Pediatric cardiologist Othman Aljohani , MD, MPH, presents definitions of heart failure (HF) in infants and kids; describes risk factors and concerning symptoms by age group; and offers clinicians an evaluation process, including how to stage and classify pediatric HF. He discusses cardiomyopathy in depth (illustrating types with echocardiogram images) and warns that providers commonly miss certain kinds of HF, such as systolic. Learn which labs to order, what an ECG will show, and why HF leads to iron deficiency.
So, uh, today I'm gonna talk about cardiomyopathy and heart failure in, uh, children. These are the objectives of, uh, my talk. We're gonna define heart failure, identify risk factors, recognize different clinical presentations, and go through the initial workup and understand. Etiology of iron deficiency in children with heart failure and finally formulate um an approach to children with suspected heart failure. I have no financial disclosures. For the um board certification for the maintenance of certification exams and in training uh certification, cardiomyopathies and infection slash inflammation of the heart are one of the topics that are tested. And for pediatric emergency medicine, congestive heart failure, cardiomyopathy, and heart transplant are also tested there. We're gonna cover um um not everything, but a lot of these topics uh today. This is a great resource that um I uh use uh when it comes to education materials for patients and their families. It's through the Children's Cardiomyopathy Foundation. If you go through the website and you click on educational materials, you'll see like these um 1 to 2 pages of every cardiomyopathy like. Incidents, presentation, and outcomes, and I found them very helpful, um, um, uh, for, for families versus just going online and Googling topics and finding some um um inaccurate or um outdated information. Throughout this talk, we're gonna talk about um causes of heart failure um uh as classified into cardiomyopathies, cardiac toxicities, myocarditis, and congenital heart disease. Now, for congenital heart disease, majority of the patients are already uh followed and uh by a cardiologist who is treating their, their heart failure symptoms. So the focus is gonna be more on how to recognize um uh an undiagnosed child with congenital heart disease and uh what to look for uh when assessing, assessing them. If you look at 10 different resources, you will likely see 10 different definitions of heart failure. So one definition is a clinical syndrome that occurs when cardiac output is insufficient to meet the metabolic demand of the body. The second definition, as you can see, it's long. It's a failure of the heart to supply blood to either systemic 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. So for the sake of this talk, and what I, I, I like to tell um families and, and, and colleagues is, is heart failure is a syndrome of uh inability of the heart to meet the metabolic demand of the body due to systolic dysfunction, diastolic dysfunction, or both. And for the sake of this uh talk, we're gonna talk about heart failure as uh in regards to ventricular dysfunction or uh pump failure. The, um, the, the action, I, I have the slide about the action uh network, which stands for Advanced Cardiac Therapies Improving Outcomes Network. It's um a relatively new network that um comes up with guidelines and teaching materials for families and patients, um, um, uh, and children with advanced heart failure. To ensure we're speaking the same language, you'll hear the term ADHF or acute commercial heart failure, which is basically any heart failure that requires hospitalization and also the term. Advanced heart failure, which is any heart failure that's refractory to conventional medical therapies. Historically, we used to say triple therapy plus diuretics and triple therapy applies to um ACE inhibitors, beta, beta blockers, and spironolactone. And as you'll see uh later that now there are uh quadruple therapy plus diuretics when it comes to management of heart failure. Big, um, um, or an important step in evaluating these patients and to ensure that communication is, is, is, is clear, um, is using the staging system and the functional class when assisting patients with heart failure. So, uh, the American College of Cardiology, American Heart Association Heart Failure staging System helps us when we think about patients in regards to follow-up and management. So it has 4 stages, A through D. So let's say we have a patient who had, uh, was diagnosed with leukemia, received tetracycline, and they are asymptomatic and they have normal cardiac function. So those patients are um under stage A and they need follow-up. Let's say that same patient now, after a year, when they come for follow-up, their function on echo is decreased, and typically at stage B, we start an ACE inhibitor or Entresto or an Arni that we'll discuss later. They see now the patient, the same patient is symptomatic. And once they are symptomatic, typically, we optimize their ACE inhibitor or Entresto. And we add beta blocker, spironolactone. And a lot of times