All right, here we go. We have a two-year-old boy who presented three weeks ago with meningococcemia who continues to be intubated and mechanically ventilated and is receiving a combination of enteral feeds and parenteral nutrition. His extremities are cool. Either his blood pressure nor his peripheral perfusion respond to the fluid resuscitation and he requires catecholamine infusions for blood pressure support. Cultures are obtained and he has started on broad-spectrum antibiotics. He has previously required arterial catheterization for blood pressure monitoring and neither radial pulse is palpable. He also has multiple areas of eschar on his lower extremities, suggestive of multiple areas of prior immunization, likely from his meningococcemia. Which of the following arteries is most appropriate for catheterization? Brachial, ulnar, posterior tibial, axillary, or temporal? Okay. I think we have a clear winner. And it looks like almost a little over two-thirds selected axillary artery. That is the correct answer. In this particular patient, the radial arteries are not available. And so you probably don't want to go to the ulnar circulation because then you don't have any collateral circulation. Brachial artery is generally not an accessible artery for catheterization because of its lack of collateral blood supply around that. And so you can get distal perfusion abnormalities pretty easily. Temporal artery would be an unusual place to put a catheter, but no one went for that one. And the posterior tibial artery would normally be a good choice because there is collateral circulation with the dorsalis pedis. But in this case, those were not accessible based on his multiple areas of eschar. So I think they're leading you towards axillary artery, which is a good choice. There's a strong collateral circulation around the axillary artery. And so that is a good central arterial option and would be the best choice for this patient. So good job. Okay. I'm up again here. All right. Here we have a 10-month-old boy with respiratory failure secondary to left-sided pneumonia with moderate pleural fusion being prepared for intubation. A chest tube has been placed and 120 mLs of foul-smelling drainage has been evacuated. He has a sandpaper rash on his chest and the rest of his skin has a sunburned appearance with a cap refill of four seconds. Vital signs are heart rate 175, blood pressure 71 over 35, 88% on high-flow nasal cannula, 15 liters at FO2 0.9. Which of the following combination sequences for induction of intubation is most appropriate? Okay, very good, so it looks like the majority selected atropine and ketamine, and that is indeed the right choice. Did not cover this specifically in the sepsis lecture earlier, but there are several studies that have shown an association with, and there are a couple of randomized control trials in adults that suggest that etomidate may actually be associated with worse outcomes. It's not definitive, but there's enough information that at least the surviving sepsis campaign suggested at this point to stay away from etomidate for intubation with sepsis. And instead, ketamine would be a nice hemodynamically neutral option in this patient, and is the drug that is generally recommended. Fentanyl would be another reasonable choice. The combination with midazolam, though, is going to make you more susceptible to develop hypotension, and so maybe not the best choice for a patient already in shock, and for those same reasons, propofol would be something to stay away from. Pentobarb is probably not a great induction agent for intubation, and nobody went with that one, so that's good. The use of atropine was not a factor in this decision, because all of the selections had that. I think they were referring to the young age. It's a bit of an outdated recommendation now to use atropine preemptively, although some people still do. I do not, personally, in my practice, although I have it available and drawn up for the younger patients, but I don't automatically give it in this scenario. But ketamine would be the major factor here for the intubation induction agent of choice in sepsis. Next is me, I think. Yeah. Okay, this is the next question. A six-year-old, 17.3-kilogram girl who was born with severe mitral regurgitation underwent plication of the clufts and aneuplasty four years ago. After surgery, her mitral regurgitation has improved from severe to mild. About two weeks ago, she had left hip pain, decreased mobility, bluish discoloration of toes, and painful swelling of her fingertips. Three days ago, she developed high fevers, 38.8, nausea, vomiting, fatigue, loss of appetite. Her mother brought her to the emergency room where blood cultures were obtained. An echocardiogram now shows severe mitral regurgitation, but with normal systolic function and two mobile echocardiographic masses on the mitral valve. So she's diagnosed with acute bacterial endocarditis. The blood cultures are sent, but we don't have the results yet, and it's transferred to the PICU. Which of the following empiric antibiotics should be started? So it's IV penicillin, IV ampicillin, sublactam, and IV gentamicin, vancomycin, and rifampin, IV ceftriaxone, ampicillin, gentamicin, and vancomycin combinations. So more than half got this right. If you don't have a