that's reassuring. Um, and like, and same if they're just kind of losing their mind and crying really hard, that, that, that can of course, uh, make them look bad too. Um, so ideally, um, try to control what we can as far as environmental factors and then, then do the assessment, but easier said than done, I know. Um, so this Kappeln-Myer curve shows that survival based on initial presentation of heart failure is more strongly associated with congestion, um, than, um, low cardiac output syndrome. And, um, as you can imagine, a combination of congestion and low cardiac output, um, i.e., wet and cold, has the worst prognosis, um, followed by wet and warm. And then have just a summary slide here. I don't know if we wanna unmute people just for a minute, and we, people can take a stab at these or Or not. We can't unmute because it's a webinar. OK, uh, they could type it in the Q&A and I could let you know. Um, that's right, I guess I'll just, uh, You can uh say them out loud to yourself, I guess, and be satisfied with yourself if you get it right. Um, so heart failure can be simply defined as, say, inadequate cardiac output to meet the metabolic demands of the body. Um, acute decompensated heart failure is, um, heart failure that's bad enough to land someone in the hospital. Um, and potentially need advanced therapies if it's advanced heart heart failure. Um, ACCHHA classification has 4 stages. Uh, with 1 being at risk, um, and 4 being the most severe, and then 2 and 3 are in the middle. And then, as per the Ross classification, failure to thrive, um, automatically makes you a level 3. So this slide is not inclusive of all causes of heart failure, but say they're the most common ones that we see in our practice. Um, I'll review the cardiomyopathies in more detail, but they're listed here. Um, so dilated cardiomyopathy, chemo-induced, and post-myocarditis are all characterized by systolic ventricular dysfunction. Then hypertrophic and especially restrictive cardiomyopathy are characterized by predominantly diastolic ventricular dysfunction, um, so, i.e., poor filling due to stiffening of the ventricle. Um, and like I mentioned earlier, we can also see heart failure with any variety of congenital heart disease. Um, single ventricle patients after their stage palliation surgeries, so, um, after the Norwood, Glenn and Fontaine surgeries, can have other significant issues, um, that are due mainly to high venous pressures due to passive blood flow to the lungs that, that backs up into the IVC and SVC, um, that, and those issues can complicate their heart failure. And the common ones that we see, um, in those scenarios are protein losing enteropathy, plastic bronchitis, um, as well as liver and kidney dysfunction. Then some other kind of various etiologies of heart failure include cardiac tumors, um, uncontrolled arrhythmias, um, Kawasaki disease, if there's significant aneurysms and, uh, myocardial infarction, uh, metabolic disease, and then, uh, patients after transplant, particularly if there's, um, episodes of rejection, they can have heart failure. And I'll just say the uncontrolled arrhythmias, um, we'll, we'll go over initial workup later, but I would say this is definitely something that um you can pick up in clinic and um that's why EKG is a good first step. Um, we, um, I remember seeing a, a teenager who got referred for, um, low ejection fraction and underwent quite a few different tests and then, um, finally got to us and he realized he had been in atrial flutter, so, um, That's why EKG and just making sure that it's not an arrhythmia is a good first step. So etiologies of heart failure differ by age. Um, infants most commonly will have congenital heart disease, such as VSDs or coactation of the aorta, of the aorta, um, and the, uh, myriad of other congenital defects, um, tachycardias and inborn errors of metabolism or other considerations for babies. Older patients, um, we also think about autoimmune disorders as they can present with heart failure, heart failure, and I also add that boys with Duchenne's muscular dystrophy will commonly develop dilated cardiomyopathy in adolescence. And in general, cardiomyopathies have a bimodal age of presentation and will most often present either in infancy or in the kind of puberty, teenage years. And then I just wanna pause and spend maybe a minute more on this slide, um, because I, I think this is probably the most important slide of the whole talk, and if you get nothing else from this, I hope you can at least um recall the clinical features of heart failure in children. Um, so it's, it's a