between stage B and C, we are doing the up titration before a patient becomes symptomatic. Stage D is a patient who is refractory or with refractory heart failure symptoms, despite optimizing their triple therapy or quadruple therapy, plus being on diuretics. And for stage D or advanced heart failure, those are the patients who might need Myronon infusion, and LVAD or lifted assist device implant, heart transplantation, or um referring them to hospice care. So most of the patients that you will see as an outpatient are likely gonna be uh stage A to to C. For the functional class, adults or adult providers use the NYHA that has um um 4 numbers, kind of like the way I remember it um with the letter NY is numerical, so 1234. So 1 is asymptomatic and 4 symptoms at rest. And then as you go, you make it worse. So 2 is mild. Uh, tachymnia with, with, with feeds or mild uh exercise intolerance. Uh, 3 is marked uh symptoms uh with, with an adult's activities and kids who typically talk about feeding. And for symptoms at rest. The nice thing about this classification that's um uh named a modified heart failure classification is it, um, it adds failure to thrive. So whenever we see a new patient or a follow-up patient who's coming for a follow-up visit, and they are um at uh or they have failure to thrive, then we know that they are at least NYHA equivalent or RSS class 3. Now it's important to, to know that it's very, very helpful to look at the growth chart, because a lot of patients with, or children with chronic heart failure, they would, you will see that their, their weight could be dropping, crossing percentile, percentiles. But there's also another pattern where they become Fluid overloaded, and they could be like going down and suddenly you see a big jump on their weight, that's related to fluid overload. And that's going to be consistent with them being wet or congested as a, as you'll see in a few slides. The presentation of children with heart failure is unlike in adults. Majority of patients develop GI symptoms or, or present with GI or respiratory symptoms. And this um this includes both patients who are um pre-heart transplant, so patient with heart failure. And patients who are in heart failure exacerbation and for post-transplant, if a patient is, for example, is in rejection, very likely they're going to develop these symptoms. So fluid overload to see in children, you can see it, but it's not as frequent as GI and respiratory symptoms, which makes it very challenging because patients Uh, to presenting to primary care, a lot of times they have these, these symptoms and later we'll discuss how to differentiate between, um, or at least make him differential for, for For these different conditions. Adults refer to this 2 by 2 table as the 2-minute assessment of heart failure. In pediatrics, it takes us way more than 2 minutes because sometimes the exam is, is challenging. The child is crying or is refusing exam. So the 2 by 2 table, uh, basically, you have warm versus cold, dry versus wet. For patients with established or non-heart failure diagnosis, we always want them to be in the warm and dry uh category. Uh, when, and when it comes to their physical exam, we want their liver to be normal or not uh far down in the right, in the right, um, uh, lower quadrant. And, um, when it comes to their lungs exam, they want, we want them to be clear. And if we are trending their weight, we want their weight to be at their dry weight. Or the cold category is a patient who is basically cold extremities, likely dizzy, and those are the patients who are likely gonna be admitted to the, to the hospital and they're gonna be in the CV ICU. It's important to note, to note that um um children in the wet category have worse outcomes. So in adults, most of the poor outcomes are related to the uh being cold versus uh being wet. But in kids, we know that um being wet is, is high risk. That's why if we have a patient who is congested, we would almost always would like to admit them, especially if they are not on diuretics, and if they're on diuretics, we want to diurese them while they're admitted where we can monitor their liver, kidney function, and their electrolytes. So in summary for the first part of the talk, heart failure can be simply defined as um A syndrome of inadequate cardiac output to meet the metabolic demand of the body due to systolic dysfunction or heart failure reduced EF diastolic dysfunction or heart failure preserved EF or both. Acute decompensated heart failure is any heart failure that requires hospitalization. The ACCHA classification or uh grading has um 4 stages And as per the Ross heart failure classification, failure to thrive makes a patient with heart failure at least class 3. OK. So, uh, for heart fail uh heart failure etiologies, um, as you know, there are a lot of, of, uh, etiologies that could lead to heart failure. And the goal of this talk to have an idea about how to do an evaluation, um, for a patient who with undiagnosed heart failure, and how to recognize if someone is in acute heart failure exacerbation. So first for etiologist for heart