positive blood culture to treat, then you're going to start with really broad-spectrum antibiotics, ampicillin, gentamicin, and vancomycin is a recommended combination. If you have a prosthetic valve, then I think slightly different. And so I think the answer really is unicin, gentamicin, and vancomycin. Do you guys want to make any comments? Okay, the next one is mine as well. So this is a 12-year-old boy who collapsed after receiving a blow to the chest while playing baseball. Bystander chest compressions were started immediately. The EKG shows the rhythm here. And which of the following is the next most appropriate step in his resuscitation? Synchronized cardioversion, one joule per kilogram. Defibrillation, two joules per kilogram. Epinephrine, vasopressin, and magnesium. Thank you. Okay, this is pretty straightforward. Ventricular fibrillation, so early defibrillation, is the treatment of choice. Can you guys tell me what the condition is? Comotia cordis. Comotia cordis, yeah. It is so rare. I have no idea why we chose. Comotia cordis is a way of demonstrating ventricular fibrillation, so there you go. It happened on live TV. Yeah. It's really rare, but it has to be the perfect ball, the perfect speed, and the perfect timing with your EKG to actually cause comotia cordis. Let's see. All right, questions on that? Okay, next one. And this is the PALS algorithm, shock, shock, epishock, a mirror. Okay. So the next one is mine as well. A previously healthy eight-year-old boy in the pediatric ICU was diagnosed with myocarditis, myomyocarditis, complicated by cardiogenic shock, requiring intubation, mechanical ventilation, and inotropic support. Echocardiogram shows severely depressed biventricular systolic function with an LV ejection fraction of 22%. After four days of supportive care and gradual diuresis, inotrope has been weaned off, ventilator pressures are minimized, and a decision is made to extubate him. A few hours after extubation, he has progressive hypotension and tachycardia. Examination is notable for cool extremities, weak distal pulses, and prolonged capillary refill with a blood gas on one liter per minute nasal cannula oxygen that shows metabolic acidosis with a lactate of 5.8. Which of the following is the most likely cause of his acute deterioration? Tachycardia, decreased venous return, tamponade physiology, increased left ventricular afterload, increased pulmonary capillary wedge pressure. All right, 94% got it. That's excellent. So this is really cardiopulmonary interactions. In patients with heart failure, mechanical ventilation is actually an inotrope. So when you withdraw it, you can have heart failure. And the mechanism by which is that you removed positive pressure in your thorax, and therefore you're increasing afterload because really what afterload in these ventricles is transmural pressure. So interthoracic pressure minus pressure within the ventricles. And by removing mechanical ventilation, you're making your thorax more negative, and therefore you're increasing afterload in patients. Great. Next one is you. Okay. Previous, so the long, let's do the trick on the long questions. We can read the last one and then read the whole thing. So the question is, in addition to the traditional sepsis resuscitation and management, investigation for which of the following diagnoses would also be undertaken? And you can see the options. So the case is a 15-month-old boy who's diagnosed with mono last week via a mono spot test. His parents bring him to the emergency room with increasing fatigue, general malaise, and fever for the past 20 days. He has not been eating or drinking well and has only one wet diaper in the past 24 hours. His parents had another child who died from sepsis at the age of one before the patient was born. The family lives in Iowa and recently traveled to Minnesota. An exam, those are his vital signs. I'll let you read them. He is toxic appearing with bounding pulses, lethargy, and marked hepatosplenomaly. The remainder of his exam is unremarkable. His labs are significant for the weight count, the CBC that you see there. You have prothrombin time, COAGs, LFTs, and a basic metabolic panel that you can read. And so then based on that, what of the following diagnoses should also be undertaken? You guys were paying attention. Good job. So a lot of the triggers, right, for primary immunodeficiency and HLH being the classic presentation of familial HLH. Nicely done. Who's next? Okay. This next one is a kid who's eight-day-old full-term baby who's post-op day number five following stage one palliation for hyperplastic left heart syndrome. So this is a Sano operation with a Sano, Norwood operation with a Sano shunt. His post-operative course has largely been unremarkable, though he remains intubated and mechanically ventilated. He has weaned inotropic support as normal renal function and is tolerating trophic entral feeds. In spite of decreasing his FiO2 to 21 percent, his arterial oxygen saturations remain greater than 90 percent. Which of the following interventions is likely to promote a more balanced pulmonary to systemic flow ratio? Extubation, addition of angiotensin converting enzyme agent, I guess, transfusion of pacred cells, inhaled nitric oxide, addition of norepinephrine. All right. The answer is addition