Report of the Association Between Multisystem Inflammatory Syndrome and Severe Myocarditis

Introduction: The people worldwide have been affected by severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection since its appearance in December, 2019. Kawasaki disease-like hyperinflammatory shock associated with SARS-CoV-2 infection in previously healthy children has been reported in the literature, which is now referred to as a multisystem inflammatory syndrome in children (MIS-C). Some aspects of MIS-C are similar to those of Kawasaki disease, toxic shock syndrome, secondary hemophagocytic syndrome, and macrophage activation syndrome. Case Presentation: This study reported an 11-year-old boy with MIS-C presented with periorbital and peripheral edema, abdominal pain, elevated liver enzymes, severe right pleural effusion, moderate ascites, and severe failure of right and left ventricles. Conclusion(s): Due to the increasing number of reported cases of critically ill patients afflicted with MIS-C and its life-threatening complications, it was recommended that further studies should be carried out in order to provide screening tests for myocardial dysfunction. Adopting a multidisciplinary approach was found inevitable.Copyright © 2023, Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

Cytokine elimination in suspected COVID-19 perymyocarditis

Background: Only recently studies have been able to demonstrate the safety and efficacy of purification therapies in inflammatory diseases. Here we present the management of a young (21y) male patient in severe cardiogenic shock due to COVID-19 perymyocarditis admitted to the ICU at Bolzano Central Hospital. November 30th 2020 the patient developed high fever (>40 C) and diarrhea. After unsuccessfully being treated orally with a macrolide he was admitted to a peripheral hospital the 4th of December. The day after he deteriorated, required transfer to the ICU, endotracheal intubation and pharmacological cardiovascular support (Norepinephrine, Levosimendan). Antimicrobial treatment was started with piperacillin/tazobactam, linezolid and metronidazole. Despite multiple radiological and microbiological diagnostic attempts the origin of this severe septic shock remained unclear. December 6th the patient was transferred to Bolzano Central Hospital for VA-ECMO evaluation. Method(s): The transesophageal echocardiography revealed 15-20% of EF, lactate (5,2 mmol/l), cardiac enzymes (TropT 1400 mcg/l) and inflammatory parameters (PCT 35 ng/ml, IL-6 685 pg/ml) were elevated. We performed cardiac monitoring via Swan-Ganz catheter. The cardiac index was 1,6 l/min/m2. The peak dosage for Norepinephrine reached 7,5mg/h (1,47 mcg/kg/min). At Bolzano ICU we facilitate the pharmacological therapy with milrinone, vasopressin and low dose epinephrine. Furthermore, we impost continuous hemodiafiltration with CytoSorb filter. Result(s): Only hours after the start of filtration therapy the patient improved and we were able to gradually reduce catecholamine therapy, lactate values decreased. A VA-ECMO implantation was no more necessary. December 10th, we saw a stable patient without ventilatory or cardiovascular support, at echocardiography we revealed a normal EF. Conclusion(s): Clinically we saw a young patient in severe septic/cardiogenic shock due to perimyocarditis. Yet diagnostic attempts (CT-scan, multiple blood/urinary/liquor cultures) remained negative. Despite multiple negative PCR tests for SARS-CoV2 infection we performed specific immunoglobulin analysis and received a positive result for IgM. We therefore conclude on a COVID-19 associated perymyocarditis. Furthermore, this case illustrates the potential benefit of cytokine filtration and elimination in COVID-19 patients with altered IL6 levels.

Uncontrolled Acromegaly Resulting in the Need for LVAD as Bridge to Heart Transplant

Introduction: Acromegaly is an uncommon pituitary disorder with an incidence of six per million persons. While hypertension is often encountered in these patients, heart failure rarely is seen with an incidence rate under 10%. We describe a case of an individual who was diagnosed with acromegaly after an acute exacerbation of heart failure with subsequent management requiring an LVAD to perform Transsphenoidal Surgery (TSS). Case Description: 37-year-old male otherwise healthy initially presented to an emergency room and was found to be in acute heart failure exacerbation. Concerning acromegaly features included macrognathia, enlarged hands and feet, swollen phalanges, widened spacing of teeth, and frontal bossing. IGF-1 level was found 455 ng/mL. MRI showed a 10mm macroadenoma. A right heart catheterization showed elevated filling pressures. Cardiac MRI showed no defects or enhancement. Endomyocardial biopsy showed no inflammatory infiltrates or evidence of infiltrative diseases. Patient had an ejection fraction of 15% corroborated by cardiac MRI along with the presence of aortic root dilatation and mitral regurgitation. The patient started on 0.5mg of Cabergoline twice weekly and 120mg weekly Lanreotide injections. Patient stabilized with plans for further close monitoring and outpatient neurosurgical evaluation. The COVID-19 pandemic and insurance gaps led the patient to spend two years off his medicines and he was unable to be seen by his medical team. Patient was seen by our system after recurrent hospitalizations for heart failure at our sister hospital, AICD was unable to be placed due to the patient's anatomy, he was placed on wearable cardiac defibrillator and required milrinone infusion for progression to end-stage heart failure with cardiac cachexia. At our institution, the patient was evaluated for Orthotopic Heart Transplant (OHT) but due to active GH secreting macroadenoma there was concern for OHT failure without TSS. Decision was made to utilize LVAD as Bridge-to-Transplant for OHT so the patient could be stabilized and safely undergo TSS. The patient tolerated surgeries well and is currently on the active transplant list. Discussion(s): Heart failure is an uncommon presentation of severe acromegaly requiring multidisciplinary management. We describe a case of a patient who initially presented with heart failure too unstable for surgery. Due to the COVID-19 pandemic the patient's disease progressed resulting in end-stage heart failure requiring LVAD placement for further treatment. We would like to draw attention to the use of LVAD placement in acromegalic patients who develop severe cardiovascular disease who are not candidates for OHT.Copyright © 2023