failure, we always talk about dilated cardiomyopathy, which is heart failure reduced ejection fraction. Um, hypertrophic cardiomyopathy or restrictive cardiomyopathy. Chemo-induced cardiomyopathy, um, typically we think of anthracyclin and postmyocarditis as about 1/3 or so of those patients can progress to dilated cardiomyopathy. Congenital heart disease, it depends on their um physiology, if they have single ventricles uh circulation versus biventricular circulation, and at what stage of their um repair they are. And other rare etiologies uh like cardiac tumors, uncontrolled arrhythmias, Kawasaki disease, metabolic causes, and retransplantation. So every child almost universally, um, who gets a first transplant eventually would require a second transplant. That could happen based on their risk factors after 20 years, it could be after 30 years, and also it could be after a few years post transplant. And this is part of the counseling we give to families when we talk about heart transplantation. Kawasaki disease is a rare cause for heart failure. Um, I've seen one, case in training, and I inquired through the Pediatric Heart Transplant Society to see how many cases, and this is the society that looks at all transplants in, in the US and there were, um, I believe less than 20 cases over a period of more than 20 years. The suspicions, uh, the way they develop heart failure, they develop coronary aneurysm, MI, and that's how they develop heart failure. It's rare. When we think of etiologies, we should, we always um think of the age of the child. Um, if it's in newborns and infants, we think of congenital heart disease and inborn errors of metabolism like fatty acid oxidation defects and mitochondrial disease uh disorders, um, uh, or uh atherectopic tachycardia that could happen in patients with, uh, with structurally normal heart. In older patients, we typically think of myocarditis and inherited uh cardiomyopathies. Um, disorders like SLE and acute traumatic fever are rare. But uh in, in, in North America. For clinical, uh, features, they, um, vary by the age of presentation and also the etiology. Um, the, and for the signs, um, we talked about the, the patterns that we can see on, uh, growth charts, uh, for vitals, um, one reference, um, describes it as the two tachys, which is tachycardia and tachypnea. And tachypnea is a sensitive sign for heart failure. And then here, as you can see here, some of the other things that we look at on physical exam. So for, for the, the physical examination for whenever I get a new referral um um or a patient who's admitted, I always try to have it focused. So, uh we talked about growth chart, we talked about vitals. So going in um Starting if you wanna start head to toe or starting from the hands and going to the head, then going down, have a systematic approach that you use all the time. So for hands, I always start with uh perfusion, check pulses, if you're gonna check radio, radio, radio femoral, then going to The head, look at dysmorphic features. Uh, when you get to the, uh, neck, um, uh, distended, uh, jugular, um, um, or JVD is very rare, uh, and difficult to see in kids, but whenever I get to the neck part of the physical exam, I always count the respiratory rate, then getting to the heart, murmur, no murmur. Um, gallop, no gallop. And something to, um, to, to keep in mind that gallop could be intermittent. So, uh, please give yourself like decent 1015 seconds when listening for a gallop. And then listening to the lungs, then going to the abdominal exam, hepatomegaly or no hepatomegaly. And if you feel it, sometimes you need to go all the way down to the right lower quadrant and then lower extremity edema, no, no edema, and um the pulses. And based on the assessment, you can uh decide where to put the patient if they are in the dry, warm versus um With warm or in the cold category. Um, And Um One of my colleagues asked me why do patients with heart failure develop iron deficiency. And if you know, if you note, I wrote iron deficiency and not iron deficiency anemia. So universally, all patients with heart failure will develop iron deficiency. And there are um many mechanisms that were described from anorexia or decreased intake, or decreased absorption because of intestinal edema. Or decreased losses because of GI bleeding uh due to uh poor gut perfusion, uh, or if they are on anticoagulants or on aspirin that they might have some GI bleeding. The other mechanism that is uh very well studied in adults. And it explains why patients with chronic inflammatory conditions can develop iron deficiency and also why obese patients can develop iron deficiency. For example, heart failure is a chronic inflammatory condition that activates all of these cytokines. These cytokines activate hepcitin, and hepcitin works at two levels. One, it inhibits the absorption of iron at the level of the intestine. And 2, it inhibits the release from the storage pool. So you have a, a, a state of um functional iron deficiency. And this is why in the, in adults, um, the guidelines are to give IV iron uh to replace uh or to correct iron deficiency. And in