So, hello, where are the presenters? Who will start first? You are Catherine Kim? Catherine Kim? Gaurav Prabhu? Okay, three. Gaurav Prabhu is the first, I guess, here. Okay. Starting with constrictive brachycarditis, masquerading as liver failure. Okay, and this is the, Catherine Kim, this is it here. Gaurav Prabhu. Okay. Hello. All right, hi. Good morning, everyone. Aloha. I'm Gaurav, one of the critical care fellows. He starts with his presentation. Okay. Do you need me to stop? Okay. Go ahead. Okay. Hi. So, the case I'm presenting today was this really interesting case that we had, and after COVID, one of the few cases that we put on ECMO that actually came off successfully without major complications. So, as the topic suggests, it was a cardiac arrest due to secondary to amniotic fluid embolism and what we did to resuscitate this patient. Like I said, I'm Gaurav, and these are my co-authors on the paper that we wrote. I have nothing to disclose. The learning objective of the session is to understand about VA ECMO in amniotic fluid embolism. So, starting my case, I had a 29-year-old female with no significant medical history who was G2P0 at that point, who came in because she thought she had spontaneous rupture of membranes. She was a week delayed at this point when she came in, and she remained in latent labor all throughout her hospital course at this point. At around midnight, she transitioned to active labor. There was planned to go for the emergency section by 8 a.m. in the morning. However, by 7.30, she was 100% defaced and dilated, so they attempted to push. At 7.40, when she attempted pushing, nothing happened, and she had an episode of tonic-clonic seizure and immediately lost pulses. They called a code blue, a PE arrest. Four minutes into the arrest, they decided to try and save the baby at that point, so they went to the perimortem C section with cardiac compressions going on. However, the infant was delivered successfully, and we got ROSC at 7.56. Four minutes after ROSC, by this time, the ICU team is already there because it's an OB service at this point. She arrests again, and at 8 o'clock, she goes into a PE arrest. With this arrest, this time, she's bleeding massively from her vaginal wall. She nearly had two liters of blood out. We called a massive transfusion protocol. Within a few minutes, we had 555 in her already. They gave her oxytocin, carbaprox, TXA, put a BACRI balloon in that's a vaginal tamponade, like a uterine tamponade balloon, to see if something stops the bleed, but nothing did. Finally got some homeostasis and ROSC after 29 minutes at this time. At 8.39, she came up to the ICU. Soon after, she coded again at 9.12, PE arrest, followed by V-fib arrest, and we got ROSC after a few minutes. Again, a second massive transfusion protocol was initiated because she put out nearly 2,000 CCs from the BACRI balloon again, and she got a huge amount of transfusion. At the end of the case, she got like 32 PRBCs, 15 platelets, and multiple cryo and FFP at this time. A decision was immediately made to send her to the IR suite for embolization. She was on the suite, she was on the table when she lost pulses again. Got ROSC within a minute. However, IR aborted the case. She was immediately transferred to the OR right next door, and the people with surgery and OB-GYN scrubbed in, did a hysterectomy for her, and luckily got ROSC after that. At this time, the primary ICU team, which was us, discussed with our ECMO specialist because she was maxed on multiple vasopressors, had completely gone into ARDS. I'll show you the x-rays afterwards. Was on very, very high ventilator settings and not improving, and then she coded again. This time, the code time was one minute only. We got ROSC. Immediately, the ECMO team was at bedside, and within 20 minutes, she was started on the circuit. These were her labs on admission to the ICU. Her pH was 6.8. This is after multiple bicarb pushes, maxed on a lot of things. Bicarb was 11. This time, she had not completely gone to DIC yet. However, that happened pretty soon. She went into DIC, 5 million down to 168. Again, we transfused more 12 units of blood, FFP, cryoplatelets in her at this time. This was the point-of-care ultrasound we got for her when she came to the ICU for the first time. For cannulating, we used an 18 French right femoral artery cannula, a 24 French right femoral venous cannula, and the distal perfusion cannula, unfortunately, could not be inserted immediately after cannulation, so she had to go back to the cat lab afterwards to get a distal perfusion cannula placed, but that was successfully done in the cat lab afterwards. This is what her X-ray looked like pre-ECMO when we went in there. Day 0 to 1 on ECMO, she was doing fine. She had a flow of 3.5, 100% of 502, 3.4. Again, because of the massive transfusion she was receiving and all the crystalloids and colloids, she lost pulses in bilateral upper extremities, swollen, all lines were removed, no pulses like proper compartment syndrome, got bilateral fasciotomies done at bedside, also had a large amount of hematuria. Urology