difficult, can be a difficult diagnosis in that, um, it mimics many other common diseases of childhood, um, particularly, um, feeding intolerance, um, that you may see with gastroenteritis or respiratory symptoms that you may see with bronchiolitis. Most patients with heart failure, um, will have fatigue or lack of energy, labored breathing, and that can be at rest or with exertion, um, or with feeds, um, abdominal pain, nausea, and vomiting. Um, so, again, just to emphasize there, the main presenting symptoms are usually not quote unquote, um, cardiac symptoms, but, um, GI and respiratory symptoms. And again, they may not be obvious in children. And, uh, resting tachycardia and tachypnea are, are two, common findings in heart failure. Blood pressure is typically normal, um, unless the patient is in cardiogenic shock. And like we know, kids can compensate for quite a while, um, until they, you know, quote unquote, crash and fall off a cliff. So, um, the goal is to identify the signs and symptoms before they get to the point where they're in shock. So things that we um look for on exam, um, as signs of congestion and heart failure, um, hepatomegaly and a gallop rhythm are two very important things to look for. And I think almost any pediatric cardiologist would tell you that the liver exam is the most underappreciated part of the cardiac exam and physical exam in general, um. It is our, you know, JVD or tibial edema assessment that adult cardiologists would look at. Um, so we spent a lot of time on the liver exam and just a few kind of practical tips if, um, if you don't do them frequently is, um, uh, number one, start, start in the pelvis because the liver could be all the way in the pelvis and if you don't start, um, feeling that low, you may miss it. And I, I think you kind of have to press harder than, um, some people do, and that's, and some people might think they. Are comfortable with because they're scared they're going to hurt the baby, but I'll just say that it's OK. You won't, um, you know, break the baby by doing a good exam. Um, and then you can also percuss the liver or do the scratch test, um, to help find the liver edge. Uh, so, normal is to not feel the liver. Um, Babies, I'll give them like 1 centimeter, centimeter below the right costal margin. Um, I think that's also normal, but if you feel the liver, um, anything below 1 centimeter, um, below the right costal margin in babies or if you feel it at all in older kids, then that is hepatomegaly. So, if you were to call me with a consult and say the patient has hepatomegaly and a gallop rhythm, my ears will really perk up because I know that the pretest probability that there's an underlying cardiac issue or heart failure is much higher than it would be for, um, pretty non-specific things like chest pain or a murmur. And then, um, again, the, Um, quote unquote adult, um, signs of heart failure such as tibial edema, ascites, JVD are, are, uh, rare, particularly in babies and other reasons, it's just really hard to see their neck, so, um. Yeah, I would say, please, uh, don't forget about hepatomegaly and then the gallop rhythm, just lots of practice listening to hearts, and there's lots of YouTube videos and things like that. Say it is. It is difficult to hear, especially if the babies are tachycardic, um, just because diastole becomes so short if they're, um, tachycardic. So a gallop, um, if we hear it is, of course, a concern, but, um, I would say not hearing it, especially if they're tachycardic, um, is not necessarily reassuring, in particular if they have hepatomegaly and other concerning signs. Um, then, of course, we look for signs of poor profusion, such as cap refill. Should be less than 3 seconds. Um, cool extremities are also concerned after the baby's been warmed up. And then you can hear a murmur in the setting of heart failure and that'd be um usually due to mitral regurgitation, um, and that's, you know, when you have systolic dysfunction, over time, the ventricle will dilate and that, um, uh, basically causes the mitral valve annulus to kind of pull apart and then, um, there's a gap, and then you can have regurgitation, um, in that setting. So why do heart failure patients develop iron deficiency? Um, so we know that heart failure, um, patients can have this, and that when they do have it, they have worse outcomes, and that's even without, um, anemia. And we think this develops due to a combination of, um, iron sequestration, um, due to inflammation, similar to that that you see with anemia of chronic disease. Um, as well as probably some decreased