All right, why don't we go ahead and get started since it's one o'clock. I'm Will Bender, I'm one of the pulmonary critical care faculty at Emory, and I'm moderating this session. It's Broken Hearts, which is a series of case reports, all of which, at least based on title, sound really interesting. So just a reminder for the presenters, if you could verbally disclose if you have any disclosures or not. And then each presenter will get eight minutes to present their case, and then there'll be about two minutes or so for questions. And if folks have questions from the audience afterwards, just go ahead and use the microphones and we can all hear them. So why don't we go ahead and get started with it looks like our first case is Dr. Ahmad with a case of malignant idiopathic polymorphic right ventricular outflow tract ventricular. Hello? Okay. Hi, guys. My name's Tanjeev. I'm one of the second-year medicine residents at Tulane in New Orleans. I have nothing to disclose. So my case is on right ventricular outflow tract, ventricular tachycardias. So the objective for this lesson is to just highlight kind of the significance of it, why it's important, and just what workup and interventions we did and recommend to prevent its progression to VTAC. So the presentation is a 21-year-old woman. She had no medical or family history. And she came to the ED after a syncopal episode. And it happened while at the exertion. She was just washing some dishes. And she actually had five of these episodes over the past two years. And even if she didn't syncopize, she would have dizziness, weakness, and other such symptoms. And they occurred at both rest and exertion while she was on the couch watching TV or walking, running. She's actually a college student. So a couple of times it happened while she was in between classes passing. And they just occurred more frequently over time. So when she arrived to the ED, this was the EKG that we had gotten. As you can see, it's a polymorphic VTAC. While this was happening, actually, she was completely awake, asymptomatic, and talking to us. And her vitals were completely stable. And so we just said it was hemodynamically stable, polymorphic VTAC. Right away, she was brought to the ICU. And an esmol and amiodarone drip were started. And it actually showed that her VTAC went away. And she was completely in sinus rhythm afterwards. We got an EKG after that. And it showed that there was a completely normal QT interval. There wasn't any delta waves that we could appreciate. And no ST segment depressions or elevations. So it's completely normal. And so some of the workup that we started pretty quickly was, after it was treated, we got a cardiac MRI to see if there was any fibrotic lesions, any structural damage that we could assess. And it pretty much just showed that she had a completely normal right and left ventricular structure. No infiltration. No fibrosis. Nothing. Quickly after that, she was brought to the EP lab the next morning. And they did a procainamide challenge to try to provoke any arrhythmias. And they didn't get anything. And they also did not get any sort of Brugada pattern with ST elevation in V2, V1, V3. And then quickly after that, we also got exercise stress testing. And that's when we saw that when she did exercise, even in the first stage, there were PVCs that were pretty apparent. And the EP doctor was able to kind of localize it to the right ventricular outflow tract. It was, it's not going to be apparent on this EKG, but in 2, 3 AVF, it was a negative deflection. In lead 1, it was a positive deflection. And in V5, V6, it was a negative deflection in the S wave of the PVCs. And so pretty quickly, it was able to localize it. And promptly, she was taken for an ablation. And it showed complete resolution of the VTAC and the PVCs. Even in repeat exercise stress testing, it was not apparent. And then they also did an isoproteranol challenge. And there was no PVCs seen then either. So this was kind of a case where it shows that the PVCs weren't exactly benign. A lot of time, they can be completely asymptomatic or just present with palpitations. But this was a pretty rare case where they were malignant. It was idiopathic since there were no structural abnormalities, no channelopathies. And if it was not treated, she could have progressed to VFib and eventually cardiac arrest, not just a syncope. So ablation was curative in this case. But if it was not and, you know, repeat isoproteranol or stress testing showed PVCs, she would have needed an ICD to prevent further VTAC. So that's my presentation. Just kind of highlighting the importance of investigating PVCs to figure out if they're benign or malignant. They require workup with, you know, looking for structural disease, channelopathies, with MRIs and stress testing. That's pretty much it. Thank you. Any questions from the group? I have a couple. The first is, you mentioned on your initial presentation that she had had episodes in the past. Had she sought care during those episodes or was she just kind of like, I don't feel well? So she said she went to her, and this is kind of classic, she went to her family medicine doctor and they said it was just anxiety and caffeine causing the PVCs. And so they never actually referred her to a cardiologist or anyone. And so she kind of presented to the ED on her own when it was getting more frequent. Got it. Okay. And then the second question I