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 have no financial disclosures. For the um birth certification for the maintenance of certification exams and and training uh certification, cardiomyopathies and infections 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 um materials for um patients and their families 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helpful um um uh for, for families versus just going online and Googling topics and finding some um um inaccurate or um outdated information. Trout this talk, we're gonna talk about um causes of heart failure um as classified into cardiomyopathies, cardiac toxicities, myocarditis, and congenital heart disease. Now for congenital heart disease, the 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 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 lung, 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 this slide about the action uh network which stands for Advanced cardiac therapy's 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 um in children with advanced heart failure. To ensure we're speaking the same language, you'll hear the term ADHF or cued 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 sinnolactone, and as you'll see uh later that now there are a 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 on the functional class when assisting patients with heart failure. So at 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, receivedanttracycline, and they are asymptomatic and they have normal cardiac function. So those patients are um under stage A and they need to follow up. Let's say that same patient now after a year when they come for a follow-up, their function on echo is decreased and typically at stage B we start an ACE inhibitor or entreto or an RE that we'll discuss later. Stage C now the patient, the same patient is symptomatic, and once they are symptomatic, typically we optimize their ACE inhibitor or intresto and we add a beta blocker, sinnolactone, and a lot of times between stage B and C we are doing the obitration 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 munal infusion and LVAD or lifted the 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 a stage um A22 C. For the functional class, adults or adult providers use the NYHA that has um um for 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, tachynia with, with, with feeds or mild exercise intolerance. Uh, 3 is marked, uh, symptoms uh with, with an adult's activities and kids we typically talk about feeding. And for symptoms at rest. The nice thing about this classification that's um uh named a modified ro heart failure classification is it um it adds failure to thrive. So whenever we see a new patient or a followup 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 ROSS 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 an. Another pattern where they become fluid overloaded and they could be like going down and suddenly you see a big jump um on their weight that's related to fluid overload, and that's gonna be consistent with them being wet or congested at as a, as you will 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 transplants, so patients 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 gonna develop these symptoms. Uh, so fluid overload to see in, 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. Uh, to present it 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 right 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 CVICU. It's important to not, to know that um um children in the weight category have worse outcomes. So in adults, most of the uh poor outcomes are related to the uh being cold versus um 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 um congested, we would uh almost always would like to admit them, especially if they are not on diuretics, and if they're on diuretics, we want to diaries them while they're admitted, uh, 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 reduce 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 for the Ross heart failure classification, failure to thrive makes a patient with heart failure at least class 3. OK. So, uh, for heart uh heart failure etiologies, um, as you know, there are a lot of, of etiologies that could lead to heart failure, and the goal of this talk is to have an idea about how to do an evaluation um for a patient to 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 reduces infection. Um, hypertrophic cardiomyopathy or restrictive cardiomyopathy. Chemo-induced cardiomyopathy, um, typically we think of anthraccycline and postmyocarditis as about 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 by ventricular circulation and at what stage of their um repair they are, and other rare etiologies uh like cardiac tumors, uncontrolled arrhythmias, Kawasaki disease, metabolic causesis, 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. 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. those patients, 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 a newborns and infants, we think of congenital heart disease and important errors of metabolism like fatty acid oxidation defects and mitochondrial disease uh disorders um uh or uh a ectopic 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 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, for reference, um, describes it as the two tachys which is tachycardia and tachypnea, and tachypneia is a sensitive sign for heart failure. And then, 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 is 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, I'm gonna check radio radio radiofemoral, then go into 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 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, hepatomly or no hepatomygaly. 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 pulses. And based on the assessment, you can uh decide where to put the patient if they are in the dry worm 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 know 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 decrease intake or decrease 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 an aspirin that they might have some jet bleeding. The other mechanism that is um 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 hepidin and hepidin 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 get 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 cardiomyopathy 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 or the ventricular septum is very thick. If you look down here, let's start with the DCM one or the red cardiomyopathy where you see that the ventricle is dilated. As and the last one I wanna show is they have 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, 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 ventte access devices and um needing heart transplantation. Both were infants. So you see 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 delicated cardiomyopathy and it's all um we always look at the age where 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. Is sorry. The Second cardiomyopathy is hypertrophic cardiomyopathy, which is the second most common. It's about 30 to 50%, which is about 5 children per 1 million, um, and its characterized by abnormal growth of muscle fibers and um hypertrophic cardiomyopathy can be obstructive versus non-obstructive. Obstructive, you'll hear the term or hypertrophic obstructive cardiomyopathy. This is an echo of a patient who had an um uh known as um season 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 rare rarest form, uh, less than 1 per 1 million and um at least um in the past 4 to 5 years, we had two patients with restrictive cardiomyopathy. One is transplanted and the other one is listed for a 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 of 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 