intake, um, if the, the baby is having feeding difficulties and then likely some decreased absorption, um, Due to some, you know, venous congestion and balledema. So, we frequently check iron labs and if patients are deficient, we give them IV iron, um, cause it's been shown that, um, oral iron is not sufficiently absorbed, um, and they need IV to, um, Replenish their iron stores. So we'll now transition to the cardiomyopathies, and I think this cartoon is a nice way to remember the three main, uh, flavors. So HCM hypertrophic cardiomyopathy is this bodybuilder, uh, dilated is this kind of sick looking bag, if you will, and then restrictive is this small but fierce and scary looking ventricle, um, that is to be feared. So this is what the cardiomyopathies will look like on echo. So this, um, Pain A is a normal heart, um, so you can use that for comparison for size, both of the, uh, ventricular wall size as well as the, the LV cavity and the atria size. So, um, this one here, uh, B, you can see is hypertrophic cardiomyopathy. So you can see the ventricular walls are much thicker, um, than is normal, and then that intrudes on the LV cavity. So the LV cavity itself is smaller. Um, so this causes the issues with ventricular filling. Um, so again, that one's mainly a, a diastolic heart failure issue. And the systolic function is, uh, usually fine. Um, in fact, it's, it often is, um, a little bit hyperdynamic. Um, C here is restrictive cardiomyopathy, and this one, has severe diastolic dysfunction due to, um, like the name suggests, just a restrictive and very stiff, uh, ventricular wall. So it becomes very high pressure, and then that backs up into the atria and causes a massive atrial dilation and, um, what we call the Mickey Mouse heart. Um, just cause the atria looked like Mickey Mouse ears. Um, and then dilated cardiomyopathy is characterized by a thin-walled LV, um, with systolic dysfunction, um, that leads to ventricular dilation. So dilated cardiomyopathy is the most common, uh, cardiomyopathy in children. It accounts for 50 to 60% of all cases. Um, again, these are, these are all rare things. Um, so the incidence of this is 6 out of a million. Um, myocarditis is the most common acquired cause, and about a third of all patients will require advanced cardiac therapy, such as a VAT or transplant. This is a more exhaustive table of the causes of dilated cardiomyopathy. Um, most commonly it's genetic or myocarditis, um, but one common one that likes to show up on boards is Barth's syndrome. So that's a combination of dilated cardiomyopathy plus neutropenia and skeletal muscle weakness. Um, and then I'll just say one other very important one, to highlight is Alkappa. So that is stands for anomalous left coronary artery from the pulmonary artery. And this presents with acute heart failure in babies. Um, so, like the name suggests, they have, um, their left coronary coming off their pulmonary artery. So as the PVR drops, they get, um, uh, basically coronary steel and very poor coronary perfusion, um, and this is, uh, deadly if it's not identified. Hypertrophic cardiomyopathy is the 2nd most common type of, uh, pediatric cardiomyopathy, and it accounts for 40 to 50% of all cases. Um, incidence is about 5 out of a million, um, and it can have, uh, left ventricular outflow tract obstruction, um, or it can be non-obstructive, and the outcomes overall are highly variable, um, but it also carries a risk of sudden death due to ventricular arrhythmias. And for this reason, um, all these patients are restricted from competitive sports. And, um, I guess I'll just add this when you're doing your sports physical, um. Or sports clearance physicals, um, what you're screening for when you have the, the kids do a, a squat and then rise up and listen for a murmur, what you're listening for is the left ventricular outflow tract, um, obstruction, um, that can be seen with hypertrophic cardiomyopathy. Um, and the squatting just changes the loading conditions of the heart. So, going from squatting to standing, you're lowering your, um, SVR, which can make the OVOT obstruction worse. You're also lowering the preload, so, um, that is why, um, that's Maneuver should be a part of your physical exams for sports clearance. Um, restrictive cardiomyopathy is a rare, um, cause in pediatrics. Um, it's overall less than 1 in a million. There's no known medications to improve survival, and patients can develop pulmonary hypertension, hypertension, which is associated with poor outcomes and may limit transplant options. Um, so we typically list them for transplant earlier than