had is, does she have any family history of any, like, sudden cardiac death or anything like that? Yeah, that was one of the first things we asked her. And surprisingly, there's nothing. Yeah. Okay. Great. Awesome. Thank you. Thank you. Hello, everyone, and thank you for participating in this session, Cardiac Arrest in Medical School, a Rare Case of Sudden Cardiac Arrest in a Healthy 26-Year-Old Male Due to Myocardial Bridging. My name is Tasmia Hawk. I'm a third-year internal medicine resident at Abrazo in Phoenix, and I have no financial disclosures. The goal of my presentation is to shed some light on myocardial bridging, what it is, how patients may present, and what are some treatment options for it. We have a healthy 26-year-old male medical student with no past medical history who was at his university gym working out when he became unresponsive after five minutes of high-intensity exercise on a stationary bike. There was a cardiologist nearby who found him to be pulseless and initiated CPR and did achieve ROSC after two minutes. During transfer to RED, he did become responsive, alert, and oriented, and was complaining of some fatigue and chest discomfort. He denied taking any pre-workout supplements, any stimulants, any testosterone, and his urine drug screen was negative. He also denied any family history of sudden cardiac death or premature coronary artery disease. In the ED, vitals noted him to be tachycardic with a heart rate of 123, and EKG showed no ST changes. His tropes were also negative times three. CT head was negative, and CTA chest were also negative. These are his labs when he first came in. He had some reactive leukocytosis, 17.8. His bicarb was 5. His gap was 37. His crowning was a little elevated, 1.67, and his lactic was like 12. But after six hours of just fluids, all of his labs came back to normal. He had an extensive cardiac workup done. He had an echo, cardiac MRI, EP study, nuclear stress test, myocardial perfusion study, and all of which were negative. He then had a CCTA, which showed a coronary calcium score of zero, but there was difficulty following the LAD artery beyond the mid-left ventricle. So then he had a left heart cath, which showed no stenosis, but it confirmed significant myocardial bridging involving the mid-to-distal LAD. The patient was started on metoprolol but could not tolerate it and was quite bradycardic. He was then evaluated by CT surgery and ultimately had an implantable loop recorder placed and was discharged with a life vest. So what is myocardial bridging? It is a rare congenital variant that occurs when a portion of the major coronary arteries descends into the myocardium rather than overlaying it, potentially causing serious complications. The true prevalence is unknown because not everyone is getting CCTA and left heart caths. But given no other identifiable cause for our patient, we believe this pathology was the reason for his cardiac arrest at the gym because when he was exercising, it constricted the intramuscular portion of the LAD, and that's why he passed out. If you look at figure 1, you'll see that the LAD during diastole and then during systole in 1B, it's very constricted and narrowed. Hopefully, this video plays. Oh, there's no audio? That I don't know. I don't believe so. OK. LAD continues. Oh, OK. Perfect. OK, let's try this again. This is the left main giving rise to the LAD and the diagonal, D1. LAD continues right here, giving rise to D2, which is here. LAD continues right here, and you can follow it, follow it. And then it goes into myocardial breaking. We're no longer able to follow it. You should be seeing it here. You're not. And it should still be here, but you don't appreciate it. There you go. Yep. This is the left main. So it's important to consider myocardial bridging as treatment can prevent life-threatening ischemia, stress cardiomyopathy, and malignant arrhythmias. Treatment is aimed at reducing heart rate and myocardial contractility with medication. Beta blockers are a first-line therapy, but my patient could not tolerate it. Nitrates are contraindicated, as are aspirins and statins, unless they actually do have coronary artery disease. If patients are refractory to pharmacotherapy, you can do some intervention like stent placement or cabbage. So I was speaking to the cardiologist. Because of his significant LAD myocardial bridging, even if he did have a stent placed, it would still get crushed during exercise, so it wouldn't really help him. And he is being evaluated for coronary bypass grafting at the moment. Diagnosis of myocardial bridging is made when a section of coronary artery is found to be within the myocardium. This can be documented during a CCTA, intraoperatively, or a post-mortem examination. Our patient had substantial myocardial bridging that led to his cardiac arrest. Thank you. Thank you. Hello everyone. My name is Chris James. I come from Miami, Florida. I'm a third-year IIM resident, and today I'll be talking about left ventricular non-compaction, a rare cause of thromboembolic stroke. I have no disclosures. So LVNC is a type of congenital cardiomyopathy that may increase the risk of heart failure, arrhythmias, and thromboembolism. Usually when the heart is forming in the fetus, the LV is very hypertrabeculated. Now as the heart forms, those trabeculations go away and it becomes more smooth. And then when a patient does have LV non-compaction, those