colleagues at that point said not to put her on CBI because they were concerned about possible laceration of the bladder when they did a perimortem C-section. She also was not responsive on no sedation multiple hours after, and she did have an episode of seizures, so we started a CEG on her to make sure that she was not in status, which she luckily was not, and given the no urine production, she was started on CRRT. This is what her ECHO looked like day 1 on ECMO, and the X-ray, as you can see, improved significantly with everything going on. Then, of course, unfortunate events for her were not finished. Day 1 of ECMO, middle of the night, her flow went down from 3.5 to 1.5. She was transitioned off all pressers, however, immediately was matched out on Levo, Epi, Neo, and Veso at this time again. Bladder pressures went up to 27, abdomen was distended, and so we did a bedside X-lab for her for abdominal compartment syndrome, drained 2 liters of serosanguinous fluid again at bedside, and ECMO flow went back up as soon as that was done. Then, in that regard, she had no complications. After, she remained on ECMO at a stable flow rate of 4 liters. She was off all vasopressors, kept her on dobutamine. The DIC resolved. All through this, her right lower extremity on the SATs, the SAO2 on the right lower extremity, was always in the mid-30s and 40s, even after we tried all our approaches to try and get the SATs up, even with the digital perfusion cannula flushing fine and everything going fine. No seizures on EEG. This was post-op day 2 on ECMO. This is what her heart looked like. Her squeeze and everything had come back to normal. Day 3 on ECMO, she remained on ECMO. She woke up. She followed commands. She went back to the operating room with urology, with gynecology, and with surgery combined. They found a necrotic ovary in her, some oozing. So they had an oophorectomy done. They found a large clot in the bladder, for which they did an anterior cystotomy, removed the clot, and then closed her back up, and luckily were able to close the abdomen. Continued on CRRT when she came back. This was her echo on day 3 on ECMO. As you can see, everything came back perfectly fine. Day 4 on ECMO, we started performing the ECMO VEIN trial on her, where we went down to a flow by half a liter every minute, and we went down to a flow of 2. At this time, she was transitioned off all vasopressors. She was only on dibutamine. As you can see, the gas improved significantly. Her vent requirements were really low. Sweep was just 2. She was taken to the OR for ECMO decannulation. Upon removing the distal perfusion catheter, we saw there was no retrograde flow from the femoral artery. She had a fogetti thrombectomy done at that point with returned flow. Then she developed a large effusion on the right side, getting a chest tube placed. Remained on CRRT. Post-ECMO course, we did daily dibutamine VEINS and were successfully off dibutamine 3 days post-ECMO. Ventilator was VEIN. She was extubated the second day after she came back from the OR after ECMO decannulation. CRRT was off by day 3 as well. By day 4, she had her fisiotomies of the upper extremities closed. By day 11, she was out of her ICU. And by day 13, she was discharged to a rehabilitation facility. Unfortunately, she had to come back after a month to have an amputation done of her right lower extremity because that never recovered. She had a below the amputation for dry gangrene, essentially, at that point. So what happened with her, what we think happened with her, is that she had amniotic fluid embolism. Of course, like the Society of Maternal-Fetal Medicine has four criteria for diagnosing anyone with an AFE. That is, like sudden onset of cardiac arrest or hypotension, sustained blood pressure less than 140, DIC, onset, peri, labor, and absence of a fever. Our patient met every single criteria for that, which, of course, complicated with DIC, human anatomy stability, and ARDS, which is 90% of mortality in AFEs because of these three things. VA ECMO was the one that rescued her. Without that, she would have definitely not made it. I mean, of course, everything was complicated by abdominal compartment syndrome, massive edema, bilateral facial upper extremity, fasciotomies, hysterectomy, oophorectomy, a right below knee amputation. But at the end of the day, she's living independently now, and the baby's doing really well as well. She came back to visit us, and it was really, I'm getting goosebumps thinking about it. It was such a nice experience to see someone this young who was a visitor in this country. It was a student for PA school who had all of this done, had no family in the country, and did not have anyone to support her. But looking at her, seeing what she is and where she is now is really awe-inspiring. Thank you, everyone, for listening. If you have any questions. Thank you very much. Thank you. Hello, everyone. I am Samridhi. I am one of the third-year fellows at Michigan State University in pulmonology and critical care, and I have nothing to disclose. So learning objectives for today are understanding