the other cardiomyopathy patients, um, due to this risk. And their um pH can be progressive also. Um, another picture of that I already went over. Um, so this slide again is just to highlight, highlight that um dilated cardiomyopathy is a systolic issue. So again, impaired ventricular ejection, and then restrictive and hypertrophic are diastolic issues, so impaired ventricular filling. Excuse me. So I have just a couple of slides here on anthracycline induced cardiomyopathy, um, which again causes a dilated cardiomyopathy picture. Uh, the risk of this is based on the cumulative dose that the patients receive. With those that get more than 300 mg per meter squared, um, likely have the highest risk, um, but really any exposure puts you at risk for this. And for this reason, all patients that get anthracyclines, um, will get interval screening echoes. And, um, radiation can also cause cardiomyopathy due to um interstitial fibrosis. Um, they can also um have um this affect the, the pericardium, so there also can be a risk for, um, uh, pericardial disease as well. And then anthracycline cardiomyopathy, um, can present at any point after exposure. Um, so there's, um, acute early onset, um, is less than a year, and then late onset progressive, um, is when it happens after a year. Um, so even if they're, More than a year out and they present with new onset heart failure and dilated cardiomyopathy, it can be progressive and go on to need um um advanced um heart failure therapies like V and transplant. I've definitely seen that before. Uh, so break. I'm gonna have a coffee, coffee sip. OK, so just a question to break it up a bit, cause I know you're tired of listening to me. So, previously healthy 3 year old girl is seen in the ED with a 5-day history of increasing shortness of breath, dyspnea with exertion, decreased appetite. She recently recovered from acute viral gastro. Chest radiograph shows normal lung fields, but cardiac size is mildly increased. Elevation of which of the following labs would be most specific of myocarditis. So I'll give people a minute or two here and you can. You can type it into the Q&A and I can read it for you if anybody wants to type their guess. Sure. Might take people a minute. Let's give them another moment. Sure, no worries. Uh, one person saying BNPA. See if anyone else tunes in, chimes in. Another A, B and P. I think that's it. OK. So good guess is, um, the answer is actually E. Um, so troponin INT are more frequently elevated in acute myocarditis, um, than, uh, CKMB, which is not as specific. And troponin has a specificity of like 86%, sensitivity of 71%. Um, so that's probably the most, um, sensitive marker of, uh, myocardial inflammation. Um, B Nancy P, um, I would say if it's severely elevated, that would for sure be more suggestive of something going on and such as myocarditis. Um But it can also be quite elevated for, in the setting of cardiomyopathies like dilated cardiomyopathy, um, whereas troponin is usually not elevated in, in cardiomyopathy, so the troponin can be pretty helpful and help in trying to distinguish between cardiomyopathy and myocarditis. And then also if it's um kind of in the acute phase, um, You could have myocardial inflammation, but you don't yet have, um, you know, like ventricular dilation or atrial dilation, which is really kind of what the BNP reflects. It's, um, basically dilation and stretch of the, of the heart. So troponin is the most specific and sensitive. So transitioning to myocarditis. So, it also is rare in children with an estimated incidence of 1 to 2 per 100,000 children. Um, in another report, myocarditis represented um 0.3% of about 14,000 patients seen over a 23 year period at Texas Children's. A higher incidence of myocarditis is noted in autopsy studies of infants and children with sudden death. Um, and myocarditis was noted in about 10 to 20% of such cases. Um, so if there's sudden death, uh, myocarditis is, uh, not an insignificant cause. And in a retrospective study from a single tertiary center, an estimated prevalence of myocarditis among children presenting to the ED was um half a case per 10,000 visits. Um, so again, rare but still things that, um, of course, should be included when the signs and symptoms suggested. Um, so just to give an overview, uh, myocarditis is an inflammatory disease of the myocardium, um, with different causes. Um, in pediatrics, it's mostly viral, um, or idiopathic. There's a a broad clinical spectrum of signs and symptoms, uh, ranging from subclinical to frank cardiogenic shock, arrhythmias, and sudden death, and the diagnosis