with the hypertrophic. And the restricted 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, uh, 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. Um, after getting exposed to a 45 mg per meter square of anthraccycline, 2, anthraccycline toxicity could happen. In a week, in a month, in a year, and 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 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 you say if you have a patient who just received um anraccycline and then if it's acute, which could happen within the first week and typically by this continuation of Therapy and addressing fluid overload and hypertension. It typically um reversible versus early and late onset progression that could happen within the year or after your post chemotherapy and that can be, can be uh progressive. And those patients typically develop in children a mixed picture of restrictive and dilated cardiomyopathy. And I'm back. So, And so with that word question. Um, previously healthy 3 year old 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 gastroentritis, 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 are rarely checked 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 you have a patient with myocarditis who is admitted, they get discharged and 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 found it to be 30 or 40 and everyone freaks out and then we find that the normal range at that lab for that essay 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, myocarddacty is extremely rare. It's about One 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 patients 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 work up for it. Likely, um, you're gonna miss it. We're gonna talk about an approach later, um. Uh, most of the microdis cases in North America are viral microiditis, and, um, um, for the past couple of years, uh, we've seen several cases of COVID micarditis, like COVID infection microitis, which is a very different entity than COVID vaccine related myocarditis which is um um. Almost, uh, nearly recoverable for all and all patients that we've seen here. Also microds and other reports from Texas Children's Hospital that was about 0.3% of about 14,000 patients. It's also related um uh or reported in cases of sudden, sudden death involves inflammation of the heart myocardium, sinus symptoms can vary and um as mentioned, viroomyocarditis is the most common cause, um, in North America. This is a study um did at um. Um, really 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 um were positive and majority of the patients, about 70% recovered. You were supported with ECMO, you were listed for transplant and unfortunately to patients. Um, uh, passed away. Um, EKG findings were, uh, non-specific, but majority of the patients had ST segment and TA abnormalities and age distribution. They were common during the first few years of life and during, um, uh, teen years. But we found that which is consistent with a 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, microiditis. And also, we noted that, um, and for that study that influenza A and B was associated uh with critical outcomes like VAECO 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 foment myocarditis who was supported on ECMO and luckily, 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 circuatory 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 microitis and acute myocarditis. So fulminant microditis 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 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 gonna be um placing them on mechanical circuitory 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 microiditis, and those patients, um, the, the rationale behind this is acute microiditis patients, a lot of them could convert to delicate 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, um, cardiac, um, or atrial enlargement here, um, so, um, there is 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 weaken 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 we 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, who will get an infusion of IVIG. It's important to know that if their function is severely decreased, you want to be cautious about um volume overload. Um, steroids, not commonly used, um, um, for treatment of myocarditis. This, these therapies are um for viralyroiditis 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 that 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 and children without uh known heart disease. So it was a study that involved um patients less than 21 years of age. They um used 4 criteria to be classified as missed on the presence of one or more heart failure symptoms, 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 gastroenteritiss. 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 pro study and complications from um. Related to to inaccurate diagnosis include cardiac arrest after intubation for a planned rotomy. 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 is uncommon syndrome, so to obtain correct history of symptoms including GI and respiratory, family history of cardiomyopathy or congenital heart disease, um, check for vital signs, tachys that we mentioned, listen for gallop and feel the liver edge and um think uh. All think of heart failure in the differential and last not least, considered 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 gonna talk about the last part of the presentation. Maria, how are you doing with time? Good, it's about 112. 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 you associated for thinking of myocarditis, um, any URI 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, going back to the history. Family history, I always ask any family history of cardiomyopathy and if the parents say, what is that is that I say heart muscle weakness. Any anyone in the family um underwent heart transplant evaluation, any sudden deaths in 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 World Cup 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 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. But the pediatric wards, um, they like to ask this question. So Alcapa, which stands for an 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 Q wave and inferior lateral leads and the diagnosis is Alkapa. Um, they like to ask this other name of the syndrome which is bland white Garland syndrome, which, um, um, I don't think in cardiology, um, providers mention it. They always say alcaba or not. These are some other EKG findings with different, um, conditions. For labs, CBC is helpful to rule out anemia. That 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 all 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 cardiologist is gonna take 2 months and you can send a patient to West to get a BNP. I think that is great because if BNP is low, it's very likely, um, um, it, it can be very reassuring because the majority of patients with heart failure will have elevated BNP. Um, uh, 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 sinnolactone. The new um uh or the updated therapies include Arni or intresto, beta-blocker, sinnolactone, and SGL2 inhibitors, which is a class of medication that initially was approved for type 2 diabetes and was found to have Um, uh, morbidity, and 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 falsartan, which is on ARP and succupitril. So with heart failure, you have to take a wall stretch, BNB gets released, breaks down to the antiprobNP and the BNP. The BNP normally would lead to na sis, 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 inresto, you should be following NTro 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 a 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 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 the 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.