of ACE inhibition. I mean, what does this patient have? The saturations are greater than 90%. And in a stage one circulation with the saturation of 90%, how is your QPQRs balanced? Is there more pulmonary or systemic blood flow? Pulmonary. More pulmonary blood flow. So you really want to increase systemic blood flow in this patient. Will extubation achieve it? I think extubation might be challenging in this patient with a volume loaded ventricle because there's more blood coming. If you have more pulmonary blood flow, more blood's coming back to your ventricle. So this ventricle has to handle that blood and the patient also has to breathe spontaneously for himself. So this is in some ways you're inducing congestive heart failure and you're extubating somebody with congestive heart failure. So that's probably not going to work as well. Transfusion of red cells. Red cells increase viscosity. And some people would say that it reduces pulmonary blood flow by increasing resistance, but that's not a definitive fix. Inhalation of nitric oxide reduces pulmonary vascular resistance. So you'll perpetuate this problem and increase saturations. And then addition of norepinephrine is really also increasing your systemic vascular resistance and worsening the problem. So the right answer is use of ACE inhibition to balance QPQS. All right. Ah, okay. This is mine too. So this is a previously healthy four-day-old full-term baby admitted to the PICU with severe cyanosis and arterial oxygen saturation of 73% on room air. Emergent echocardiography reveals isolated critical pulmonary valve stenosis, a one-millimeter patent ductus arteriosus, and a widely patent foraminal valley with left to right flow. After initiating prostaglandin E1, his oxygen saturation is improved to 95%. He undergoes a balloon pulmonary valvotomy. Vital signs on return to the PICU is a heart rate of 155, respiratory rate of 35, blood pressure of 75 over 32, and an arterial oxygen saturation of 98% in room air. Prostaglandin infusion is discontinued. 12 hours later, oxygen saturations have decreased to 84% despite supplemental oxygen. Heart rate's 162, respiratory rate is 38, blood pressure 78 over 45. Which of the following is the most likely explanation for recurrence of hypoxia? So this is a kid with critical pulmonary stenosis that was duct dependent. We've opened the outflow tract, and now we've come off the prostaglandins. So that's kind of where we are. So development of pulmonary edema, continued poor right ventricular compliance, worsened pulmonary vascular resistance, restenosis of the pulmonary valve, and closure of the foraminal valley. So the right answer for this is reduced right ventricular compliance. In patients with critical pulmonary stenosis, your right ventricle has hypertrophy because the ventricle has been working against this increased resistance to the pulmonary valve. So most likely that there's hypertrophy of the ventricles. If you have hypertrophy of the ventricles, the ventricular compliance is reduced. Now we've opened up the pulmonary valve and created an outflow for the right ventricle and the ductus is nearly gone. So if somebody had asked, if the question had contained, I would reopen the ductus and I would have chosen that. However, the reason why this patient is blue is because there is reduced compliance in the right ventricle. The patient has a patent for AMIN and he's got a large right to left shunt. So blood that comes back into the systemic veins that gets into the right ATEM has two ways to go. One is it can go down the tricuspid valve into the right ventricle, but we know that the compliance of the right ventricle is poor. And then the other way the blood can go is through the patent for amino valley to the left side of the heart. So blood always chooses the pathway of least resistance. So in this situation, the compliance of the right ventricle prevents blood from flowing easily into the tricuspid valve, but rather it more easily goes across the foramen ovale into the left side of the heart. So that's why this patient is hypoxic. Over time, now that you've taken away the obstruction of the right ventricle, the right ventricular hypertrophy will resolve. And that if you measure oxygen saturation in these patients, when they come back for follow-up in two months' time, they'll be more normally saturated because the ventricle compliance has improved. Our goal is to really take them through that period of time where the right ventricular compliance is poor and whether one would wait patiently or do other things such as a shunt and those are questions that are very specific to programs. Often we would go back on prostaglandins and put in a PDA stent and await the right ventricular compliance to improve. That would be our approach to it. But you can just wait and give the patient time to improve. So it's the compliance of the right ventricle that increases flow across the patent for amino valley that causes you to have hypoxia. Actually, I'm going to use that. Okay. Okay. Next one is a 10-year-old boy with ALL who's admitted to the pediatric ICU. His vitals are there. You can read them. He is tachypneic but does not require intubation. He's cold and appears peripherally vasoconstricted with weak pulses. The attending physician, which is you now, begins