is often made on clinical grounds, um, without a biopsy or, um, identification of a causative uh virus or other source. So this uh retrospective study looked at 29 patients, um, less than 18 who were diagnosed with myocarditis at a tertiary center from 2000 to 2018. Um, you can see that idiopathic is the most common, uh, quote unquote cause, about 21%. Um, I don't think of rhinovirus as actually being a cause of rhino of, um, myocarditis, but, um, they found that in this study. Um, other important causes, um, influenza, um, COVID, I think this was, yeah, pre-COVID, but, um, COVID can definitely do it. And then other ones to for sure think about, um, parvovirus, um, Coxsackie, um, Paraflu, and, um, HHV-6 is, is another one that. Is definitely seen in, was seen in other studies, but not so much this one. Um, and overall, about 3/4 of the patients recovered, but 10% of them needed ECMO, and, um, all of those that needed ECMO, um, likely had fulminant myocarditis and recovered. Um, and 10% were listed for transplant, and two of the patients died. Um, so with myocarditis, um, you can really see any, any EKG, to be honest. Um, any EKG abnormality can, can be a sign of myocarditis, um, but the ones to look for that are perhaps the most common are, um, ST and T wave abnormalities, um, so elevation and PR depression, and it can be diffused. It, it can also be in, um, uh, coronary distribution. Um, and then virtually any arrhythmia. And then also a normal EKG does not rule out myocarditis. So, um, EKG is an important part of the workup, but it, it alone is, uh, not sufficient to make the diagnosis of myocarditis. Um, so, and this is another, um, study, I think it is the same one actually, sorry, um, so influenza was associated with critical outcomes, um, and they found that younger age of presentation, females increased length of stay, um, lower presenting EF and other, uh, and higher levels of, uh, BNP were found to be significant predictors of critical outcomes, and again, nearly half of them presented with non-cardiac symptoms. Um, so another, just a plug for the flu shot, um, cause, uh, influenza can definitely cause myocarditis, and it, um, can lead to fulminant myocarditis where it's bad enough where you need eCMO. Um, I'll skip over this one. It had largely similar findings. Um, so the signs and symptoms of myocarditis again can range from, uh, mild, uh, things like chest pain to acute cardiogenic shock and heart failure. Uh, and again, uh, respiratory and GI symptoms and hepatomegaly are common. Uh, there can also be a gallop and signs of poor profusion, and then a viral prodrome, um, is seen in about 40% of cases. And we classify myocarditis as fulminant when patients become acutely ill after a distinct viral prodrome, and they present with severe um CV compromise, frequently requiring mechanical circulatory support. Um, but if patients with fulminant myocarditis are well supported, um, they do typically recover and are able to come off of ECMO, um, within days or a week or so. Um, and then acute myocarditis has less of a distinct onset of illness, um, and they're less likely to respond to therapies and in general have worse outcomes. Um, this table just shows that at baseline presentation, fulminant myocarditis patients are typically sicker, with 73% of them having, um, NYHA, which is, you know, equivalent to the ACC AHA staging system that I reviewed earlier. So 73% of them have stage 4. Um, which is slightly higher than, uh, acute myocarditis. Um, and then this Kalenmeyer curve, uh, from an adult study showed that fulminant myocarditis, um, can have, um, excellent long-term prognosis. This is 93% survival at 11 years out from presentation, um, and that's again if it's identified and if they get, um, appropriate, um, aggressive support during the initial acute illness. Um, so that's a little over double of that, of those who had acute myocarditis. Um, and then I, I guess this slide is supposed to be, uh, looks like a massive pleural effusion, probably some pulmonary edema in the setting of myocarditis. Looks like they got a PICC line, probably some Merone epi, and then got better. Um, here's a picture of, um, EKG with the classic findings of, uh, myopericarditis. So there's, um, PR depression and then, um, ST, pretty significant ST elevations, um, that you can see here. So PR depression and then, um, ST elevation. Um, echo findings of myocarditis are, uh, non-specific, but we typically see depressed LV function and dilation. Um, this can be mild or can be severe if it's fulminant. Um, there can be RV involvement, um, and