pediatrics, there are about 10 publications or so using IV iron if patients are iron deficient. So next, we're gonna talk about cardiomyopathies and we talked about some, some of, of, of, of them. Um, so first, this picture, it shows you and I'm gonna play a few videos uh later. This is a um a normal heart, so right atrium, right ventricle, left atrium, left ventricle as compared to the heart on the right side with, for a patient with hypertrophic cardiomyopathy where the ventricular septum is very thick. If you look down here, let's start with the DCM1 or the cardiomyopathy, where you see that the ventricle is dilated. As, and the last one I want to show is restrictive cardiomyopathy where the atria are hugely um enlarged. So let's start with dilated cardiomyopathy. Since it's the most common form of cardiomyopathy in children, about 50 to 60% of all cardiomyopathies, about 6 cases per 1 million um of children. Um, it's characterized by LV dilation and dysfunction, which is the most common presentation. Sometimes you have biventricular dysfunction. Myocarditis is the most common acquired cause of dilated cardiomyopathy. About 1/3 of patients with dilated cardiomyopathy um would require advanced cardiac therapies. And these two echoes are um for patients with dilated cardiomyopathy who ended up um placed on ventricular access devices and um needing heart transplantation. Both were infants. So you see a right atrium, right ventricle, left atrium, left ventricle, and you see how the blood is swirling in the left ventricle because it's not squeezing well. And you see, you can also see it here. There are multiple etiologies for, for dilated cardiomyopathy and it's all, um, we always look at the age or we're talking about a newborn versus infant versus someone who is older and typically, once we, the patients are referred to a cardiologist, we always get a full uh echocardiogram to look at all, uh, to rule out all of these um conditions. We'll talk about the initial workup, uh, um, in a few slides. I'm sorry. Second cardiomyopathy is hypertrophic cardiomyopathy, which is the 2nd most common. It's about 30 to 50%, which is about 5 children per 1 million. Um, and it's characterized by abnormal growth of muscle fibers. And um hypertrophic cardiomyopathy can be obstructive versus non-obstructive. Obstructive, you'll hear the term hokum or hypertrophic obstructive cardiomyopathy. This is an echo of a patient who had an um uh known as um disease and as you can hear um or syndrome and as you can see here, the ventricular walls are very um hypertrophied. And the outcomes are variable. But, uh, click. Third is restrictive cardiomyopathy, which is the rarest, rarest form, uh, less than 1 per 1 million. And um at least um in the past 4 to 5 years, we had 2 patients with restrictive cardiomyopathy. One is transplanted and the other one is listed for heart transplant. Unfortunately, with restrictive cardiomyopathy, there are no um Um, medical options. The patient is symptomatic, they need to be listed for heart transplantation. The classic textbook description for us, uh, in cardiology, which is a broad question, is Mickey Mouse ears. So you see the atria are, um, um, extremely enlarged when I play this video. So almost the left atrium as the size of the rest of the heart. And the issue with restrictive cardiomyopathy, if you can think of the heart muscle as a very stiff rubber band that does not relax and over time, the atria become dilated. And this is comparing the three, the dilated, the hypertrophic. And the restrictive So the next topic we're gonna discuss is uh cardiac toxicity. So, um, and as you know, majority of the chemotherapy includes, um, anthraccycline, um, related, um, products and or medications. And those medications can lead to myocyte damage and replacement by fibrosis. Um, we always talk about the cumulative dose. Is it more or less than 300 mg per meter square? Because this is the number where we almost always see cardiac toxicity if it's above this number. But it's very important to know a few things. One, cardiac toxicity could happen at any time and after any dose of exposure. There are reports that cardiac toxicity could happen after getting exposed to 45 mg per meter square of anthracycline. Two, anthracycline toxicity could happen. In a week, in a month, in a year, in 10 years, in 20 years, those patients should always have follow up with a cardiologist, even if their cardiology follow up for 5 years or 10 years is normal. They should not be discharged from cardiology, and they should always have cardiology follow up. And um as compared to radiation, uh, typically the damage is, is uh basically specific diffuse uh and interstitial fibrosis that could also affect the conduct the um cardiac conduction system. I use this table for almost all of the consults uh we get um from oncology, like if you say, if you have a patient who just received um uh anthracyclin and then if it's acute, which should, which could happen within the first week and typically by discontinuation. Therapy and addressing fluid overload and hypertension, it typically um reversible versus early