hypertrabeculations serve as a nidus for clot formation. So here we present a patient found to have LVNC after presenting with a stroke. She's a healthy 46-year-old female represented with focal neurological deficits, specifically her right arm is numb, right side facial droop. She was given thrombolysis and emergently taken from mechanical thrombectomy with complete resolution of her symptoms. Cardioembolic workup with a TE was performed and revealed no evidence of emboli or PFO, but showed a ratio of 0.29 from trough of trabecular recess to peak of trabeculation, which is diagnostic of LVNC per the Chin Criteria. Now there's many criteria you can use. I personally use Chin Criteria, which is essentially the ratio from the peak to the trough in end diastole. You can also use the Jenny Criteria, which is a similar ratio of 2 to 1, but that's an end systole. And there's also Stoll-Berger Criteria, which is number of trabeculations from the LV. I believe the Stoll-Berger Criteria, it's more than 3 hypertrabeculations from the LV wall. Now here we have the TE imaging of the LV. Here you can see at least 3 hypertrabeculations, so it's diagnostic not only by the Chin Criteria, but by the Stoll-Berger Criteria as well. So with LVNC, although thromboembolicism is a known complication of this condition, the incidence is currently unknown. It's first established as the etiology of her thromboembolic stroke due to no other cause behind it being identified. Now in addition, there is no evidence-based guidelines that exist about managing patients with LVNC after a thromboembolic event. Given her hypocoagulable state due to her blood stasis in her non-compacted myocardial tissue and her overall low bleeding risk, the decision was made to anticoagulate her with Xarelto. Now there's no guidelines, so I chose Xarelto because it's once a day, but you can do Eliquis. Last line would be Warfarin, but Xarelto and Eliquis are my go-tos. Guidelines aren't clear as to what you can prescribe, but it's essentially physician-dependent. So in summary, LVNC is an extremely rare congenital cardiomyopathy with unestablished guidelines to prevent recurrent thromboembolic events. Therefore, these patients do require an individualized approach to determine a need for anticoagulation. Thank you. Any questions? What was the discussion like with the Xarelto and the Eliquis? I mean, DOAC's statins are kind of taking over the world and everyone's going to be on one at some point, but I mean, I'm assuming, like you said, there's not a lot of literature. Is there anything described in the literature to use? No, so the guidelines just recommend anticoagulation, but they're very vague. For her, she doesn't really like taking medication, so Xarelto once a day, as opposed to Eliquis twice a day. So we figured she'd be more compliant with Xarelto. Interesting. Okay. Got it. Cool. Thank you. Alright, next up is Dr. McAuliffe with fistula to failure, an unexpected source of biventricular cardiomyopathy. All right. Good afternoon, everyone. My name's Jacob. I am a chief resident at EVMS. I have no disclosures. I'm going to be talking about a pretty uncommon etiology for biventricular cardiomyopathy and then hope to discuss some management considerations and future perspectives on the topic. So jumping right into the case presentation, we have a 59-year-old who's showing up to the ED with a chief complaint of 24 hours of persistent dizziness and some left transient extremity weakness. You see his past medical history detailed above, decent amount of comorbidities, but in significant cardiac, past medical history is only significant for remote PFO closure. His physical exam documented by the ED physician was unremarkable. Initial lab work, unremarkable, slightly elevated troponins, but stable. So he was ultimately admitted for a CVA workup, which included a protocol-driven echo that revealed a new systolic failure with an EF of 25%, global hypokinesia. And this is in comparison to an echo about six years prior that was fairly unremarkable. This prompted stress testing, which showed a large area of scar, but also some areas of viable revascularization targets. Here we have initial coronary angiography, which you see there's definitely something abnormal. He was found to have a preserved cardiac index, and this prompted engagement for the complex coronary, the CHIP team. And he was ultimately discharged from this index hospitalization on new GDMT and maintenance diuretics with a wearable defibrillator and referred to a tertiary referral center for interventional options. Further workup helped characterize the lesion. Here we see a coronary CTA that characterizes it as a fistula between the coronary sinus and the left circumflex. You can see it's quite torturous. And if you don't have a marker, but you see over the label that the diameter is about twice the size of the left circumflex artery. So here we have repeat coronary angiography. Sorry, the bottom's a little cut off. But this is before percutaneous transcatheter embolization. To reduce a fairly technical procedure into a few bullet points here, an aspiration catheter was advanced as distally possible within the fistula. At this point, a vascular plug was deployed, which resulted in immediate but incomplete flow reduction. And so this was followed by proximal coil embolization. And we see here post-procedural repeat imaging. You see the coil's pretty prominent. And