then, um, Annoyingly or trick trickily, they can also have segmental wall motion abnormalities that can mimic an MI, um, so that, of course, makes us nervous, um, but we know that this, um, happens with myocarditis. So if, if you're a 60 year old or 80-year-old and show up with this myocarditis, you, of course, probably buy yourself a trip to the cath lab for a coronary angio, but in, um, kids and teenagers, we know that it's, um, can be seen with myocarditis. Um, and then similar to, um, cardiomyopathy, you can get, uh, mitral regurgitation and the setting of the ventricular dilation. Um, you can also get pericardial effusions, and then early in the course, you can also see, um, left ventricular hypertrophy, um, which is due to myocardial edema. And then that typically will um transition to more of a dilated um cardiomyopathy after the initial phase. Um, and then, Historically, biopsies were a kind of a big part of the diagnosis, and this is actually still considered the gold standard, if you will, um, but it's, it's rarely done in the US, um, this chart, I think is from 2011 and I, I can say the biopsies are even. I, I don't think I've actually, I didn't see a biopsy just for myocarditis. I don't think in my, in my training, so it's again a very rare thing to do. Um, and I think that's largely because MRI has, um, proven to be quite helpful in the diagnosis. Um, and then we look for, uh, myocardial, um, edema, um, hyperemia or scar, which is the Lake Louise criteria to diagnose myocarditis via MRI. Um, typical therapies for myocarditis include, um, some combination of IVIG and or steroids. There's quite a bit of variation. And then the other hallmark of treatment is supportive care, such as inotropes or um uh ECMO. Um, so getting close on time. I think I, I think I should be OK. Let me see how many slides I have left. Yeah, I think I should be good. Yeah, one second. OK. So now that I probably scared everyone into thinking that every child could have myocarditis, um, I'll transition into the clinical evaluation part of the talk. Um, so, like I mentioned, these are really are kind of needle in the haystack diagnoses and things that maybe you'll just see, you know, once or a few times in your career, um, but it's definitely something that you don't wanna miss, um, as it can lead to worse outcomes. So this paper looked at the frequency of missed heart failure, first presentation among kids with no known heart disease who were ultimately found to have new onset heart failure, and they used 4 criteria to be classified as missed, so. More than one heart failure symptom, um, or heart failure sign. Not mentioning heart failure and differential, um, is an important part of it. And then, um, those that had a workup or treatment, uh, who did not have a heart failure diagnosis within 24 hours. Um, so overall, half of the patients were missed on first presentation, and again, the most common wrong diagnoses were bacterial infection or sepsis, viral illness, or gastroenteritis or hepatitis. Um, so half of them had errors in history taking, half had no documentation of a differential. Unnecessary tests were performed in 10% of the patients, including CT scans of basically every part of the body, and others had unnecessary and invasive tests, including an LP and a liver biopsy. And there were other complications because of the incorrect diagnoses, with one patient having a cardiac arrest after they got intubated for a laparotomy. Um, one was started on dialysis, and then, um, two patients received, um, pretty dangerous amounts of IV fluids, um, prior to the diagnosis. Oh, sorry about that. Can you see my slides OK? Think so. OK. Um, so, OK. Um, so my take-home message, uh, for the evaluation of kids for heart failure is to number one, obtain an appropriate history and be wary of feeding intolerance, abdominal pain, vomiting, and respiratory symptoms, um, especially if there's no URI history or exposure history for gastro. And include a family history of um childhood heart conditions. Um, important to always, um, check vital signs, and if there's tachycardia and tachypnea, be suspicious of heart failure. And then again, on exam, uh, please evaluate for hepatomegaly, um, listen for a gallop, and then I think most importantly, just, um, include it in your differential, um, so add it to your smart phrases if you have them, so you at least think about it. Um, and then these tests here are all good first steps in evaluation that can be done in your clinics to help risk stratify. So, um, we'll, we'll go over all of them. Um, OK. So I think chest X-ray is a great