Acute or chronic? Failing left ventricle in a 34 year old

Submission content Introduction: An unusual case of a very young patient without previously known cardiac disease presenting with severe left ventricular failure, detected by a point of care echocardiogram. Main Body: A 34 year old previously well man was brought to hospital after seeing his general practitioner with one month of progressive shortness of breath on exertion. This began around the time the patient received his second covid-19 vaccination. He was sleeping in a chair as he was unable to lie flat. Abnormal observations led the GP to call an ambulance. In the emergency department, the patient required oxygen 5L/min to maintain SpO2 >94%, but he was not in respiratory distress at rest. Blood pressure was 92/53mmHg, mean 67mmHg. Point of care testing for COVID-19 was negative. He was alert, with warm peripheries. Lactate was 1.0mmol/L and he was producing more than 0.5ml/kg/hr of urine. There was no ankle swelling. ECG showed sinus tachycardia. He underwent CT pulmonary angiography which demonstrated no pulmonary embolus, but there was bilateral pulmonary edema. Troponin was 17ng/l, BNP was 2700pg/ml. Furosemide 40mg was given intravenously by the general medical team. Critical care outreach asked for an urgent intensivist review given the highly unusual diagnosis of pulmonary edema in a man of this age. An immediate FUSIC Heart scan identified a dilated left ventricle with end diastolic diameter 7cm and severe global systolic impairment. The right ventricle was not severely impaired, with TAPSE 18mm. There was no significant pericardial effusion. Multiple B lines and trace pulmonary effusions were identified at the lung bases. The patient was urgently discussed with the regional cardiac unit in case of further deterioration, basic images were shared via a cloud system. A potential diagnosis of vaccination-associated myocarditis was considered,1 but in view of the low troponin, the presentation was felt most likely to represent decompensated chronic dilated cardiomyopathy. The patient disclosed a family history of early cardiac death in males. Aggressive diuresis was commenced. The patient was admitted to a monitored bed given the potential risk of arrhythmia or further haemodynamic deterioration. Advice was given that in the event of worsening hypotension, fluids should not be administered but the cardiac centre should be contacted immediately. Formal echocardiography confirmed the POCUS findings, with ejection fraction <35%. He was initiated on ACE inhibitors and beta adrenergic blockade. His symptoms improved and he was able to return home and to work, and is currently undergoing further investigations to establish the etiology of his condition. Conclusion(s): Early echocardiography provided early evidence of a cardiac cause for the patient's presentation and highlighted the severity of the underlying pathology. This directed early aggressive diuresis and safety-netting by virtue of discussion with a tertiary cardiac centre whilst it was established whether this was an acute or decompensated chronic pathology. Ultrasound findings: PLAX, PSAX and A4Ch views demonstrating a severely dilated (7cm end diastolic diameter) left ventricle with global severe systolic impairment.

PPP1R13L-Related Cardiomyopathy Stability Following IL1 Inhibition

Introduction: Variants in PPP1R13L are associated with severe childhood-onset cardiomyopathy resulting in rapid progression to death or cardiac transplantation. PPP1R13L is proposed to encode a protein that limits the transcriptional activity of the NFkappaB pathway leading to elevated IL-1, IL-6, and TNF-alpha production in murine models. Optimal medical management for PPP1R13L-related cardiomyopathy is unknown. Here we report usage of a targeted anti-IL-1 immuno-modulatory therapy resulting in cardiac stabilization in a pediatric patient with congenital cardiomyopathy secondary to PPP1R13L variants. Case Report: A 4-year-old boy presented acutely with fever in the setting of persistent abdominal pain, vomiting, fatigue, and decreased appetite for two months following a mild COVID-19 related illness. Echocardiogram revealed severely depressed biventricular systolic function with an ejection fraction of 30%. Due to acute decompensated heart failure symptoms with hemodynamic instability, he was intubated and placed on continuous inotropic infusions with aggressive diuresis. Cardiac MRI demonstrated extensive subepicardial to near transmural fibrosis by late gadolinium enhancement in right and left ventricles. An implantable cardioverter-defibrillator (ICD) was placed due to frequent runs of polymorphic non-sustained ventricular tachycardia. Testing for viral pathogens was positive for rhino/enterovirus. Initial genetic testing was non-diagnostic (82-gene cardiomyopathy panel) but given the patient's significant presentation whole genome sequencing was pursued that showed two separate PPP1R13L variants in trans (c.2167A>C,p.T723P and c.2179_2183del,p. G727Hfs*25, NM_006663.4). Patient serum cytokine testing revealed elevations in IL-10 (4.7 pg/mL) and IL-1beta (20.9 pg/mL). Given the patient's tenuous circumstances and concern for continued progression of his cardiac disease, a trial of IL-1 inhibition via anakinra dosed at 3 mg/kg or 45 mg daily was initiated following hospital discharge. With approximately 6 months of therapy, the patient's cardiac function is stable with normalization of IL-10 and IL-1beta serum levels. Notably, the ventricular arrhythmia decreased after initiation of anakinra with no ICD shocks given. Therapy overall has been well tolerated without infectious concerns. Conclusion(s): In patients with PPP1R13L-related cardiomyopathy, immuno-modulatory therapies should be considered in an attempt to slow cardiac disease progression.Copyright © 2023 Elsevier Inc.