it's a little bit more subtle. But the vascular plug can be seen, especially in the left-sided image. And you see total occlusion of the fistula. And here are some still grabs from the same angles for reference. Looking at the hemodynamics, the shunting degree was quite significant at 1.6. And you see almost total resolution post-stenting. Pulmonary hypertension also improved immediately after occlusion. And you see the right heart filling pressures and the wedge pressure also decreased. Ultimately, the patient tolerated the procedure very well without any complications. He was monitored for 24 hours and discharged. Surveillance left heart cath at six weeks, showed persistent occlusion. Unfortunately, his repeat echo was delayed. But he did have it completed in the interim between submitting these slides and today. And unfortunately, his EF did not recover. So he was referred for defibrillator placement. So moving on to the discussion, coronary artery fistulas are very rare. It's a little bit uncertain what their true prevalence is. Large retrospective coronary angiography record surveys put the initial prevalence at 0.18% of the general population. But with newer modalities of imaging, coronary CTA scans estimates that there might be a higher rate up to 0.9%. Majority are congenital. Largely, 9 out of 10 are going to be congenital. But acquired forms have been described, usually iatrogenic related to surgery or coronary instrumentation. But traumatic fistulas have also been described in the literature. The interesting thing about coronary artery fistulas is that their range of presentation is highly variable. We have cases where anomalous murmurs led to their discovery. And then at the extreme end, ACS, acute chest presentations. It's important to further characterize coronary artery fistulas by a couple of factors. The vessel of origin, the saccubara typing is largely proximal versus distal. The site of termination, whether this creates an AV fistula or a chimeral fistula, one that terminates in a heart chamber. Micro versus macro fistulas, morphology, simple versus complex forms, such as the plexiform variants, which are particularly rare. And then, obviously, it's important to understand the associated pathophysiology that the fistula creates. In this case, left to right shunting. And you have to be aware of coronary steel phenomenas and other intrinsic changes, such as aneurysm or thrombosis within the fistula. So management is largely driven by expert consensus. There's relatively few case series. The big points of consideration are whether invasive closure is warranted or if medical observational management is indicated. And when we discuss invasive closure, we're largely talking about either surgical ligation or percutaneous transcatheter embolization, which our patient underwent. I'm not going to go through all of these, but there's a great JAC article that summarizes some recommendations. A few key points is if they're particularly large, surgical ligation or embolization can be indicated, even if they're asymptomatic. Any symptomatic fistula should warrant some invasive closure. Then, in regards to percutaneous closure, arterial versus venous approaches are going to be informed by that further characterization. I discussed initially that the type A, the proximal ones, are more readily amenable to arterial approaches. And distal, very dilated fistulas can be more amenable to venous approaches. So future perspectives, it's resolving the specific indication for closure, the degree of shunting, the type of circuit created, the timing of intervention, these are all things that would warrant further investigation. And longitudinal comparison between the modalities of closure would also be a ripe area for further investigation. So just in conclusion, we're talking about a very rare form of anatomic anomaly, and this manifests along a spectrum of pathophysiologic significance. In our case, we had a large symptomatic CAF that led to left systolic heart failure via coronary steel syndrome, and also right ventricular dysfunction due to left-to-right intracardiac shunting compounded by group 2 pulmonary hypertension. And with that, I'd be happy to take any questions. Thank you. Is this the patient's initial presentation was the first time they had developed symptoms? Yeah, so he has a very high comorbidity. He had undergone actually a stress test, I think, four years prior that had a normal EF. If you look back at the stress test imaging, though, I found it – I don't know that anyone would have picked it up initially, but you see – I don't know if the – on the cross sections, you see that there's almost anomalous uptake in the transverse – well, actually in all of the sections, that area of particularly high white. So that's actually the fistula that you can notice. I don't know that anyone would – could make that diagnosis off of this, but he had a stress test for three years prior – or three or four years prior that had similar anomalous uptake. But yes, this was his initial presentation with – for – that led to this discovery. Great. Awesome. Thank you. All right. And then our next case is with Dr. Pass, a mixing artifact on imaging misinterpreted as a pulmonary embolism, an adult congenital heart disease with hemoptysis. Awesome. Try this again. Oh, wait, wait. Hopefully it works. Let's see what happens. It's okay. I'll just regale everyone. Draw pictures. Or