initial screening test, and we specifically look for cardiomegaly and, uh, pulmonary congestion. Um, and I think it's important to note that the absence of these does not rule out heart failure, but of course, if you see them, um, they need to come see us. Um, so here's a chest X-ray of a 13 year old boy, cardiomegaly, um, pulmonary interstitial edema, um, who was ultimately found to have, um, dilated cardiomyopathy. Again, EKG is another important test. Um, like I mentioned, um, ruling out arrhythmias is an important first step. Um, and then. If you have time, uh, I would just Google a picture of Al Kappa EKG. That's another important thing to not miss. Um, again, that's the babies that show up in heart failure, and they have deep Q waves and then, um, STT wave changes in the left and lateral leads. Um, Initial lab workup, um, that, that you can start in your clinics, um, should include CBC, CMP, troponin if there's concern for myocarditis, uh, BNACP, uh, blood gas with lactate, um, is, is not a bad test, um, and then infectious workup, uh, thyroid function, if there's hypo or hyperthyroid, um, that can cause, um, cardiac issues. And then if they're adolescents, um, tox screen also. Um, just in the interest of time, I'll probably skip through some of these. Um, I'll just note that BNP would say is, is a very helpful test and for us in that it's almost always elevated if there's significant heart failure, and it's almost always negative, um, if there is no heart failure. If it's mildly elevated, um. May or may not be actual heart failure, uh, and then we also use it to trend severity of disease as well as response to therapies. And it can be falsely low in patients with a lot of adipose tissue. Um, echo, EKG, that's our side of things, and then I'll just spend a few minutes here talking about heart failure medications. Um, so there are 4 mainstays of therapy that, uh, together, are termed, uh, guideline-directed medical therapy or GDMT, and they all target different pathways that are dysregulated in heart failure. Um, so we use ACE inhibitors or um Entresto, which is um an RE angiotensin receptor neprolysin inhibitor. So these counter the up regulated RAS system that causes vasoconstriction and fluid retention. And then, um, Entresto inhibits the breakdown of BNP, which is actually a beneficial hormone that the body makes to cause some, uh, natrisis and diuresis as, as you get fluid overloaded. Uh, beta blockers target the upregulated sympathetic nervous system, uh, that can cause increased heart rate and contractility, which is more work for the heart to do, which we don't want. Um, spironolactone blocks aldasterone, which helps inhibit fluid retention, and then, uh, the newest agents are, um, sodium glucose link transporter 2 or SGLT2 inhibitors, such as, these are the, um, glyphosins, so dapogluhosin or ampaglihosin. Um, they were made as diabetes drugs. They cause glucosuria, um, which causes some diuresis. Which is part of the reason they help and then um they've just been, they've been shown to be very beneficial in adults and I think we still are um as a community trying to figure out why, but um importantly, they've um Decrease mortality and increased EF, um, which are good things, obviously. And then in general, we see patients frequently as we up titrate these medicines, so we start low, um, we start a lot of them at the same time, um, and then up titrate to goal dosing, um, or as high as tolerated based on things like blood pressure, heart rate, and kidney function. Um, I'll skip the Entresto one. So if you see our patients, they have a bunch of glucoseuria, you know that they're probably on dapagliflozin or ampaglifozin. Um, So just to summarize. Um, please always take a good history for, even for common problems such as gastro or bronchiolitis. Always keep heart failure on the differential, um, and I would just urge everyone to make the, you know, the heart failure exam part of your physical exam, particularly if the child is presenting with respiratory GI symptoms. Um, of course, look at your vital signs. Don't ignore tachypnia, tachycardia, particularly in a calm child at rest, and assess for hepatomegaly and gallops. Um, and then if there's known cardiomyopathy, abdominal pain is, is heart failure until proven otherwise, and in transplant patients, um, they have rejection until proven otherwise. Um, so I think that's all I have. Here's a list of some resources, um, and thank you guys for having me, and I hope this is useful. Created by Related Presenters Zachary Hutchinson MD pediatric cardiologist View full profile