Atrio-Ventricular Conduction Abnormalities in Multisystem Inflammatory Syndrome in Children

Purpose of study Since mid-April 2020 in Europe and North America, clusters of pediatric cases with a newly described severe systemic inflammatory response with shock have appeared. Patients had persistent fevers >38.5 C, hypotension, features of myocardial dysfunction, coagulopathy, gastrointestinal symptoms, rash, and elevated inflammatory markers without other causes of infection. The World Health Organization, Centers for Disease Control, and Royal College of Paediatrics associated these symptoms with SARS-CoV-2 as multisystem inflammatory syndrome in children (MIS-C). Cardiac manifestations include coronary artery aneurysms, left ventricular systolic dysfunction evidenced by elevation of troponin-T (TnT) and pro-B-type naturietic peptide (proBNP), and electrocardiogram (ECG) abnormalities. We report the clinical course of three children with MIS-C while focusing on the unique atrioventricular (AV) conduction abnormalities. Case #1:19-year-old previously healthy Hispanic male presented with abdominal pain, fever, and non-bloody diarrhea for three days. He was febrile and hypotensive (80/47 mmHg) requiring fluid resuscitation. Symptoms, lab findings, and a positive COVID-19 antibody test were consistent with MIS-C. Methylprednisolone, intravenous immunoglobulin (IVIG), and enoxaparin were started. He required epinephrine for shock and high flow nasal cannula for respiratory distress. Initial echocardiogram demonstrated a left ventricular ejection fraction (LVEF) of 40% with normal appearing coronaries. Troponin and proBNP were 0.41 ng/mL and proBNP 15,301 pg/mL respectively. ECG showed an incomplete right bundle branch block. He eventually became bradycardic to the 30s-50s and cardiac tracing revealed a complete AV block (figure 1a). Isoproterenol, a B1 receptor agonist, supported the severe bradycardia until the patient progressed to a type 2 second degree AV block (figure 1b). A second dose of IVIG was administered improving the rhythm to a type 1 second degree AV block. An IL-6 inhibitor, tocilizumab was given as the rhythm would not improve, and the patient soon converted to a first-degree AV block. Cardiac magnetic resonance imaging showed septal predominant left ventricular hypertrophy and subepicardial enhancement along the basal inferior/anteroseptal walls typical for myocarditis. Case #2: 9-year-old previously healthy Hispanic male presented after three days of daily fevers, headaches, myalgias, diffuse abdominal pain, and ageusia. He was febrile, tachycardic, and hypotensive (68/39 mmHg). Hypotension of 50s/20s mmHg required 3 normal saline boluses of 20 ml/kg and initiation of an epinephrine drip. Severe hypoxia required endotracheal intubation. After the MIS-C diagnosis was made, he was treated with IVIG, mehtylprednisolone, enoxaparin, aspirin, and ceftriaxone. Due to elevated inflammatory markers by day 4 and patient's illness severity, a 7-day course of anakinra was initiated. Initial echocardiogram showed mild tricuspid and mitral regurgitation with a LVEF of 35-40%. Despite anti-inflammatory therapy, troponin and proBNP were 0.33 ng/mL and BNP of 25,335 pg/mL. A second echocardiogram confirmed poor function so milrinone was started. Only, after two doses of anakinra, LVEF soon normalized. Despite that, he progressively became bradycardic to the 50's. QTc was prolonged to 545 ms and worsened to a max of 592 ms. The aforementioned therapies were continued, and the bradycardia and QTc improved to 405 ms. Patient #3: 9-year-old African American male presented with four days of right sided abdominal pain, constipation, and non-bilious non-bloody emesis. He had a negative COVID test and unremarkable ultrasound of the appendix days prior. His history, elevated inflammatory markers, and positive COVID- 19 antibody were indicative of MIS-C. He was started on the appropriate medication regimen. Initial ECG showed sinus rhythm with normal intervals and echocardiogram was unremarkable. Repeat imaging by day three showed a decreased LVEF of 50%. ECG had since changed to a right bundle branch block. Anakinra as started and steroid dosing was increased. By day 5, he became bradycardic to the 50s and progressed to a junctional cardiac rhythm. Cardiac function normalized by day 7, and anakinra was subsequently stopped. Thereafter, heart rates ranged from 38-48 bpm requiring transfer to the pediatric cardiac intensive care unit for better monitoring and potential isoproterenol infusion. He remained well perfused, with continued medical management, heart rates improved. Methods used Retrospective Chart Review. Summary of results Non-specific T-wave, ST segment changes, and premature atrial or ventricular beats are the most often noted ECG anomalies. All patients initially had normal ECGs but developed bradycardia followed by either PR prolongation or QTc elongation. Two had mild LVEF dysfunction prior to developing third degree heart block and/or a junctional escape rhythm;one had moderate LVEF dysfunction that normalized before developing a prolonged QTc. Inflammatory and cardiac markers along with coagulation factors were the highest early in disease course, peak BNP occurred at approximately hospital day 3-4, and patient's typically had their lowest LVEF at day 5-6. Initial ECGs were benign with PR intervals below 200 milliseconds (ms). Collectively the length of time from initial symptom presentation till when ECG abnormalities began tended to be at day 8-9. Patients similarly developed increased QTc intervals later in the hospitalization. When comparing with the CRP and BNP trends, it appeared that the ECG changes (including PR and QTc elongation) occurred after the initial hyperinflammatory response. Conclusions Although the mechanism for COVID-19 induced heart block continues to be studied, it is suspected to be secondary to inflammation and edema of the conduction tissue. Insufficiency of the coronary arterial supply to the AV node and rest of the conduction system also seems to play a role. Although our patients had normal ECG findings, two developed bundle branch blocks prior to more complex rhythms near the peak of inflammatory marker values. Based on the premise that MIS-C is a hyperinflammatory response likely affecting conduction tissue, our group was treated with different regimens of IVIG, steroids, anakinra, and/or tocilizumab. Anakinra, being an IL-1 inhibitor, has been reported to dampen inflammation in viral myocarditis and tocilizumab has improved LVEF in rheumatoid arthritis patients. Based on our small case series, patient's with MISC can have AV nodal conduction abnormalities. The usual cocktail of IVIG and steroids helps;however, when there are more serious cases of cardiac inflammation, adjuvant immunosuppresants like anakinra and toculizumab can be beneficial. (Figure Presented).