Prevalence and characteristics of newly diagnosed heart failure in patients with shortness of breath after coronavirus infection.

Aim. To determine the prevalence and show the features of the development of newly diagnosed heart failure (HF) in patients with dyspnea after a coronavirus disease 2019 (COVID-19). Material and methods. This clinical prospective observational study was conducted during 2020-2022. The study consecutively included 368 outpatients with shortness of breath, who applied to the clinic. Depending on the presence of prior COVID-19, the patients were divided into 2 groups: the first group consisted of 205 patients with shortness of breath after COVID-19, the second group - 163 patients without prior COVID-19. All patients underwent a clinical examination within 3 days after presentation with an assessment of outpatient records and other medical documents for the differential diagnosis of dyspnea. The severity of dyspnea was determined using the Modified Medical Research Council Dyspnoea Scale (mMRC). The diagnosis of HF was verified in accordance with the 2020 Russian Society of Cardiology guidelines and in some cases reclassified in accordance with the 2021European Society of Cardiology guidelines. For further analysis, 2 subgroups of patients with HF were identified depending on the presence and absence of prior COVID-19. The subgroup analysis excluded patients with acute heart failure, acute illness, and conditions requiring hospitalization and/or intensive care. Results. Among 368 patients who presented to the clinic with dyspnea during 2020-2022, 205 patients (55,7%) had COVID-19. The average period of treatment after COVID-19 was 3,5 [1,5;22,4] months. Patients after COVID-19 applied earlier after the onset of dyspnea, which is associated with higher mMRC score. The prevalence of HF among patients with shortness of breath after COVID-19 was significantly higher than in patients without this pathology in history, and amounted to 19,0% vs 9,8% (p=0,021). Prior COVID-19 increased the relative risk (RR) of HF in patients with shortness of breath by 1,7 times. RR for HF in systolic blood pressure >140 mm Hg increased by 1,9 times, while in diastolic blood pressure >90 mm Hg - by 1,9 times, with the development of a hypertensive crisis - by 28%, with a heart rate >80 bpm at rest - by 1,4 times, with the development of type 2 diabetes - by 31%, in the presence of pulmonary fibrosis - by 2,3 times. Patients with shortness of breath after COVID-19 had more severe HF, both according to clinical tests and according to the blood concentration of N-terminal pro-brain natriuretic peptide (NT-proBNP), mainly with the preserved ejection fraction (EF) with a higher prevalence of left atrial (LA) enlargement in combination with a decrease in right ventricular (RV) systolic function and its dilatation. In patients after COVID-19 in the presence of chronic kidney disease, the RR for HF increased by 4,5 times;in the presence of C-reactive protein >4 mg/l - by 1,6 times. Conclusion. Every fifth patient with shortness of breath 3,5 months after COVID-19 had more severe HF, both according to clinical tests and according to blood NT-proBNP concentration, mainly with preserved EF with a higher prevalence of LA increase in combination with a decrease in RV systolic function and its dilatation. The risk of HF is interrelated with the female sex and multiple comorbidities.Copyright © 2023, Silicea-Poligraf. All rights reserved.