the roles of eosinophils in eGPA and eosinophilic myocarditis, reviewing differences between blood and tissue eosinophilia, and learning about therapeutic options such as biologics in this case. So the case presentation here is I have a 44-year-old male who presented with chronic rhinosinusitis. About two years ago, he had septoplasty and turbinate reduction done, asthma, for which he was on PR and albuterol per his PCP, and CKD3, presenting with fever, headache, nausea, vomiting, and watery diarrhea. Initial vitals, he was febrile, with elevated heart rate, respiratory rate, and satting more than 90% on room air. His white count was slightly elevated. Procal was elevated to 3.32, set rate of 65, and CRP of 336. The investigations, all the cultures were negative, including the CSF and BAL studies, although he was started on broad-spectrum antibiotics initially. Here I'm pointing out a mass that we saw in his left hilum, and this was along the left lingula, extending to the left lower lobe, measuring about 3.7 centimeter. And along with this CT chest finding, there was also diffused esophageal thickening with inflammation of the second and proximal third portion of the duodenum. So the hospital course was a bit complicated. He decompensated quickly after arriving, and he was brought to the ICU on day two, and was started on pressers. And upon arrival to the ICU, transfluoresic echo showed global hypokinesis with EF of 17%. Then he was quickly taken for Bi-Cath. He got the right heart cath, which showed mean PA pressure of 30, VEG of 25, and cardiac index of 1.7, with a mixed VENUS of 39%. And obviously his ejection fraction was reduced, so impella was placed, and they also obtained endomyocardial biopsy during that procedure. So ultimately, interestingly, the endomyocardial biopsy showed eosinophilic infiltrate, which was suggestive of eosinophilic myocarditis. I don't know if I can point out these bi-lobed structures. With bi-lobed nuclei. Those are the eosinophils. And ultimately, the course made him undergo EBUS as well, because he had that mass that was going on from the left lingula to the left lower lobe. And on biopsy, it shows necrotizing granulomatous inflammation. So the clinical course showed that he was started on pulse-toe steroids for the first three days, and ultimately he was discharged on prednisone. And repeat echo, he had normalization of his EF completely, from 17% in the start to 65% on day 15. Also, the repeat CT showed complete dissolution of his mass after being on steroids for about 15 days. Interestingly enough, he did not have any peripheral eosinophilia. And I can talk about a bit of definitions of what that precludes. The peripheral eosinophilia is when your eosinophils are more than 500 per microliter. And the HES, or hyper eosinophilic syndrome, is defined when you have peripheral eosinophilia more than 1,500. And BAL eosinophilia is when you have eosinophils more than 25%. This patient neither had eosinophils on BAL, nor did he had eosinophils in peripheral bloodstream. It was barely 150 in 2019. And during his admission phase, it was barely between, or less than 50. I would want to talk a bit about the life cycle of eosinophils with you guys, so that you know that what's the difference between the peripheral and the tissue eosinophilia. So eosinophils arise from granulocyte macrophage precursor, and GATA1 signaling is required for it to make into the eosinophilic precursor. And eosinophils have a pretty short lifespan in the peripheral bloodstream. They get attracted to the peripheral bloodstream by eotaxin, which is the CCR3. They remain in the bloodstream for about three hours to 24 hours, and ultimately their home is peripheral tissues, where their lifespan is unknown. And the cells that are responsible for their survival in peripheral tissues are the T helper 2 cells and innate lymphoid cell 2, which we also talked to you about as a T2 immunity. And they secrete this potent interleukin 5, which is responsible for eosinophils to mature, to survive, and to proliferate in the peripheral tissues. And majority of our eosinophils remain in your small and large intestine, which is about 5 to 25%. And a small amount of it are there in your lungs, mammary gland, uterus, thymus, and adipose tissue based on your developmental and your hormonal cycle. And mostly in the lungs, they are in your alveolarization phase. And so switching gears and talking more about how eosinophils affect eGPA, typically the classification of eGPA that was recently published by ULAR in 2022 gives you five points. If you have peripheral eosinophils more than 1,000, this patient did not meet that criteria. And this patient also was ANCA negative. And I have not had any case reports in which patients were eosinophil negative and ANCA negative and went through this catastrophic event in their lifetime. So we do have ANCA negative eGPA because ANCA positivity is only in about 40% of individuals. ANCA negative individuals present as eosinophilia, and we already know the three phases in which you have first the chronic rhinocytous phase, then the