Effusive Constrictive Pericarditis, a Heart Failure Mimicker

Background Effusive constrictive pericarditis can initially mimic heart failure and ultimately result in cardiogenic shock. Case Patient is a 57-year-old female with history of recent massive pulmonary embolism status post systemic alteplase, chronic diastolic heart failure, and history of COVID-19 infection presenting with increasing dyspnea on exertion and weakness despite compliance to outpatient diuretics. Patient was noted to be hypotensive, and fluid overloaded on exam. Decision-making Due to concern for constriction right heart catheterization (RHC) was completed and showed cardiac index of 1.1 with elevated filling pressures, discordant variation of right ventricle (RV) and left ventricle (LV) pressure tracings, diastolic equalization of pressure, and dip and plateau pattern of RV and LV diastolic tracing suggestive of constrictive physiology. Transesophageal echocardiogram showed no pericardial effusion with increased echo-density of the pericardium. Cardiac MRI showed mild diffuse thickening and subtle enhancement of the pericardium with septal bounce and no significant pericardial effusion consistent with constrictive pericarditis. Due to persistent hypotension requiring milrinone infusion, the patient underwent pericardiectomy with improvement of hemodynamics and symptoms. Conclusion Effusive constrictive pericarditis can mimic heart failure and should be ruled out in those with evidence of low cardiac output to avoid cardiovascular morbidity and mortality. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

Milrinone-induced platelet inhibition in patients supported on veno-aterial extracorporal membrane oxygenation: a case series

Case Report: Purpose: Milrinone is an inodilator that is used in the treatment of cardiogenic dysfunction and shock. It causes increased cardiac output by stimulating myocardial contractility, enhancing cardiac relaxation, and reducing afterload via phosphodiesterase III inhibition, preventing cyclic adenosine monophosphate (cAMP) degradation. Increased cAMP concentrations are known to inhibit platelet aggregation. Veno-arterial-extracorporeal membrane oxygenation (VA-ECMO) is an extracorporeal treatment option for inotrope-refractory cardiogenic shock and is often used in conjunction with inodilators. Often, patients supported on ECMO require systemic anticoagulation to prevent clotting complications. Therefore, thromboelastography (TEG) with platelet mapping is used to help gauge a patient's clotting status and gives clinicians information about the degree of platelet inhibition present. We present the case of two patients, both supported on VA-ECMO, who developed platelet inhibition with clinically significant bleeding while on milrinone, requiring the cessation of the milrinone infusion. Cases: First, we present an adult female in her fourth decade of life who required VA-ECMO for Covid-19 ARDS and cardiogenic shock. TEG platelet mapping was obtained for clinically significant bleeding from her trachea and gastrointestinal tract. Ten days after starting milrinone, adenosine-5'-diphosphate (ADP) inhibition was elevated at 67.4% and arachidonic acid (AA) inhibition normal at 1.8%. Twenty days after starting milrinone, ADP inhibition was 93.3% and AA inhibition was 76.4%. Milrinone discontinued and repeat TEG platelet mapping (10 days after discontinuation) showed ADP inhibition of 76.8% and AA inhibition of 0%. Her lowest ADP inhibition was 41.9%, approximately 1 month after milrinone discontinuation. Milrinone again attempted and ADP inhibition was 87.9% and AA inhibition 89.2% within 24 hours of initiation. No data available for platelet inhibition prior to starting milrinone. Next, we present a 9 year old female with acute myeloid leukemia who required VA-ECMO for septic shock. Initial TEG platelet mapping, obtained 2 days after milrinone initiation, showed ADP inhibition of 43.6% and AA inhibition of 98.7%. Two days after discontinuation of milrinone, her ADP inhibition was 19.6% but AA inhibition remained elevated at 91.9%. However, after 4 days off milrinone, her ADP inhibition was normal at 15.5% and AA inhibition mildly elevated at 33.6%. No data available for platelet inhibition prior to starting milrinone. Conclusion(s): Milrinone is a known platelet inhibitor due to increased intracellular cAMP concentrations. For patients on ECMO and milrinone, care should be given to the degree of platelet inhibition and potential risk of clinically significant bleeding. Further studies are needed to further investigate the correlation between milrinone, platelet inhibition, and clinically significant bleeding in ECMO patients. Copyright © 2023 Southern Society for Clinical Investigation.