Clinical evolution of patients with AF and diabetes mellitus in the COVID-19 pandemic. REFADI Registry

Introduction and objectives: To analyze the evolution of patients with atrial fibrillation (AF) and diabetes in the mid-term follow-up during the COVID-19 pandemic and to describe its impact on this population. Method(s): Multicenter and prospective registry that included patients with AF and diabetes attended in cardiology clinics. A multivariate analysis was performed to determine the variables associated with the occurrence of clinical events and mortality. Recruitment was performed in February-December 2019. Result(s): The evolution of 633 patients, 96,2% of those included in the REFADI registry with a median follow-up of 835 days was analyzed (mean age 73.8 +/- 8.5 years, 54.3% male, CHA2DS2-VASc 4,34 +/- 1,4, HAS-BLED 2,47 +/- 0,96) were analyzed. The proportion of anticoagulated patients remained constant (95.6% vs 94.5%;P = .24). There was a decrease in the prescription of vitamin K antagonists (from 31.4% to 19.7%;P < .01), and an increase in the use of direct anticoagulants (from 62.0% to 70.3%;P < .01). During the follow-up there was an increase in the prescription of SGLT2 inhibitors (from 20.0% to 25.5%;P < .01) and GLP1 agonists (from 4.2% to 9.1%;P < .01). During this period, 17.2% of patients died, the majority from cardiovascular causes, 6.4% from COVID-19, 2.8% from stroke, and 1.8% from hemorrhage. Older age, lower ejection fraction, lower hemoglobin levels, and especially lower direct anticoagulants prescription were associated with mortality. Conclusion(s): Patients with AF and diabetes have a high thromboembolic risk and a high risk of developing complications, especially of cardiovascular origin.Copyright © 2023 Sociedad Espanola de Cardiologia

Case Report of a Transfusion-Associated Circulatory Overload Leading to Cardiac Arrest in a COVID-19 Positive Patient

Although case reports have been made regarding adverse transfusion reactions, few have been made regarding blood transfusions leading to cardiac arrest. Today, we present a case of a COVID-19 positive Bahraini male, triple vaccinated, transfused with packed red blood cell (pRBC) after finding out he has low haemoglobin levels (64 g/dl) after routine laboratory investigations. During the blood transfusion, he developed hypertension, tachycardia and tachypnoea. The patient went into cardiac arrest within a few minutes of this presentation. Return of spontaneous circulation was achieved, and the patient was managed as transfusion-associated circulatory overload (TACO) with a good overall outcome.Copyright © 2023, Bahrain Medical Bulletin. All rights reserved.

A Case Report of Ivermectin-Induced Liver Failure

Introduction: Ivermectin is an antiparasitic medication that is primarily metabolized by the liver. During the COVID-19 pandemic, researchers demonstrated that Ivermectin successfully inhibited the replication of SARS-COV-2 in vivo, but current research has failed to demonstrate clinical benefit for treatment of COVID-19. Despite this, misinformation campaigns have misled patients to ingest Ivermectin at concentrations meant for domestic animals. Here, we present a case of acute liver failure secondary to the use of Ivermectin. Case Description/Methods: A 61-year-old man with medical history of ischemic cardiomyopathy with last echocardiogram showing ejection fraction at 21%, atrial fibrillation on warfarin for oral anticoagulation, and previously treated Hepatitis C presented with generalized weakness and yellowish discoloration of the skin worsening over the last two weeks. The patient denied significant alcohol use, acetaminophen use, or illicit drugs. He admitted to injecting himself with two doses of weight-based horse ivermectin, for COVID prophylaxis, two weeks prior to his presentation. Physical exam was pertinent for scleral icterus and hepatomegaly with no abdominal tenderness. Initial labs revealed elevated liver chemistries in a mixed pattern (Figure 1). Acute hepatitis panel, HSV, and CMV were negative. Hepatitis C antibodies were positive, but the patient was in sustained virologic response. Full workup for chronic liver disease was unremarkable. Ultrasound revealed hepatosplenomegaly with patent portal and hepatic vasculature. Subsequently, the patient developed hepatic encephalopathy along with his coagulopathy, raising concern for acute hepatic failure. The patient was transferred to the ICU and started on NAcetylcysteine, rifaximin, and supportive care. The patient recovered well and fortunately did not require liver transplant. Discussion(s): While the FDA recommends against the use of Ivermectin for COVID-19, many continue to inappropriately consume it. Ivermectin-induced liver failure is a rare but deadly side effect. Given our patient's rapid onset of symptoms post-self injection of Ivermectin, his liver injury was presumed to be related to Ivermectin. The drug interaction between Ivermectin and warfarin had worsened the patients coagulopathy. Physicians should be aware of the ways Ivermectin overdose may clinically present to avoid delayed treatment. This case demonstrates the detriments of perpetuation of medical misinformation to care.