eosinophilic phase, and then the vasculitis phase. So in the ANCA negative subgroup, there is more so predominance of cardiomyopathy and glomerulonephritis. And in ANCA negative group, there is more so predominance of glomerulonephritis, peripheral neuropathy, and purpura. For the management of eGPA, we divide the groups into severe and non-severe. The severe groups are classified based on the five-factor score, and you get that score if you're age more than 65, if you have cardiac involvement, and you have renal involvement. So it's a cumulative score, and if you score more than one, or if you have a catastrophic event like diffuse alveolar hemorrhage or any other organ life-threatening manifestation, then you're started on glucocorticoids plus cyclophosphamide. And ultimately, for the remission, you remain on the glucocorticoids plus rituxan and or mepolizumab these days. For the non-severe cases, as in our patient, it was severe to begin with, but just by starting pulse-toe steroids, the EF completely resolved. He was not hypoxemic, so he was just put on glucocorticoids, and there were some talks with rheumatology to maybe consider cyclophosphamide if he was to deteriorate, but he remained pretty well controlled with glucocorticoids and now he's about two years post this course, and he's still on 15 of PRED, and he has a prior auth pending for starting him on mepolizumab. I'll briefly mention what mepolizumab is. It's a monoclonal antibody, and since we know that interleukin-5 is the interleukin that's responsible for eosinophilic maturation and being there in the tissues, this monoclonal antibody binds to the IL-5 receptors that are present on the eosinophil that inhibit their proliferation and survival in the tissues. And it has been shown, this is a 2017 NEGM article, which has shown that if you put patients on mepolizumab as compared to placebo, first of all, they have more chances of remission at both 36 and 52 weeks. They have more cumulative weeks on remission, and they also have decreased dose of glucocorticoids because 44% of people on mepolizumab group had their glucocorticoid dose reduction to less than four milligram, and only 4% in placebo group could reduce their glucocorticoid dose to less than four milligram per day. So in conclusion, this is a pretty rare event to happen. I did not find any other case reports in which patients had almost zero to minimal eosinophilia in the peripheral bloodstream. So this made this diagnosis pretty challenging and also associated with eosinophilic myocarditis as it was ANCA negative in setting of normal eosinophils and vasculitic labs. So history, patient presentation can give you a bit of clue of multisystem involvement. So in this case, as I've said, biopsy was pretty important early on for his diagnosis and his survival. Thank you. Thank you very much. So the next presenter will be Dr. Kim, constrictive pericarditis masquerading as liver failure. Good morning. My name is Catherine and I'm a second year pulmonary critical care fellow at VCU. And my case today is constrictive pericarditis masquerading as liver failure. I have no disclosures. To introduce our case, we have a 71 year old male veteran who presented to the emergency department with 18 months of shortness of breath, abdominal distension and early satiety. He had no significant medical history and he was a lifelong non-smoker. He previously worked at a Honeywell chemical plant for decades before he retired. He was an army veteran who served in the Vietnam War. Extensive workup had been done over the past 18 months by outside specialists. Cardiac workup revealed non-obstructive coronary artery disease and transthoracic echocardiogram was reported to be unremarkable. His PFTs were normal, but he was trialed on ICS lava inhaler therapy for possible asthma, but he had no symptomatic improvement and chest imaging was unrevealing. He subsequently developed abdominal discomfort and early satiety. LFTs were consistent with the cholestatic pattern of liver injury and abdominal MRI revealed advanced liver fibrosis without any biliary pathology. The plan was for an outpatient transjugular liver biopsy to further evaluate the etiology of his liver fibrosis, but the patient's shortness of breath became unbearable and he presented to our ED. His vital signs were stable in our emergency department. Physical exam was notable for jugular venous distension. He had decreased breath sounds in the right lung base and, of course, he had abdominal distension. Labs were notable for thrombocytopenia, which was chronic. AST and ALT were normal with elevation in his alk-fos and T-belly, which was consistent with that cholestatic liver injury. CTHS was negative for pulmonary embolism, but it did show a new right-sided pleural effusion. Pulmonary was consulted at this time for thoracentesis to confirm the suspicion that the effusion was a hepatic hydrothorax secondary to his liver fibrosis. We reviewed the imaging and we made note of the right-sided effusion, but what was really striking to us on imaging was the degree of IV contrast reflux into the IVC extending