uh and late onset progression that could happen within the year or after a year post-chemotherapy. And that can be, can be uh progressive. And those patients typically develop uh in children, a mixed picture of restrictive and dilated cardiomyopathy. And I'm back. So, And so the birth question, um, previously healthy 3 year old, uh, girl is seen in the emergency department with a 5-day history of increasing shortness of breath, dyspnea with exertion, decreased appetite, recently recovered from acute viral gastroenteritis, chest X-ray with normal lung fields, but cardiac size is mildly increased. Um, elevation of which of the following labs would be most specific for the diagnosis of myocarditis. And the answer is Troponin. Um, it's important to know that um the question is about more specific and ask about myocarditis. And as you can see here, troponin are more frequently elevated in acute myocarditis than CKMB. Whenever we suspect uh myocarditis, we rarely check for CKMB in, in kids. And it's important to note, whatever troponin you use or you are trending after discharge, you should be using the same one. If it's high sensitivity or regular troponin, if it's troponin I or troponin T. Sometimes we have a patient with myocarditis who was admitted. They get discharged and with, with near normal but still mildly elevated troponin, they go to a different lab and they have high sensitivity troponin and then they, they're found. We find it to be 30 or 40 and everyone freaks out and then we find that the normal range at that lab for that say is less than 40 or less than 50. So it's important to look at the reference range. Uh, takes us, uh, this question to the topic of myocarditis. First, myocrodactys is extremely rare. It's about 1 visit every 20,000 visits to the emergency room. And I think this is very important to to know. And it's important when we have patient with, let's say myocarditis, who presents after a couple of visits and the parents are frustrated or mad because, yeah, the child was doing, wasn't doing well, and why was dismissed? Because it's very rare. And unless if you think about it, and you do workup for it. Likely, um, you're gonna miss it. We're gonna talk about an approach later. Um, Uh, most of the microditis cases in North America are viral microditis. And, um, um, for the past couple of years, uh, we've seen uh several cases of COVID microditis, like COVID infection microditis, which is a very different entity than COVID vaccine-related microditis, which is, um, um, Almost, uh, nearly recoverable for all in all patients that we've seen here. Also, microditis and other reports from Texas Children's Hospital that was about 0.3% of about 14,000 patients. It's also related and uh or reported in cases of sudden, sudden death involves inflammation of the heart, myocardium. Sign and symptoms can vary and um as mentioned, viral myocarditis is the most common cause um in North America. This is a study um did uh um. Um, we did Children's in San Diego where I did my training. This was pre-COVID where we looked at myocarditis etiologies over a period of, um, 18 years. And as you can see, there are different viruses that were, um, that, uh, were positive and majority of the patients, about 70% recovered. Few were supported with ECMO. Few were listed for transplant, and unfortunately, two patients. Um, uh, passed away. Um, EKG findings were, uh, non-specific, but majority of the patients had ST segment and T wave abnormalities and age distribution. They were common during the first few years of life and during Um, uh, 10 years. But we found that which is consistent with the bigger study from the Stanford Group that um, Uh, majority of the patients did not present with cardiovascular symptoms, which is very important to, to think about when you're suspecting, um, microarditis. And also, we noted that, um, and for that study that influenza A and B was associated with critical outcomes like VA ECMO listing for transplantation and death. And recently, uh, or early this year, we, um, we have seen several cases of, of, um, low myocarditis. Um, and we had one patient who had presented in fulminant myocarditis who was supported on ECMO. Unluckily, um, recovered, uh, completely, and in retrospect, the patient was, um, scheduled for a flu vaccine, but because of a little bit of runny nose around that time, um, the, the decision was made to defer it until a month later or so, and, um, the patient had the infection. So it's very important, um, as you know, the, the flu, uh giving flu vaccine is, is important because flu infection can be life-threatening. Even in healthy individuals. So this is an older study that looked at outcomes um for patients with myocarditis also as mentioned, we have bimodal distribution for the age and um less than 10% present uh required mechanical circuitory support and um about 5% required um heart transplantation. Different presentations including chest pain, respiratory symptoms, and GI symptoms, and about 40% presented with a viral symptoms. It's important to um To differentiate between fulminant myocarditis and acute myocarditis, so fulminant