Tocilizumab as a promising treatment for MIS-C in JIA: Implications for countries with inaccessible anti-IL1 agents

Background: Multisystem inflammatory syndrome in children (MIS-C), causing high morbidity and mortality, is the hyperinflammatory response following COVID-19 infection (CI). According to the MISC management guideline, Anakinra (anti-IL1) is the preferable agent among other biologic agents: Infliximab, Tocilizumab (TCZ), and baricitinib if the patient is refractory to intravenous immunoglobulin (IVIG) and systemic corticosteroid (CS). However, these are not available in a number of countries, including Thailand. Our case represents refractory MIS-C in a systemic juvenile idiopathic arthritis (SJIA) patient responding well to TCZ. Method(s): Diagnostic investigations, including basic and immunological blood tests, and echocardiography assessment, were conducted. Result(s): A 12-year- old boy has been diagnosed with SJIA since he was 2 years old, according to the presentation of prolonged fever, hepatomegaly, and evanescent rash. CS, cyclosporin-a, and TCZ have been prescribed, and he has been in clinical remission off medication for two years. He experienced acute fever, rash, shortness of breath, nausea and vomiting for few days. Physical examination revealed a febrile boy with respiratory failure, compensated shock, and a generalized persistent maculopapular rash. The other was unremarkable. MIS-C was one of the possible diagnoses according to fever accompanied by more than two systems involved and his previous CI four weeks prior. Laboratory investigation revealed an elevated inflammatory response (Figure 1). The echocardiography was done by an experienced cardiologist with concern for myocardial dysfunction in MIS-C and showed a significant poor ejection fraction of the left ventricle of 42% under dobutamine, milrinone, and norepinephrine. Broad spectrum antibiotics and IVIG (1 g/kg/dose for two days) were initiated. After hemoculture did not report bacteria growth, pulse intravenous methylprednisolone (IVMP) 1000 mg for 3 days was given for the MIS-C treatment. After initial aggressive treatment with IVIG and pulse IVMP, the patient still has a high grade fever with laboratory revealed ongoing elevated inflammatory markers. The other possible causes of fever, such as infection and active SJIA were suspected. Immunological profiles returned with positive SAR-COV2 IgG, negative SAR-COV2 IgM, which confirmed the diagnosis of MIS-C with refractory to IVIG and CS. After multidisciplinary team discussion, TCZ was given. He had neither fever, dyspnea, nor heart failure. His clinical condition gradually improves together with laboratory parameters (Figure 1). Conclusion(s): In conclusion, our case demonstrated TCZ as a potential therapeutic agent in refractory MIS-C patients living in countries with limited access to anti-IL1 agents. The multidisciplinary care team together with prompt management is advisable to the best benefit of the patient. (Figure Presented).

Impact of Paediatric Multisystem Inflammatory Syndrome Temporally Associated with Covid-19 (Pims-Ts) on Critical Care - an Hdu Experience

Aims Background Alder Hey is a tertiary children's hospital in North-West England with co-located Intensive Care and High Dependency units, covering North West England, North Wales and Isle of Man. PIMS-TS is a new multisystem inflammatory condition which has led to an increased demand on critical care beds. Some children presenting with PIMS-TS need haemodynamic support in the form of inotropes, which would traditionally need an PICU bed. Aim Review of all patients managed on Critical Care with PIMS-TS. Methods All patients in the region were discussed in a PIMSTS multidisciplinary meeting attended by Paediatrics, Infectious Diseases, Rheumatology, Cardiology and Critical Care daily. Patients across the region needing haemodynamic support or cardiology evaluation were highlighted as, in need of either HDU or PICU bed and transferred by the North West & Wales Paediatric Transport Service (NWTS). This is a retrospective analysis of all children admitted to HDU or PICU with a diagnosis of PIMS-TS, from October 2020-December 2021. Results Thirty (10%) patients were admitted to HDU from the 300 patients discussed over the 15month period. 16 (53%) of patients were female. Mean age was 10 years (range 3-17). Median length of stay (LOS) on HDU was 2 days (range 1-8) with a median hospital LOS of 6 days (range 2- 10). All patients admitted were monitored appropriately and had full echocardiography assessment. Twenty nine (97%) patients admitted to HDU required inotropic support, twelve (40%) patients required a single agent and seventeen (57%) required double agents with a combination of adrenaline, noradrenaline and milrinone. Median fluid resuscitation was 40mls/kg (range 20-70mls/ kg). Eight patients (27%) were escalated to PICU for either invasive ventilation (4) or higher inotropic requirements of 0.2micrograms/kg/minute. There were no adverse events. Conclusion Most children with PIMS-TS have low to moderate haemodynamic instability that can be safely managed on HDU with appropriate monitoring and agreed limits to vasopressor therapy. Our experience in managing with these patients successfully and safely in a high dependency setting has helped in the use of a critical care bed efficiently, thus reducing dependency on the availability of a PICU bed.

Inhaled Milrinone via HFNC as a Postextubation Cardiopulmonary Elixir: Case Series and Review of Literature

Pulmonary hypertension (PH) often complicates perioperative course following pediatric cardiac surgery, often presenting unique challenges to the attending cardiac anesthesiologist. Apart from difficult weaning from cardiopulmonary bypass, PH can often compound weaning from mechanical ventilation in this postoperative subset. From pathophysiological standpoint, the former can be attributed to concurrent detrimental cardiopulmonary consequences of PH as a multisystemic syndrome. Therefore, with an objective to address the affected systems, that is, cardiac and pulmonary simultaneously, we report combined use of inhaled milrinone (a pulmonary vasodilator) through high-frequency nasal cannula (oxygen reservoir and continuous positive airway pressure delivery device), purported to complement each other's mechanism of action in the management of PH, thereby hastening postoperative recovery. This article additionally presents a nuanced perspective on the advantages of combining the aforementioned therapies and hence proposing the same as a possible postoperative cardiopulmonary elixir.