all the way into the hepatic veins, which is visible on that CT scan. At this point, we took a diagnostic pause. The primary focus up until this point was regarding his liver fibrosis, and they had sent extensive lab work, and GI was working on expediting his liver biopsy. His CT findings, however, were suggestive of congestive hepatopathy secondary to a cardiac etiology. After taking this pause and thinking through the diseases associated with contrast reflux, we recommended reevaluation of his heart with another echocardiogram. In the meantime, we are pulmonologists, so we did perform a thoracentesis for the new pleural effusion. The pleural effusion was transudative by Leitz criteria and Gram-Stain culture and cytology were all negative. The read on the echocardiogram actually was normal other than showing a severely dilated IVC. These findings were discordant with the CT findings, and because our suspicion for cardiac etiology was still high, we requested a right heart catheterization. The right heart catheterization had several significant findings. We saw prominent X and Y descents on the right atrial pressure tracing, which is labeled in red. The sharp and deep Y descent, also known as phragic sign, reflects the rapid ventricular filling in early diastole. Moving to the right ventricular pressure tracing, we saw the dip and plateau sign, otherwise known as the square root sign, which corresponds to the rapid early diastolic filling, followed by an abrupt halt or plateauing, which occurs once the ventricle has reached a set volume that's dictated by a stiff pericardium. And finally, up in the right corner, you could see that there was equalization and elevation of his diastolic pressures. These findings together raised our suspicion for constrictive pericarditis, and so there was a second echocardiogram that was done with very focused imaging to look for findings consistent with constrictive pericarditis. Looking at the four-chamber view, imaging of the septum showed abnormal beat-to-beat septal motion, which is known as a septal shutter or a septal bounce. There was also evidence of ventricular interdependence, which can also be appreciated on the four-chamber view. You can see shifting of the septum into the left ventricle with inspiration. Other findings that we saw were annulus reverses and expiratory reversal of the hepatic vein flow. We did do a confirmatory cardiac MRI, and there was pericardial thickening along the right atrial and ventricular free wall, but with only minimal pericardial enhancement. Although the pericardial thickening was not as impressive as we had hoped, the array of findings on the echo, the right heart cath, and the cardiac MR was convincing enough to make the diagnosis of constrictive pericarditis. Regarding our management strategy, due to its significant functional limitations and the severity of his liver dysfunction, we referred the patient to cardiothoracic surgery. He underwent surgical pericardiectomy, and he had complete resolution of his shortness of breath and fatigue. He came off his diuretics very quickly, and his liver enzymes normalized. Quickly on constrictive pericarditis, it is a rare cause of heart failure that's characterized by a stiff and noncompliant pericardium that impedes ventricular filling. Most cases are idiopathic or post-viral, but constrictive pericarditis can also occur as a sequela of cardiac surgery or mediastinal radiation. Tuberculosis remains the leading cause in developing countries. This case is a prime example of the diagnostic conundrum that often arises with constrictive pericarditis. Patients often have an insidious disease course, and they present with nonspecific symptoms, and this is compounded by the fact that unfortunately there is no single pathopneumonic finding for the diagnosis of constrictive pericarditis. And this knowledge of the hemodynamic alterations and its manifestations on invasive and noninvasive tests can aid in early detection. One final point I did want to touch on is premature closure and its pitfalls during the diagnostic process. And so early closure can be avoided by keeping a broad differential that evolves with the discovery of new information and by using a comprehensive approach when synthesizing data. Diagnosis of rare diseases such as constrictive pericarditis can be exceptionally difficult, and it requires a high degree of suspicion. Prompt recognition followed by definitive treatment with pericardiectomy can result in dramatic improvement, as we saw in our patient. And on the right you can see his postoperative echocardiogram, where the septal bounce has mostly resolved and the ventricular interdependence is no longer there. And that's it. Thank you very much. So we have no more presentations unless somebody else wants to present something. And this concludes the session. Thank you very much for attending this session, and I wish you aloha and enjoy Hawaii and the rest of this conference. Thank you. ♪

presenters

cardiac arrest

amniotic fluid embolism

extracorporeal membrane oxygenation

pericardiectomy

diagnosing

rare diseases