myocarditis is basically a patient presenting in Acute cardiogenic shock. And it's the, it's important because as you can see in this table for patients with fulminant myocarditis, um, almost all of them had flu-like illness. Majority of them had fever. Um, it was very acute, and they had a higher functional class, um, uh, in regards to heart failure symptoms as compared to acute myocarditis. And this is important because when we look at their outcomes, actually fulminant myocarditis has better prognosis as long as the patient is referred. To a center that is going to be um placing them on mechanical circuitry support. And their, their survival is based on them surviving mechanical circulatory support or ECMO um um. Um Uh therapy, uh, without major complications. As compared to acute microarditis, and those patients, um, the, the, the rationale behind this is acute microarditis patients, a lot of them could convert to dilated cardiomyopathy and progress to advanced heart failure. X-rays uh could be normal in myocarditis as you can see here in regards to the lung, um, to the presence of pulmonary edema. There's some, uh, cardiac, um, or left atrial enlargement here. Um, so, um, there's no one test that could rule in or rule out, um, acute myocarditis. So physical history, physical exam, and then talk about X-ray. An EKG with myo pericarditis, we, we can CPR depression and ST segment, diffuse ST segment elevation. And the echo is typically non-specific and typically look at function effusion and um uh wanna rule out any um undiagnosed congenital heart disease. When we look at the, the therapies or the workup for these patients, a lot of um um cardiac biopsies um are not commonly done since they are replaced by cardiac MRI. The advantage of the cardiac MRI that could characterize um um cardiac muscle edema and scarring. And as far for therapies, if you look at majorities of, of the, of the centers in, in North America, uh, IVIG is about 50% or so. And typically, if a patient is having a significant troponin leak, a segment elevation, then they will likely get an IV, we will get an infusion of IVIG. It's important to know that if their function is severely decreased, you wanna be cautious about uh volume overload. Um, steroids not commonly used, um, um, for treatment of myocarditis. And these, these therapies are um for viral macroditis or this study was from the pre-COVID um era. Uh, when it comes to COVID, we always consult um a rheumatology, ID and make a decision about steroids, IVIG and other therapies if they are indicated. So since this is a very rare condition and um someone asked a question about misdiagnosis of new onset systolic heart failure in children without known heart disease. So it was a study that involved um patients less than 21 years of age. They um use four criteria to be classified as missed one, the presence of one or more heart failure symptom, one or more heart failure sign. No mention of heart failure in the differential. Um, uh, diagnosis workup, um, and no heart failure diagnosis within 24 hours prior to the transfer to the center. They found that up to 50% of those patients were missed from the first presentation and most common wrong diagnosis there were bacterial infections, viral infections, and gastroenteritis. They also found about 50% um uh errors in history taking. And um here are some of the things that, that they noted, and I think this is one of the most important ones. Like one patient had a liver biopsy, one patient had an LP, 2 patients had EGDs, and one patient had a pH probe studied, and complications from um Related to, to inaccurate diagnosis include cardiac arrest after intubation for a planned laparotomy. Um, one patient was placed on dialysis for renal failure, while the renal failure was secondary to low cardiac output. One patient underwent liver biopsy and two patients received, um, significant fluid, um, uh, resuscitation. And the take-home message of this, uh, since it's rare, is, uh, for new onset heart failure, it's uncommon syndromes, so to obtain correct history of symptoms including GI and respiratory, family history of cardiomyopathy or congenital heart disease, um, check for vital signs, the two tachy that we mentioned, listen for gallop and feel the liver edge and, um, then, uh, Policing of heart failure and the differential, and last, not least, consider chest X-ray, BNP and um ECG. We know that in primary care, um, it's very hard to get labs and, and studies, at least where I did residency, it was very hard. We almost always had to send patients to the emergency room where they get All of the um workup and then if something turns to be positive, then they get diagnosed with, with heart failure. And then take another 12th break. We're gonna talk about the last part of the presentation. Maria, how are you doing with time? Good, it's about 1:12. We have a couple of questions already in the queue, so however much longer you think. OK. So I'm gonna go through the um Uh, the rest of the slides and we'll answer the questions. So I think for the history part, it's important to, to think of is this an acute process? Was