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

An Eventful Rotational Atherectomy Over the Left Main Stem and Left Anterior Descending Artery in an Elderly Male

Clinical Information Patient Initials or Identifier Number: SHS Relevant Clinical History and Physical Exam: Mr. SHS was admitted in August 2022 for acute decompensated heart failure secondary to NSTEMI, complicated with ventricular tachycardia (VT). CPR was performed for6 minutes on the day of admission and was subsequently transferred to the Cardiac Care Unit. His hospital stay was complicated with Covid-19 infection(category 2b) which he recovered well from. During admission, he developed recurrent episodes of angina. Physical examination was otherwise unremarkable. His ejection fraction was 45%. Relevant Catheterization Findings: Cardiac catheterization was performed, which revealed significant calcification of left and right coronary arteries. There was a left main stem bifurcation lesion (Medina 0,1,1) with subtotal occlusion over ostial the LAD, receiving collaterals from RCA and 90% stenosis over ostial LCx. RCA was dominant, heavily calcified with no significant stenosis. He was counselled for CABG (Syntex score26) but refused. As he was symptomatic, he was planned for PCI to the left coronary system. [Formula presented] [Formula presented] [Formula presented] Interventional Management Procedural Step: The left main was engaged with a 7F EBU 3.5guiding catheter via transradial approach. Sion Blue wired into LAD and LCx. IVUS catheter couldn't cross the LAD and LCx lesions, hence we decided for up front rotational atherectomy. Sion blue was exchanged to Rotawire with the assistance of Finecross microcatheter. A 1.5mm burr was used at 180000 rpm. After the first run of rotablation, patient developed chest pain and severe hypotension (BP ranging 50/30). 4 inotropes/vasopressors were commenced. The shock was refractory hence an intraarterial balloon pump was inserted. Symptoms and blood pressure improved. Another 2 runs of atherectomy done (patient developed hypotension after each run). IVUS examination then showed calcification of proximal to mid LAD with an IVUS Calcium score of 3. LAD was further predilated with Scoreflex balloon 3.0/20mm at 8-22ATM. LCx was predilated with Scoreflex balloon 2.0/15mm at 12-14ATM. DCB Sequent Please NEO2.0/30mm was deployed at 7ATM at ostial to proximal LCx. Proximal to mid LAD was stented with Promus ELITE 2.5/32mm at 11ATM, which was then post dilated with stent balloon at 11ATM. Ostial LM to proximal LAD (overlap) was stented with Promus ELITE 4.0/28mm at 11ATM. LMS POT was then done with NC Balloon 4.0/15mm at 24ATM. LCx was rewired and kissing balloon technique with NC balloon 4.0/15mm at 14ATM (LAD) and NC balloon 2.0/10mm at 12ATM (LCx) was done, followed by a final POT with NC balloon 4.0/15mm at 14ATM. Final IVUS showed good MSA. [Formula presented] [Formula presented] [Formula presented] Conclusion(s): This patient developed hemodynamic instability with each rotational atherectomy run, hence we decided not to perform rotablation to the circumflex artery. His hemodynamic condition improved with the use of intra aortic balloon pump. IABP use can reduce procedural event rate and potentially reduce long term mortality in appropriately selected patients who are at high risk of adverse events. He was followed up a month following the procedure and remained asymptomatic. For complex, calcified coronary lesions involving the left main stem, coronary artery bypass graft surgery is an alternative option.Copyright © 2023