Single-centre study: MIS-C impact on blood pressure, coronary arteries and left ventricular function

Background and Aim: MIS-C is a hyperinflammatory syndrome caused by Sars-CoV-2 virus. Cardiovascular system impairment is observed up to 100 % of all MIS-C patients with a wide spectrum and severity of symptoms. It is important to identify the course of the disease and its outcome, which could significantly improve public health. Method(s): A single-centre study, prospective cohort study, con-ducted in the Children's Clinical University hospital in Latvia from January to December 2021. Patients between the ages of one to seventeen years who met the MIS-C criteria were included in the study. We evaluated blood pressure, left ventricular heart func-tion, size of coronary arteries and hospital course. Result(s): Thirty-one patients were included who met the MIS-C criteria. The median age was 8.0 years, 52% were boys. Of all patients 77% initially presented with hypotension of whom 42% required inotropic support. Treatment in PICU was required in 58% of all patients. Reduced left ventricular ejection fraction was observed in 35% of all patients. Mildly decreased ventricular ejection fraction (lt;55%) was observed in 19% of cases but mod-erate dysfunction (ejection fraction lt;45%) was observed in 16% of patients. Twelve percent of patients received milrinone to improve left heart function. Left heart function significantly improved in all patients during the hospitalisation. In 6 % of all patients coronary artery dilations was observed. All patients had dilations resolution at the time of discharge. Median length of hospitalisation was twelve days and median length of PICU stay was three days. Conclusion(s): All patients cardiovascular symptoms had resolved at the time of discharge. Whether patients will have chronic cardiac impairment is unknown therefore it is crucial to perform long-term follow-up.

Clinical features and treatment of mixed cardiogenic and distributive shock in patients with multisystem inflammatory syndrome in children (MIS-C)

Background and Aim: Mixed shock in multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 is con-sequence of acute heart failure, inflammation-induced vasodilation and potential volume loss. Method(s): Retrospective analysis included 25 patients (7 girls) with MIS-C-related combined shock, treated in period from April 2020 to December 2021. Result(s): Mean age of patients was 12.6 +/- 4.0 years. Admission was 6.1 +/- 1.6 days after symptoms onset. Systemic inflammatory response was manifested with neutrophilia (10.7 +/- 4.2 x109/), lymphopenia (1.1 +/- 0.7 x109/L), elevated CRP (220.9 +/- 86.1 mg/L), ferritin (684.5 +/- 549.5 mug/L) and D-dimer (1528 +/- 1254 ng/mL). One third of patients had acute kidney injury with glomerular filtration rate of 64 +/- 22 mL/min/1.73 m2 and urea level of 16.0 +/- 8.4 mmol/L. All patients had acute heart failure with ejection fraction 47.2% +/- 7.7% and fractional shortening 23.6% +/- 4.9%, 92% of patients had NTproBNP gt;1500 pg/mL and 58% had elevated troponin I (1.34 +/- 1.47 ng/mL). Z-scores for end-diastolic left ventricle, interventricular septum and pos-terior wall diameters were 0.7 +/- 1.1, 1.7 +/- 1.3 and 0.6 +/- 0.7 respectively. All patients had mild/moderate mitral regurgitation, and 60% had mild pericardial effusion. Inotropes, administered during first 3.7 +/- 1.6 days, were divided in three groups: 1) dop-amine (n = 14), 2) dobutamine + dopamine (n = 5), 3) milrinone +/- dopamine (n = 6). Additional treatment included diuretics and captopril. Total fluid balance (including insensible loss of 300 mL/m2/day) through days 1-7 was +860 mL/m2, +128 mL/m2,-108 mL/m2,-36 mL/m2,-306 mL/m2,-335 ml/m2,-298 ml/m2 (total-95 ml/m2). Methylprednisolone/intravenous immuno-globulin and low-molecular-weight heparin/acetylsalicylic acid were administered and fever persisted 1.2 days averagely. Oxygen supplementation was needed in 71% of patients. Transitory bradycardia was noticed and there was no difference in heart rate between treatment groups. Profound hypotension was revealed on admission and correction differed regarding treat-ment (p lt;0.05) (Figure 1). All patient survived with clinical improvement (one had mechanical ventilation, and one had stroke). Conclusion(s): Mixed shock is the most severe manifestation of MIS-C, and treatment of heart failure should be combined with cau-tious fluid resuscitation.