there any viral infection if associated for thinking of myocarditis? Um, any UI symptoms in the past couple of weeks or past couple of months? When was the last time the patient was at their baseline and And what were the new symptoms. The physical exam, um, sorry, we're going back to the history. Family history, I always ask any family history of cardiomyopathy and if the parents say, what is that? It's like, I say, heart muscle weakness. Anyone in the family um underwent heart transplant evaluation? Any sudden deaths in um um younger um individuals without a reason? And just trying to get a, a story or a detailed family history. The physical exam we covered and the workup, we covered most of it. So for the X-ray as mentioned, you might see pulmonary edema or not. Likely you're gonna see cardiomegaly. Uh, sometimes if it's borderline, then you need to likely investigate more. This is a patient with pulmonary edema and cardiomegaly. The EKG as mentioned, um, or, and this is now we're talking about workup for heart failure in general. Um, sinus tachycardia is the most common sign, and you can see a lot of EKG abnormalities. For the pediatric boards, um, they like to ask this question. So, ACAPA, which stands for anomalous left coronary artery from the pulmonary artery, that could lead to ischemia, it's typically the scenario, an infant who is 1 or 2 months um of age, who is very irritable, um, Um, and not tolerating feeds, check pulses, they are normal. Get an EKG that has key wave and inferior lateral leads, and the diagnosis is Alkappa. Um, they like to ask this other name of the syndrome which is bland-Y Garland syndrome, which, um, um, I don't think in cardiology, um, providers mentioned it. They always say Alkappa or not. These are some other EKG findings with different, uh, conditions. For labs, CBC is helpful to rule out anemia. We had a patient with a hemoglobin of 2 with severe heart failure a few months ago, uh, by correcting uh anemia, heart failure improved. Um, CMP to look at liver and kidney function. Um, blood gas lactate, this is typically when a patient is in the ED and if they, if there are concerns for cardiogenic shock. Troponin is helpful if you are suspecting myocarditis or coronary ischemia. We always get thyroid function in all new diagnosis, um, uh, or new referrals of heart failure, urine tox in, in, um, uh, teenagers, and we always get a COVID testing and RVP. BNP, let's say if you're in the, in, in the, in, in the remote area, the referral to cardiology is gonna take 2 months, and you can send a patient to Quest to get a BNP. I think that is great because if BNP is low, it's very likely um um You, you, it, it can be very reassuring because the majority of patients with heart failure will have elevated BNP. Um, and it's important to know that BNP can be low in obese patients because it gets metabolized, the BNP get, gets metabolized by adipose tissue. Echo and other tests, typically, cardiology is gonna be involved at this stage. So I'm gonna spend um a minute talking about um medications. Historically, we always talked about triple therapy, which is an ACE inhibitor, a beta-blocker, and spironolactone. The new um uh or the updated therapies include Arni or Entresto, beta-blocker, spironolactone, and SGL-2 inhibitors, which is a class of medication that initially was approved for type 2 diabetes and was found to have Um, uh, morbidity, uh, mortality benefits in patients with heart failure. Entresto, um, you will likely see a lot of heart failure patients on Entresto. It has two components, valsartan, which is an ARP, and saccupitril. So with heart failure, you have ventricle wall stretch, BPNB gets released, breaks down to the NTPRBNP and the BNP. The BNP normally would lead to natiuresis, which is loss of sodium, diuresis, and vasodilation. And giving Entresto that inhibits this enzyme, basically you'll have a higher level of BNP. The reason why the guidelines state that if you have a patient with on Entresto, you should be following NT pro BNP. So for any new consults, we always check both at baseline. If you see this for a patient who is on an SGLT2 inhibitor, this is the expected um um UA. They will have significant amount of glucose. As long as they have no ketones, they should be continuing on the medication. So this class of medication basically promotes diuresis uh through glucose urea and UTI has been described up to 15% in children. Um, the FDA has a recommendation of stopping it, um, about 3 days before any major procedure. And that's always a discussion, uh, when we have a patient going through any procedure with our cardiac anesthesia because there is risk of ketoacidosis. The risk of the conditions um for hypertrophic cardiomyopathy since their ventricular wall is very thick, we always say keep the heart full and slow. There's a new class of medication that's under a clinical trials in pediatrics. For restrictive cardiomyopathy, we always talk about if they're symptomatic, they need to be listed for transplant. And for myocarditis, we talk about IVIG and we have discussed that. I think we can open the floor for questions.