A Case of Post Covid-19 Endotheliitis

A 49-year-old man presented with shortness of breath and fever. He was in diabetic ketoacidosis on admission and tested positive for COVID-19 on PCR. He became bacteraemic with streptococcus pneu- moniae secondary to a super-added left lower lobe pneumonia. He developed new heart failure felt to be secondary to myocarditis, evidenced by a resolving ejection fraction throughout his admission and an unremarkable cardiac MRI. After developing confusion on the ward, a CT head and MRI brain identified a spontaneous frontal haematoma and multiple micro-haemorrhages throughout the cerebral hemi- spheres, cerebellum and the pons. Repeat MRI brain with diffusion weighted imaging identified multiple silent infarcts in the small vessel territories. Bacterial endocarditis was excluded with Cardiology input and hypoperfusion also excluded based on normotension throughout admission. The case was discussed at the Encephalitis and Neurovascular MDT meetings where MRI vessel wall imaging was reviewed and felt to represent a post-infectious endotheliitis. He was treated with intravenous methylprednisolone for 3 days and a further 5 days, due to new silent infarcts on a subsequent MRI brain, before a 10 week oral steroid taper. Multi-system complications from COVID-19 are not limited to those in the intensive care unit or with severe respiratory illness.

Post-COVID-19 cardiovascular complications and their association with clinical characteristics, symptoms and comorbidities - the LATE-COVID study

Background: Among many complications of coronavirus disease 2019 (COVID-19) there is a wide range of cardiovascular (CV) problems ranging from mild to severe ones. Even asymptomatic patients and those with mild course of COVID-19 may develop severe CV complications. Factors leading to such state have not been extensively studied so far. Purpose(s): We aimed to assess which factors were linked to the severe complications of COVID-19. Method(s): We included 200 consecutive patients admitted to the Department of Cardiology and Adult Congenital Heart Diseases of the Polish Mother's Memorial Research Institute (PMMHRI) due to post-Covid cardiovascular complications. SARS-CoV2 infection was confirmed with real-life PCR testing. Laboratory tests, 24-hour ECG monitoring and echocardiography were performed in all patients from the investigated group. For the purposes of our study severe complications were defined as: Myocarditis, a decrease of ejection fraction >10% from the pre-disease value, thromboembolic complications, angina pectoris requiring myocardial revascularization and the new onset of atrial fibrillation of supraventricular tachycardia. Some patients presented more than one of the above. Statistical analysis was performed using the software Statistica v.13 (TIBCO Software Inc., Palo Alto, CA, USA). Data were presented as mean +/-SD or median (25th- 75th percentile) for continuous variables and as proportions for categorical variables. Comparisons between groups were performed using Student's t-test for independent variables and the Mann-Whitney U test or chi2 test with Yates's correction, as appropriate. For all calculations p-values <0.05 were considered statistically significant. Result(s): Finally, we included 200 consecutive patients (aged 54+/-16 years, 76 males - 38%), hospitalized for COVID-19 complications after a median 3 (2-6) months following the acute phase of infection. On admission patients presented with dyspnea (23%), impairment of exercise tolerance (47%), chest pain (32%), increase in blood pressure (29%), palpitations (25%), weight loss (13%), brain fog (6%), general malaise (11%), headache (5%), limb pain (13%), swelling (14%). Severe complications of COVID-19 were diagnosed in 31 patients (16%).Taking into consideration symptoms, the presence of severe COVID-19 complications was significantly associated with dyspnoea and deterioration of exercise tolerance. In comparison to patients with mild complications, severe ones were linked to age (the older patients, the higher risk), previous history of heart failure and diabetes mellitus. We did not observe statistically significant differences in severity of complications depending on smoking status (Tables 1 and 2). Conclusion(s): Previous history of heart failure and diabetes mellitus as well as symptoms (dyspnoea and deterioration of exercise tolerance) along with older age are related to more severe complications following COVID- 19.

Changes in self-care maintenance during quarantine in patients with heart failure