USE OF POINT-OF-CARE ULTRASONOGRAPHY (POCUS) TO PREVENT DILTIAZEM-INDUCED CARDIOGENIC SHOCK

SESSION TITLE: Using Imaging for Diagnosis Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Point of care ultrasonography (POCUS) uses an ultrasound technique that helps physicians augment physical examination findings and guide clinical decision-making at the bedside. We present a case that became a watershed moment for internal medicine residents at Abington Jefferson Hospital to use POCUS for every patient with atrial flutter/fibrillation with RVR prior to initiating diltiazem drip. CASE PRESENTATION: A 73-year-old male presented to the emergency department with complaints of palpitations. He was tachycardic with a heart rate in the 150s, and his rhythm was irregular. His basic labs were normal;an electrocardiogram investigation showed that he was experiencing an atrial flutter with 2:1 and 3:1 blocks. Chest X-ray was clear. He was given IV metoprolol 10 mg twice without achieving rate control and then started on a diltiazem drip, which initially improved his heart rate to 70s with rhythm changing to atrial flutter with 4:1 block. However, he started to become hypoxic, requiring intubation and then hemodynamically unstable, requiring initiation of pressors. Postintubation CXR indicated bilateral diffuse pulmonary edema and vascular congestion. Subsequently, he had Pulseless electrical activity (PEA) arrest. Return of spontaneous circulation (ROSC) was achieved after 3 minutes of chest compression and one round of epinephrine injection. Transthoracic echocardiogram showed an ejection fraction of 10%. He had a right heart catheterization which showed a CI of 1.7 and elevated PCWP and RVP. He was started on milrinone for ionotropic support and needed norepinephrine, vasopressin and phenylephrine to sustain his blood pressure. DISCUSSION: Atrial flutter and fibrillation are routinely seen arrhythmias in hospital settings. Patients with irregular rhythm who are in rapid ventricular rate and normotensive are often given IV metoprolol few times and then started on a diltiazem drip if RVR continues. Diltiazem not only decreases heart rate (negative chronotropic) but also decreases ventricular squeeze (negative ionotropic). It is contraindicated in patients with reduced ejection fraction. Patients’ ejection fraction values are not always known, especially if they have never had a transthoracic echocardiogram in the past or prior records are not available. POCUS helps physicians and residents to access and estimate LV function quickly and augments clinical decision making at the bedside. CONCLUSIONS: Internal Medicine Residents at Abington Hospital have made it a part of their protocol to always perform bedside ultrasonography in patients with atrial flutter/fibrillation with rapid ventricular rate before initiating diltiazem drip to prevent further avoidable cardiogenic shocks. Reference #1: Fey H, Jost M, Geise AT, Bertsch T, Christ M. Kardiogener Schock nach bradykardisierender Therapie bei tachykardem Vorhofflimmern : Fallvorstellung einer 89-jährigen Patientin [Cardiogenic shock after drug therapy for atrial fibrillation with tachycardia : Case report of an 89-year-old woman]. Med Klin Intensivmed Notfmed. 2016 Jun;111(5):458-62. German. doi: 10.1007/s00063-015-0089-9. Epub 2015 Oct 6. PMID: 26440099. Reference #2: Bitar ZI, Shamsah M, Bamasood OM, Maadarani OS, Alfoudri H. Point-of-Care Ultrasound for COVID-19 Pneumonia Patients in the ICU. J Cardiovasc Imaging. 2021 Jan;29(1):60-68. doi: 10.4250/jcvi.2020.0138. PMID: 33511802;PMCID: PMC7847790. Reference #3: Murray A, Hutchison H, Popil M, Krebs W. The Use of Point-of-Care Ultrasound to Accurately Measure Cardiac Output in Flight. Air Med J. 2020 May-Jun;39(3):218-220. doi: 10.1016/j.amj.2019.12.008. Epub 2020 Jan 14. PMID: 32540116. DISCLOSURES: No relevant relationships by Fnu Aisha No relevant relationships by Lucy Checchio No relevant relationships by Ans Dastgir No relevant relationships by Shravya Ginnaram No relevant relationships by Syeda Hassan No relevant relationships by Chaitra Janga No relev nt relationships by Rameesha Mehreen No relevant relationships by Rahat Ahmed Memon No relevant relationships by Binod Poudel No relevant relationships by Shreeja Shah

Cardiac Management in Pediatric COVID-19 Disease

Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can involve children of all ages, although less frequently and with a milder presentation than adults. Cardiovascular abnormalities (myocardial injury, acute myocarditis, cardiomyopathy, heart failure, arrhythmias, pericarditis, cardiogenic shock, pulmonary embolism, myocardial infarction) may accompany, especially with the multisystem inflammatory syndrome in children and adolescents (MIS-C). Severe disease is managed in the hospital setting. Supportive care is the mainstay of therapy. Antiviral therapy, immune-mediated therapies, empiric antibiotics, and therapy for influenza infection are used in selective patients. Cardiac management focuses on maintaining hemodynamic stability and providing adequate systemic perfusion. Children presenting with shock should be resurrected according to standard protocols. Vasoactive agents such as epinephrine or norepinephrine and, if possible, milrinone is used in fluid-refractory shock. Children with Kawasaki disease (KD) features should receive standard therapies for KD, including intravenous immune globulin (IVIG), aspirin, and glucocorticoids. Patients with severe LV dysfunction, intravenous diuretics and inotropic agents, such as milrinone, dopamine, and dobutamine are suggested. Continuous cardiac monitoring is essential. In cases of the fulminant disease, mechanical hemodynamic support may be necessary. For moderate or severe manifestations (shock, left ventricular systolic dysfunction, elevated troponin or brain natriuretic peptide, arrhythmia, coronary artery aneurysm, or presentations requiring PICU care), therapy with combined IVIG plus a glucocorticoid is suggested. Patients may be at risk for venous thromboembolism due to COVID- 19 associated hypercoagulability. Patients with MIS-C and those with severe LV dysfunction or CA aneurysms are at increased risk. It is suggested that all patients with MIS-C receive low-dose aspirin, and severe cases requiring PICU care receive prophylactic-dose anticoagulant therapy. Patients with current or prior VTE, severe LV dysfunction, large or giant CA aneurysms, markedly elevated D-dimer should receive therapeutic anticoagulation (low molecular weight heparin) plus aspirin. Most children with cardiac involvement have recovery of function by hospital discharge. The overall mortality rate for MIS-C is approximately 1 to 2 percent. Cardiology follow-up after discharge is recommended.