resuscitation with isotonic fluid and quickly inserts both a left subclavian CVL and a right radial artery. On transduction of the arterial line, hypotension and a noted decrease in systolic blood pressure are confirmed along with a wave amplitude that during inspiration approximately 20 millimeters. On transduction and confirmation of proper placement on the radiograph, the CVL reveals the following data. So a CVP of 18 with appropriate waveform with steep X and tenured Y descent and mixed venous sat of 55%. The question is, which of the following actions will be most immediately beneficial? You want to see the questions time again? Yeah. OK, so what do you guys think is wrong? Yeah, good job. Most of you got that. And so I think they're trying to get at the direct correction of the problem, which is why that is the right answer. I saw a lot of epinephrine, which might help support the patient but wouldn't reverse the underlying problem. Do you guys have any comments on that one? Yeah, there was a question asked this morning with another case of pericardial effusion as to what would you do first and what would sometimes. And volume was the answer to that question. But if you ask what would you do to definitively resolve the problem, then it's pericardial synthesis. What would you do in an emergency to maintain a blood pressure? Because patient's hypotensive would be volume expansion. And I don't think that was an option, so that at least made it easier. Yeah. I don't know if it was. I felt like it was. But if it was, most immediate medical would be fluid, correct? Yeah, I think if you want to get somebody out of hypotension, I think if you want to manage hypotension acutely, I think volume expansion was. I mean, it takes a little while to get your echo machine and a pigtail catheter and all of that stuff. So I think I would get volume to maintain your blood pressure till we get there. It's not, yeah, it's not an option here. So you didn't have that choice. I think it was actually assigned to Scott, but I'm happy to do it. Okay. We have a, all right, so we can skip to the end and just, I'll go with it. I'm going to roll with it. I'm going to roll with it here. All right. So we want to know which of the following is the most appropriate initial antibiotic coverage. So we're intensivists, so we're going to pick the most broad spectrum choice possible. Regardless of the stem, but we'll read it anyway. A 13-year-old boy who received a hemorrhagic stem cell transplant for AML three months ago was admitted to the ICU with fever. On arrival, he was awake, but tired and weak. He denies any cough, difficulty in breathing, chest pain, or bleeding. He also denies any recent sick contact. Vital signs are febrile, tachycardic, tachypneic, blood pressure 95 over 35, white blood cell count 0.7, hemoglobin 8.5, platelets of 35, 20% neutrophils, sodium 140, potassium 4.5, BUN 18, creatinine 1, and lactate of 3. Chest radiograph shows clear lung fields. He started on his third round of 20 mLs per kilo normal saline fluid bolus through his implanted port. The answer to the following is the most appropriate initial empiric antibiotic coverage. Okay, so a lot of controversy here, so we'll walk through this. I mean, I think everyone got this one. I think the key thing is, right, you need to have pseudomonal coverage. So you definitely want an antibiotic that has that. And so, actually, all of the choices there would fulfill that. And then vancomycin would be added in this case for broader coverage, because this is the case of shock, as well as the line makes this person a little bit at a higher risk as well, plus AML and so on. So good. The fungal coverage there, you know, is an interesting one. No one got tripped up by that. Would be something to potentially think about, although has not been febrile for that long, but AML would put the patient at slightly higher risk. So if this was prolonged fever, that would be something to think about. But empirically, the vancomycin-cefepime is a better combination, initially. Totally agree. That's you. Okay. Just before I start, I could say, when I reviewed this question, I actually struggled with it. So this is an 8-year-old, 28-kilogram girl who presents with a past medical history significant for a ventricular septal defect, which was close, and aortic valve was repaired for aortic insufficiency at four years of age. Since then, she's had progressive aortic insufficiency, and then had an aortic valve replaced last week with a mechanical valve. So she's going to need anticoagulation. After surgery, she was started on warfarin for anticoagulation for the mechanical valve, and a heparin infusion was started as a bridge. And her goal INR is 2.5 to 3.5. She was started on 5 milligrams daily, and after four doses, her INR is still 1.4. And which of the following is the most appropriate strategy to increase her INR? So increase an oral warfarin dose to 10 milligrams daily, and recheck INR tomorrow. Increase warfarin to 6 milligrams daily, and recheck INR in two to three days. Increase oral warfarin dose to 7.5 milligrams daily, and recheck INR tomorrow. Maintain the current warfarin dose, and recheck INR in two to three days. I struggle with this. My answer is call a pharmacy. Is that inappropriate? Yeah. That would be nice. Yeah. So my