draw pictures of it on the wall. Oh, I think we have to do the 4019. Oh, that one? This one? The bottom one, yeah. Okay. Maybe not. Sweet. Cool. Cool. Hey, my name is Mason. I'll do a quick presentation on a fun case that we had, basically hemoptysis, hypoxemia, and a broken heart. I am a PGO2 at OHSU in Portland, Oregon, and I have no disclosures. Objectives, we will leave blank because that would ruin all the fun. So here's our case. A 27-year-old Spanish-speaking male presented to an outside ED with small-volume hemoptysis for two days. Initial vitals were concerning for saturations in 80% on room air. Understandably, differential was PE, PE, PE, so they got a CTA, and lo and behold, it demonstrated acetyl-PE. So PERT was activated, and he was requested to come to our MICU for catheter-directed thrombolysis. Seems pretty open and shut, right? So we'll take a look at the images. We'll go with the axial first. So a little slow go. We'll go from superior to inferior here. And there's some unusual things in these images, but you've got the aorta and then pulmonary arteries. There's a mess right there, and it definitely looks like a PE. There's some unusual things in the heart, too, but we'll leave that there for now. No one's staring at the pulmonary artery at this point because he's been billed as such. So we'll leave that there for now. He comes to our MICU. Oh, here's the money shot, too. This is what they were looking at when they found what they thought was acetyl-PE, which, you know, looks fairly convincing. But he comes to us after we get the images, and he's on high flow at max and then also at heparin drip. Comes in, he's satting in the low 90s, otherwise looks fine. This is where things started to not add up for us because he's well-appearing. He's no acute distress. He's conversant. Exam is pretty non-focal, maybe mild crackers in the right lower lobe, but he can give a whole history. He's sitting up in bed, positive cell phone sign, the whole nine yards. Has pretty unremarkable medical history. His mom does say that he had two heart surgeries as a child, but otherwise doesn't really know much else. And then our initial workup was notable for a hemoglobin of 20. And then also on exam, we noticed some pretty impressive clubbing in his toes and his hands as well, which started to bring us to the idea that maybe this hypoxemia wasn't acute at all. So we took a look back at the images, went through the coronal section, and we noticed something pretty unusual. This is a little bit faster, but I'll go back and we'll see it again. Notice something unusual about the SVC. And then also some of the right pulmonary vasculature as well. So we'll zoom in on some of this, but this is the best image right here. You notice that the SVC actually empties into the main pulmonary artery, which is the second step of Fontan procedure for congenital heart disease. This gentleman had only completed two parts of the three-step Fontan, and this is what's called a Glenn anastomosis. So this is what he had. And then we had our friendly radiology college take a look at it. They noted a couple of things. First, they noted the Glenn anastomosis, as I already mentioned. They also noted as well that there was no large PE allowing for mixing artifact in the setting of this Glenn anastomosis. Additionally, they pointed out some right lower lobe ground glass, which maybe could have been the focus of our hemoptysis in this gentleman. So just to take a minute to think about his anatomy based on what they saw as well, was that this gentleman had a hypoplastic right heart with a large VSD and then had completed the Glenn part of his procedure but not the Fontan. So his SVC was emptying into the pulmonary artery that you see on the right. So, you know, to wrap up the case, we took a closer look at the right lower lobe ground glass. Bronchoscopy was performed, and we noted a bleeding in the right basal segment there, consistent with AVMs in the setting of his Glenn. So rounding it off, we ran into the room as soon as we realized this, stopped the Heparin drip, and then the bronchoscopy was performed. As I noted, IR was consulted, and they embolized the arteries feeding these AVMs. He did really well, had no further hemoptysis, and then adult congenital heart disease service was brought on board, facilitated establishment of his final anatomy, which is, as I discussed, tricrespid atresia with a large VSD and an F Glenn without a Fontan. So why does this matter? Why is this important? I think really it's most important because as the CHD population's life expectancy increases, we as adult providers are going to see more and more of these folks, and so we're going to need to maintain a broad differential that hemoptysis hypoxemia isn't always just a PE. It can be other things such as aberrant cardiac anatomy, as in this gentleman. And then also we demonstrated how to manage those pulmonary AVMs that we can see in this particular scenario. So we'll go over the lesson objectives, which is we discussed a case of hemoptysis with suspected PE, explored adult congenital heart defects and their varied presentations, and determined the best management for said AVMs, as in this case. Thank you very much for your attention, and I'll take any questions. Thank you.

pulmonary critical care

syncopal episodes

myocardial bridging

LVNC

coronary artery fistula

acute pulmonary embolism

congenital heart disease