Massive pulmonary embolism post decannulation from Extra Corporeal Membrane Oxygenation: an unexpected complication

Introduction: Massive pulmonary embolism is a rare complication following Veno-Venous Extra Corporeal Membrane Oxygenation (VV-ECMO) decannulation. Management can be challenging. The authors present a case that required VV-ECMO re-cannulation and catheterdirected thrombolysis. Main body: 58-year-old gentleman, background of hypertension and asthma, admitted with severe respiratory failure secondary to COVID-19 pneumonitis. Due to lack of improvement with conventional ARDS treatment, he was referred and retrieved on VV-ECMO. After being off sweep gas for more than 24 hours he was decannulated on day 7. Five hours after decannulation the patient acutely deteriorated. He became tachycardic, hypotensive and hypoxic. A bedside TTE showed severely dilated and impaired right ventricle. The patient was started on milrinone and nitric oxide. Nevertheless, he deteriorated further and became profoundly hypoxic and hypercapnic, and a decision was made to start him on VV-ECMO. A TOE was done to guide cannulation and showed a thrombus in the RV and in the left pulmonary artery. Next day, a CT-pulmonary angiogram (CTPA) was done which showed saddle-shaped pulmonary embolism, with a large occlusive clot in the left main pulmonary artery causing complete non-perfusion of the left lung. After a multi-disciplinary team discussion, the patient had catheterdirected thrombolysis, with some haemodynamic improvement. Within 48 hours, TTE was repeated showing no significant improvement on RV function. CTPA showed very mild decrease of the clot burden. Decision was made to repeat catheter-directed thrombolysis and partial thrombectomy. Repeated imaging revealed decrease in the size of the left main pulmonary artery thrombus. It is thought that the massive pulmonary embolism could have been caused by showering of ECMO cannulas-related thrombi, which were dislodged during decannulation. Patient remained on VV-ECMO for 32 days and was decannulated successfully afterwards and was discharged home on apixaban and long-term pulmonary hypertension follow-up. Conclusion: ECMO cannulas related thrombi are not uncommon complications because of prolonged stay and coagulopathy related to ECMO circuit. However, massive embolism is rarely seen. The use of echocardiography was paramount on the differential diagnosis. In this TTE study, the right ventricle looks significantly dilated with severely impaired both longitudinal and radial functions. Additionally noted septal flattening in systole indicating RV pressure overload, diastolic notching of RVOT doppler trace consistent with significantly raised pulmonary artery pressure and mild to moderate tricuspid regurgitation. Otherwise, the left ventricle is small and has preserved function. (Figure Presented).

GETTING TO THE HEART OF THE MATTER

CASE: 45-year-old woman with PMHx systemic sclerosis presents with fever, weight loss, chest tightness, weakness and altered mental status for 2 weeks. Home meds are prednisone, mycophenolic acid, lasix. On presentation she is febrile to 38.9C, HR 110, BP 97/64, SpO2 96% on RA. Exam shows telangiectasis, normal cardiopulmonary exam, mild sclerodactyly. Oriented only to self, has bilateral LE 3/5 weakness. Labs with WBC 2.6K, Hgb 7.1, plts 126K. Cr normal. Liver enzymes mildly elevated. BNP 3900. Trop 251. Lactate 4.9 Blood cultures negative, CMV/EBV negative, COVID-19 negative, Ferritin > 15,000, Triglycerides 274 LDH 495, Fibrinogen 274, D-Dimer 755, ANA 1:1280, + dsDNA, low titer Smith, + RNP, + SSA, + RNA Pol III. TTE with EF 27% and diffuse hypokinesis. Cardiac MRI with myocardial fibrosis no active myocarditis, suggestive of scleroderma. Lumbar puncture with high protein, borderline increased oligoclonal bands, elevated IgG index but elevated synthesis rate, suggestive of CNS inflammation. Patient is in cardiogenic shock secondary to hemophagocytic lymphohistiocytosis/macrophage activating syndrome (HLH/MAS) related to systemic sclerosis/scleroderma with SLE overlap requiring inotropes and aggressive diuresis. She develops severe pain and bright red purpura on bilateral legs. Hypercoagulable w/u showed low protein C/S, low complement, negative cryoglobulin. Skin biopsy showed vaso-occlusive process c/w HLH/MAS. Receives IV methylprednisolone for empiric treatment of HLH/MAS and IV cyclophosphamide for possible lupus cerebritis. Patient improves and is discharged on long-term milrinone, Plaquenil, and steroids. IMPACT/DISCUSSION: Secondary HLH or MAS is a life-threatening condition of extreme inflammation that can occur in autoimmune conditions, infection, or malignancy Diagnosing HLH requires high clinical suspicion - >10K ferritin level is highly sensitive and specific for diagnosis of HLH This patient has multisystem involvement of autoimmune disease given history of scleroderma The LP studies raise concern for lupus cerebritis, specifically the IgG index and IgG synthesis rate are helpful for this diagnosis Underline subtype of systemic sclerosis-overlap syndromes and here particularly scleroderma lupus overlap Highlight the utility of cardiac MRI in characterizing myocarditis / fibrosis Discuss need for high alert for necrotizing fasciitis with painful palpable purpura Overview treatment of HLH/MAS with high dose steroids Reflection on high mortality of HLH/MAS and question of recovered heart function CONCLUSION: Teaching Point 1: Secondary HLH is a syndrome of extreme inflammation caused by underlying malignancy, autoimmune condition, or infection. Teaching Point 2: HLH and MAS have a great deal of symptom/clinical presentation overlap. Ferritin level > 10,000 is highly sensitive and specific for diagnosis of HLH Teaching Point 3: Systemic sclerosis can present in a variety of ways including cardiac, lung, skin involvement.