answer was increase warfarin to 6 milligrams, and recheck INR in two to three days. The way I kind of worked this question was, any time you increase warfarin dose, you're not going to check the INR the next day. So you can rule out the two stems, which have INR tomorrow, that can be ruled out. So then you're left with two to three days. You already know that after four days, you don't have an adequate INR response, so you're going to increase the dose. So that's kind of how I arrived at a 6 milligram dose. But I think this is a good example of how sometimes you have to eliminate the stems just by things that you wouldn't do or are not recommended, and then come up with the right answer. So the right answer is 6 milligrams, check INR in two to three days. It was assigned to me, but it's fine. Okay, so a seven-year-old boy with ascending paralysis admitted to the pediatric ICU because the acute care ward is concerned for impending respiratory failure. On physical exam, he has diffuse weakness and lack of reflexes in the lower extremities. He appears to be breathing comfortably with SATs of 98% on room air and a respiratory rate of 18. But negative inspiratory force, so a NIF on the acute ward, has gone from negative 40 to negative 35 in the past eight hours. His lungs are clear and his chest radiograph is normal. His ABG shows a pH of 7.4, CO2 of 38, and bicarb of 25, and a base deficit of negative 0.2. Cough and gag are intact. He's able to swallow without difficulty. He reports no dyspnea or dysphagia. He's alert and cooperative and says he's tired and would like to go to sleep. His only past medical history is upper respiratory infection two weeks ago. And screening labs that included CBC and a metabolic panel are normal. CSF exam shows no RBCs or white cells, but has mildly elevated protein. And earlier today, an MRI of the brain and spine were normal. His blood pressure and heart rate are normal for each. So which of the following is the most appropriate next intervention? Do you guys need the stem more, or are you good? So what does he have? And so either IVIG or phoresis would be an appropriate answer, but you only were given one of those. The reason that the ventilatory support options that I saw some of you choose were not the right answers, because the stem's not describing a patient with respiratory failure, right? He's neither hypoxic nor hypercarbic and doesn't seem in any distress, so maybe you can treat the underlying cause. It sounds like you have space to do that. And then methylpred is actually potentially harmful with Guillain-Barre, so IVIG is the right answer. Any comments on that? All right. Who's next? STCU2. Still? Yeah. Okay. So a three-month-old boy is intubated for RSV bronchiolitis. He initially had a rocky course that stabilized on day 14 of mechanical ventilation. His vital signs this morning are afebrile, his heart rate is 120, blood pressure is 82 over 40, and a respiratory rate of 37. He is on SIMV pressure control. Peak inspiratory pressure is 25 with a PEEP of 5 and a rate of 12, FiO2 40%. His ABG is 7.4, CO2 is 52, and PaO2 is 98. CBC shows a white count of 8,000, normal differential hemoglobin 7.9 with an MCUA of 92, and platelets are 250, and BRTIC is 6%. The physician plans to wean his ventilator towards extubation tomorrow. Which of the following is the most appropriate next step in managing this patient's anemia? All right. So what's the cause of his anemia, probably? And how would you describe his MCV? Great. So that's why the other ones aren't correct. Doesn't need iron. Just don't send more labs. They're going to get sick of my voice. So a 14-year-old girl whose height and weight are there received a lung transplant approximately two months ago to do cystic fibrosis, an end-stage lung disease. Her current immunosuppressant therapy is oral tacro and MMF, as well as prednisone. Her most recent tacro trough was 15. Her most recent surveillance bronchoscopy grew aspergillus, and her galactomanin was positive. She started an IV VORI, two-dose load, and then Q12 maintenance. Which of her immunosuppressant medications should be adjusted because of the addition of VORI? Yeah, you guys got it. So, this has to do with CYP inhibition, so it is TACRO, would need to be adjusted. You guys got that? MMF is not cleared through CYP. Yeah. I'll just add, I feel like those questions, the board tends to like those CYP inhibitors and accelerator questions, so just Google or look for a list of medications with those common interactions and just be familiar with the common ones, because they do come up a lot. All right, the next question is, a healthy 3-year-old boy presents with fever and several-day history of progressive dyspnea, rapid progression to ARDS, secondary to H1N1 influenza. He continues to be hypoxemic and is on high-frequency oscillation and prone ventilation and inhaled nitric oxide. His most recent oxygenation index is 35. A decision is made to initiate vena-vena secmo because of refractory ARDS, and immediately after cannulation he receives a bolus of unfractionated heparin, 50 units per kilogram, followed by initiation of an unfractionated heparin infusion. The infusion's been increased to 50 units per kilogram per hour, however, the ACT remains low. The target ACT is 180 to 220, so which of the following substances is the likely explanation for inadequate heparin effect or heparin resistance in this patient? Protein S, antithrombin 3, plasminogen, 1-valerbran factor, and protein C. Am I going to get through all the questions? We deserve an award. All right. So heparin reacts to antithrombin 3, and if you have deficiency of antithrombin 3, then heparin doesn't provide anticoagulation, and if you're on 50 units per kilogram, and if you haven't reached your target levels, that's heparin resistance. Some people will define it as 30 units per kilogram on patients for ECMO, and the only opportunity then is to change the anticoagulant. Very many people are using bivalorin. You can either change to that, or you can replace antithrombin 3, and antithrombin 3 is available as an antithrombin 3 concentrate, or you can just do an FFP. Either of those will work. Comments? An 11-month-old infant recently immigrated from Nigeria is admitted to the ICU with severe anemia. The vital signs are there, heart rate 165, respiratory rate 35, blood pressure 90 over 60, and a front brow with a SAT of 96% on room air. On physical exam, the patient is pale and tired, but not toxic. Lungs are clear. Cardiac exam shows a 3 of 6 systolic murmur in the left upper sternal border. The abdomen is soft with normal active bowel sounds, and the spleen tip is palpable 6 centimeters below the left costal margin. No hepatomegaly, and the extremities are pale with 2 post-distal pulses. The hemoglobin is 3.2, hematocrit of 10. The white count is 5.5, platelets are 650. Malaria is negative. Which of the following lab tests would most likely lead to the diagnosis? All right. What does the patient have and what's the acute complication? Yeah. Yeah. With the spondymegaly, right? So yeah, they want the definitive diagnosis, right? So you want the diagnostic test. Like the reticulocyte might be abnormal, but that's not going to tell you the diagnosis. Hemoglobin electrophoresis would. Cool. Questions? Comments? No, I'll just add. So what would the direct COOMS test and the total bilirubin and lactate dehydrogenase be looking for? Hemolytic anemia, right? And then what about the von Willebrand factory Cleveland protease assay? What's the other name for that? ADAMS-13. And so the patient had sepsis. She might be worried about TAMOF as a subphenotype. But what's the other thing that test for? Another microangiopathic hemolytic anemia? TTP. Yeah, exactly. But that didn't trick you. That's right. Okay. This is another question with heart disease. 16-year-old with hypoplastic leptarotomy syndrome. Okay. Presence to the emergency department after being hit by a motor vehicle accident while walking to school. She has previously undergone a FONTEN procedure. The decision is made to intubate her with etomidate, rocuronium, and lidocaine. She's intubated without complication and shortly thereafter has a cardiac arrest, which of the following is the most likely cause of arrest? Systemic ventricular dysfunction due to congenital heart disease that is worsened by induction agents, decreased systemic ventricular preload due to change in positive endothoracic pressure, pulmonary hypertensive crisis leading to hypoxic pulmonary vasoconstriction, and ventricular fibrillation due to elevated intracranial pressure and herniation. Wow, that's impressive, 100%. Everybody got it. This is the biggest problem with Fontan circulation. If you increase into thoracic pressure, you're going to lose cardiac output. If you don't have a fenestration, your cardiac output is completely dependent on what comes into your pulmonary arteries, and anything that impedes that will reduce cardiac output and then lead to cardiac arrest or cardiogenic shock. Any comments? What would you do to prevent that, if you needed to intubate this patient? I think volume loading to get some volume in, and then once you've intubated, it's really important that you're not bagging really hard, and you're not bagging with a lot of peep. You just want to make sure that you've got enough exhalation time, and you just have to be mindful about how you bag. I think it's a good example where sometimes giving fluid to a patient with congenital heart disease is actually the right thing to do, whereas most places say, heart disease, no fluid. You can put those things together, and that could actually be worsening this patient's problem. I think the next one's mine, too. Next one's a cardiac question as well. An infant with hyperplastic left heart syndrome is about to undergo stage one palliation with Norwood procedure, which of the following is a physiological advantage of the Sano shunt over a modified bailout toxic shunt? Sano shunt is connection of the right ventricle to the pulmonary circulate, so you're providing pulmonary blood flow through an RV to PA conduit, and BT shunt is systemic to pulmonary artery shunt. Pulmonary blood flow will be higher, so pulmonary arteries will develop better, so you're asking what is an advantage of a Sano shunt? Pulmonary blood flow will be higher, so pulmonary arteries will develop more significantly. The coronary perfusion pressure will be independent of pulmonary to systemic flow ratio. Stenosis of the distal anastomosis is uncommon with a Sano shunt. The Sano shunt restricts pulmonary blood flow more successfully than the right