TROMBO ATRIAL DIREITO ASSOCIADO AO CATETER EM PACIENTE COM LINFOMA DE HODGKIN

Sexo feminino, 25 anos, Linfoma de Hodgkin Esclerose Nodular (IVBX) bulky mediastinal, refratário a 6 ciclos de AVD, recaída precoce após 3 ciclos de ICE, resposta parcial após 4 ciclos de Brentuximab. Em Março/2021, diagnosticada com Covid-19, RT-PCR persistentemente positivo, sendo suspenso Brentuximab. TC de Tórax de 26/04/2021, devido a piora da dispneia, com sinais de TEP acometendo as porções distais das artérias pulmonares bilaterais;massa mediastinal com 13,6 x 9,7 cm, atividade glicolítica ao PET-CT (Deauville 4). Internou na Unidade de TMO do Hospital Monte Sinai para realização de TMO Autólogo. Coleta de células tronco periféricas em 01/05/2021. Realizado ecocardiograma prévio ao condicionamento em 07/05/2021: 2 imagens hiperecogênicas intra-atriais à direita (maior com 2,7 x 1,8 cm), sugestivas de trombos intracavitários, confirmados pelo ECO-TE, associados a cateter totalmente implantável fundidos à parede atrial. Função ventricular esquerda preservada e aumento de ventrículo direito. Pela gravidade do quadro e impossibilidade cirúrgica, indicado anticoagulação plena com Heparina Não-Fracionada em BIC e condicionamento iniciado em 11/05/2021 com esquema: Lomustina, Etoposide, Ciclofosfamida, Mesna. Infusão de CTP em 16/05/2021. Período de neutropenia sem maiores intercorrências, com pega medular em 26/05/2021 e alta hospitalar em seguida. Em 11/06/2021, intercorreu com febre e bacteremia, internada para tratamento de infecção associada a catéter de curta permanência (implantado para infusão de CTP) por S. hominis sensível à oxacilina. No dia 24/06/2021, apresentou piora clínica importante da dispneia e anasarca. ECO-TE: PSAP em 90 mmHg, aumento da disfunção de VD. Nova Angio-TC de tórax sem trombos novos. Iniciado Milrinone na Unidade Coronariana e discutido cirurgia de urgência, sendo novamente contra-indicados pelas equipes assistentes. Em 27/06/2021 paciente evolui com choque obstrutivo, evoluindo a óbito em 30/06/2021. Discussão: Cateteres totalmente implantáveis podem ser em pacientes com Linfoma de Hodgkin, pois têm menos infecção em comparação com outros cateteres. As complicações associadas aos cateteres são incomuns e incluem infecções e tromboses, porém a trombose atrial direita ainda é rara e potencialmente fatal. A formação desses trombos é assintomática e altamente associado a posição da ponta do cateter no átrio direito. A incidência é incerta, variando entre 3-23%. A mortalidade geral é de 27,1% e parece estar relacionada a sua associação com TEP grave e ao tipo de tratamento instituído. A presença do trombo cardíaco é um marcador prognóstico. O manejo ainda é controverso. As opções terapêuticas incluem anticoagulação com heparina, embolectomia cirúrgica ou trombólise. Sendo este último com taxa de mortalidade de 11,3% em comparação a 28,6% com anticoagulação e 23,8% com embolectomia. No caso relatado, foi contra-indicado o tratamento com trombolítico, pelo tempo da evidência do trombo em exame de imagem (mais de 14 dias), e o procedimento cirúrgico, por paciente apresentar massa mediastinal extensa. Portanto foi optado por manter a anticoagulação com heparina não fracionada. Conclusão: O diagnóstico de trombo em átrio direito é desafiador. O receio de complicações em um paciente grave, explica o tratamento conservador utilizado. Ainda não existe um consenso sobre o tratamento mais adequado, sendo necessária a avaliação individualizada dos casos.

DON'T BLAME THE VACCINE

Background: The mRNA COVID vaccine is a rare cause of myocarditis in young patients. We describe a case of cardiogenic shock with extensive workup ruling out COVID vaccine induced myocarditis. Case: 42-year-old female who drinks 5 Monster energy drinks and 3-4 cups of coffee daily presented to the hospital with palpitations two weeks following her mRNA COVID vaccine. EKG showed atrial tachycardia with heart rates of 160 beats per minute. Adenosine and Lopressor were administered resulting in hemodynamic instability requiring norepinephrine. An echocardiogram showed dilated cardiomyopathy with ejection fraction of 15%. Right heart catheterization was performed, and the cardiac index was 1.22 L/min/m², systemic vascular resistance was 1918 dynes*sec*cm-5 and wedge pressure was 31 mm Hg. The patient was started on nitroprusside, furosemide, and milrinone drips and she began to improve. The patient was adamant the vaccine is what triggered her heart failure and extensive testing was performed to rule out COVID vaccine induced myocarditis. Workup showed normal coronary arteries and no evidence of infiltrative disease or myocarditis on cardiac MRI. The etiology was from tachycardia induced cardiomyopathy triggered by excessive stimulants and the patient had successful atrial tachycardia ablation of the right superior pulmonary vein. She was discharged on medical therapy for heart failure and advised to stop drinking energy drinks. Decision-making: Once the patient did not respond to the rate controlling agents an echocardiogram showed reduced ejection fraction. Right heart catheterization confirmed cardiogenic shock and nitroprusside and milrinone were started to help reduce afterload and improve contractility. Workup to exclude COVID induced myocarditis lead to the diagnosis of tachycardia induced cardiomyopathy and atrial tachycardia ablation was performed. Conclusion: We report a case of cardiogenic shock with workup diagnosing tachycardia induced cardiomyopathy induced from a combination of excessive monster energy drinks and coffee. She was treated successfully with afterload reduction, inotrope support, and atrial tachycardia ablation.