modified bailout toxic shunt. The answer is that systemic pulmonary aortations cost steel. In hyperplastic left heart syndrome, the steel is really steel from your coronary circulation. Therefore, if you, for some reason, inadvertently bag the kid or give him nitric oxide, drop your PBR, you're just going to empty your ascending aorta and your pulmonary arteries, and then have myocardial ischemia and arrest. If you look at the other stems, just as a matter of interest, pulmonary blood flow will be higher, so pulmonary arteries will develop more significantly. That's not a feature of Sarno-Shun's. The newer version of Sarno-Shun's include a valve in it. Previous versions of Sarno-Shun's didn't include valves in them. Blood would flow into pulmonary artery and then there'll be diastolic flow reverse in the pulmonary artery, so net pulmonary blood flow was decreased. Everything in congenital heart disease and pediatric cardiology is flow. Flow gives you growth. If you don't have enough flow, then your pulmonary arteries may not grow very well. A stenosis of distal anastomosis is uncommon. One of the Achilles heels of the Sarno operation is stenosis of both the distal and the proximal conduit. We've modified the operation now with reinforcing the proximal conduit with rings, so that you don't get stenosis, but you can still get stenosis by muscle that grows underneath the conduit, so that's not the answer. Then the Sarno-Shun restricts pulmonary blood flow more successfully than the right modified Balog-Taussig-Shun's. That's not right, mostly because there are two things that I don't know. I don't know what the size of the Sarno conduit. If it's a large conduit, then that's not going to restrict them. There's no size given as a way of being sure that it's your answer. The answer is the coronary perfusion is dependent on a pulmonary systemic blood flow, and that is the Achilles heel of the Norwood operation with the BT-Shunt. Okay. That's me as well or? It's Scott. Scott. All right. Previously healthy 16-year-old, 60-kilo woman presents to the emergency department with a complaint of weakness, rash, and foul-smelling vaginal discharge. Her initial vital signs are as follows, heart rate 145, blood pressure 80 over 45, respiratory rate 25, 88 percent on room air. Notable exam findings include diffuse, flushing, and bounding peripheral pulses with warm extremities. This patient's current physiologic state is best represented by which of the following? Okay, great. So this somewhat contradicts what I spoke about earlier, but I think your best assessment based on the clinical exam findings that you had available to you in the stem would be that this is a warm shock state with low systemic vascular resistance, the bounding pulses, the flushing, and a high cardiac output state. I believe there was a wide pulse pressure and low diastolic as well. And then the tachycardia and the clinical vignette suggests low preload in this patient who probably has toxic shock syndrome. So I think that's correct. The issue, and I think that's the best you can answer based on this vignette and choices that you're given, I just want to add that be careful with the test, fine, but in clinical practice just be careful because you may make that assessment, which may or may not be true, but if you make that assessment and treat blindly, and let's say start a vasopressor, let's say Norepi, maybe a fine choice, but even if you were right, the patient could still have myocardial dysfunction, which you could exacerbate by escalating the vasopressor support. And so if the patient gets worse clinically after you are up titrating norepinephrine, if that's what you started, sometimes our reaction is to give more of that medicine, oh, they need more of it. And you've missed the fact that now you've increased the SVR, and now you've unmasked myocardial dysfunction. That is now evident. So doing an early cardiac point-of-care ultrasound would really be helpful, and trending that over time, particularly if the patient isn't responding as you expect them to do. So getting more information is really important beyond just the clinical signs, which could be, one, misleading, or two, it could change over time. OK, last two questions. This is a six-month-old girl admitted to the Pediatric Intensive Care Unit following surgical anastomosis of the superior vena cava of the pulmonary artery. So this is the bidirectional Glenn procedure. He's extubated in the operating room, brought to the PICU on melronone, 0.25, and a little bit of epinephrine. And after four hours, he became she, is noted to have decreased urine output and cool extremities. Her vital signs include a heart rate of 168, blood pressure of 85 over 60, a respiratory rate of 30. Her oxygen saturation is 75% in 2-liter nasal cannula. The common atrial pressure is 5. Central venous pressure measured in the common atrial pressure is 5. The right jugular vena pressure is 7. Her blood gases show a pH of 7.23, PCO2 of 34, PO2 of 42, and base deficit of minus 7. Which of the following is the most appropriate next course of action, intubation, normal saline bolus administration, nitric oxide, or chest X-ray? So, this is a bidirectional glen. The filling pressures are relatively low, so you really want to volume expand these patients. I think in patients with cable pulmonary connection, so the glen and the fountains, I think if there's any suspicion that the patient may be hypovolemic, administering volume is the best option moving forward. Clinically, at the bedside for you all, when you practice, I think giving a little bit of volume makes sense. All right, next one is ... All right, 88. It's you, but I don't know. Oh, yeah, okay. It doesn't have to be. It's fine. All right. A six-year-old boy presents to the emergency department with a hemoptysis. The father reports the child has been healthy until three weeks ago when he developed fever, coughing, vomiting, and decreased appetite. Due to fever, the patients had taken him to his primary care provider. He's had some weight loss and had increased work of breathing, and he's on non-invasive post-depression ventilation. He's got an X-ray that looks like this, bilateral airspace disease. He's got ... Sorry. A PAOTO of 32 and hematocrit of 12.9. He has a BUN of 116 and a creatinine of 6.8. He is mildly coagulopathic with a D-dimer of 5.1. His ESR is elevated, 145. And the question is, which of the following serological tests is most likely to be positive and abnormal in this patient? And the choices are basement membrane antibodies, ANCA, myeloperoxidase, cytoplasmic antibodies, and antinuclear antibodies. This was not assigned to any of us. Okay. Sorry. No, it's okay. No worries. I should have made Scott do it. This was the hardest one. Yeah. In the set. I agree. I don't know. The warfarin one was a little hard too. I don't know when the last time that I ate those warfarin was. Okay. They know it though. I wouldn't have guessed this right. Honestly, when I read this question, I also guessed antiglominal or basement membrane antibody because I was thinking of Goodpasture syndrome. But the answer is myeloperoxidase, antineutrophils, cytoplasmic antibodies. And being a cardiologist, I have no idea what that is. So I think the main reason why it's probably not antiglomerular basement membrane antibodies, that's Goodpasture's, right? So the epidemiology of that would be uncommon in a six-year-old boy, right? It's usually more of an older female predominance. So you're probably more in the ANCA-associated vasculitides, which would be B and C. Beyond that, I'd have to look at the... An ANA is almost never the answer, right? Like acutely, ANA never gives you an acute illness. ANA is usually not very helpful. It's more of a chronic marker. But yeah, so... Sorry, say that again. Your son said something about his son. And it's about love. Yeah, so that was a difficult question, I would say. Yeah, that one's a tough one. OK. Tough way to end there. That was a. No, there's one more. Oh, there's one more. OK. The very last question. We can redeem ourselves. Yeah. So this is a six-month-old girl admitted to the pediatric ICU after VSD closure. She's developed complete heart block, which was noted in the operating room. Her vital signs include a heart rate of 50. See, bradycardia, I told you, in the cardiac ICU. A blood pressure of 80 over 50, and a respiratory rate of 30 breaths per minute. Her saturation is 98% in nasal cannula oxygen. And there's an ECG, which I will show you in a minute. She's hooked up to a temporary pacemaker, and the intention is to pace her. And the EKG looks like this. Like this. I can see a number of P waves that are not conducted, and narrow QRS complex. Let's see. So what is the most appropriate next action for this patient? So heart rate's 50, but you've got a reasonable blood pressure, and you're looking to see if you want to pace the kid at a faster heart rate. So increase pacemaker sensitivity by decreasing threshold, millivolt threshold. Increase pacemaker sensitivity by decreasing millivolt threshold. Increase delivery of electrical impulses by increasing MAs. Decrease pacemaker sensitivity by increasing MV threshold. And the pacemaker, the patient does not need pacemaker support. Interesting. Actually, maybe. Maybe, OK. Blood pressure of 50, and it's extubated. You like your six-month-olds to have a heart rate of 50? Not usually, but yeah. So the answer is decrease pacemaker sensitivity. So when you set the pacemaker, if you set the sensitivity such that the sensitivity detects any electrical impulses, it'll inhibit the pacemaker. And so, you know, this, the EKG in this is that there are no pacing spikes first. So even though you have set a pacemaker, a patient on a pacemaker, there are no pacemaker spikes. So my first response would be to go to the bedside and turn up the milliamps just to make sure that there's enough current passing through. So that would be my first response. And the second response, well, when I do that, I'm going to realize that the pacemaker is sensing and inhibiting any impulses that the pacemaker would put. And then I would actually decrease sensitivity and allow the pacemaker to pace more in an asynchronous mode so that you can overcome some of the sensitivity issues. So that would be my approach to thinking about this. And with that, I think that was the last question.

Meningococcemia

Pediatric Intubation

Bacterial Endocarditis

Commotio Cordis

Pediatric Myocarditis

Hemophagocytic Lymphohistiocytosis

Post-Operative Management

Immunosuppression

ANCA-associated Vasculitis

Pacing Management