Background: Patients with heart failure (HF) experience challenges in self-care that contribute to poor quality of life and high health care utilization. The COVID-19 pandemic required HF patients, especially living in countries with strict lockdowns and quarantines, to change their lifestyle including health promoting behaviors. Purpose(s): To assess changes in self-care maintenance in patients with HF changed during quarantine compared to before quarantine. We hypothesized that the self-care maintenance behavior physical activity was most effected during quarantine. Method(s): This is a cross-sectional survey study. From the medical chart, patients' disease severity (NYHA-class), ejection fraction and comorbidities were collected. Self-care maintenance was assessed using subscale Self-Care of Heart Failure Index 6.2. The total score ranges from 0-100, where 70 or higher is seen as having good self-care maintenance. With all the questions in the self-care maintenance scale a question was added: Did this change due to the COVID-19 pandemic Patients could answer yes or no. When patients answered yes, they were asked the elaborate. Patients self-reported physical activity, before and during the pandemic, using the International Physical Activity Questionnaire (short form). Descriptive analyses were used for self-care maintenance, content analysis was applied for the qualitative data. Paired sample t-test were performed to assess the change in the total physical activity. Result(s): In total, 120 patients with HF were included in this study (mean age was 79+/-13, 39% female, 78% NYHA-class I/II). Patients had a mean self-care maintenance of 32 (+/-6). No participant had good self-care maintenance (score of 70 or higher). Patients were non-adherent to regular physical activity (77%, n=92), keeping weight down (74%, n=89) and weighing themselves (66%, n=79) during quarantine (Figure). Behaviors which changed the most included keeping medical appointments (81%) and regular exercise (82%). Significantly higher levels of physically active was noticed before quarantine (776 Mets-minutes) compared to during quarantine (106 Mets-minutes) (p<0.01). The change in self-care maintenance was explained by change in motivation and self-confidence. Because of the pandemic and the quarantine, patients reported not being able to rely on health care and their usual social support system. Patients tried to create new habits when trying to maintain their self-care. Conclusion(s): Patients with HF reported experiencing low self-care maintenance during quarantine due to not being able to go to medical appointments and decrease in physical activity. Public health policies during crisis events such as a pandemic should strive to provide support in coping with these changes and offer alternative ways of maintaining their self-care maintenance, especially with physical activity. (Figure Presented).

Evaluation of cardiac involvement in patients with clinical post-COVID-19 syndrome

Introduction: The underlying pathophysiology of Post-COVID-19 syndrome remains unknown, but increased cardiometabolic demand and state of mitochondrial dysfunction have emerged as candidate mechanisms. Cardiovascular magnetic resonance (CMR) provides insight into pathophysiological mechanisms underlying cardiovascular disease and 31-phosphorus magnetic resonance spectroscopy (31P-MRS) allows noninvasive assessment of the myocardial energetic state. Purpose(s): We sought to assess whether Post-COVID-19 syndrome is associated with abnormalities of myocardial structure, function, perfusion and tissue characteristics or energetic derangement. Method(s): Prospective case-control study. A total of 20 patients with a clinical diagnosis of Post-COVID-19 syndrome (seropositive) and no prior underlying cardiovascular disease (CVD) and ten matching controls underwent 31P-MRS and CMR at 3T at a single time point. (Figure 1) All patients had been symptomatic with acute COVID-19, but none required hospital admission. Result(s): Between the Post-COVID-19 syndrome patients and matched contemporary controls there were no differences in myocardial energetics (phosphocreatine to ATP ratio), in cardiac structure (biventricular volumes, left ventricular mass), function (biventricular ejection fractions, global longitudinal strain), tissue characterization (T1 and extracellular volume [ECV] fraction mapping, late gadolinium enhancement) or perfusion (myocardial rest and stress blood flow, myocardial perfusion reserve). One patient with Post-COVID-19 syndrome showed subepicardial hyperenhancement on the late gadolinium enhancement imaging compatible with prior myocarditis, but no accompanying abnormality in cardiac size, function, perfusion, ECV, T1, T2 mapping or energetics. This patient was excluded from statistical analyses. (Table 1) Conclusion(s): In this study, the overwhelming majority of patients with a clinical Post-COVID-19 syndrome with no prior CVD did not exhibit any abnormalities in myocardial energetics, structure, function, blood flow or tissue characteristics.

Hypofibrinogenemia in a Patient with Cardiogenic Shock, Left Ventricular (Lv) Thrombus, Covid-19, and Severe Liver Injury: A Case Report of Complex Decision Making in the Setting of Multiorgan Failure with Unclear Causality

Background We present a case of a young male with new severe cardiomyopathy requiring critical care within 24 hours. Case A Latino male with alcoholism was admitted for COVID and severe liver injury due to alcohol-induced hepatitis. Within hours, he developed hypoxia, worsening metabolic acidosis with undetectable bicarbonate level and partial respiratory compensation, coagulopathy, acute kidney injury, right lower lobe infiltrates without pulmonary embolism. Reduced ejection fraction heart failure at 15-20% with a large left ventricle apical thrombus was also found. Worsening signs of cariogenic shock despite sustaining normal blood pressure was identified on a physical exam. The patient was transferred to ICU with confirmation of cardiogenic shock with right ventricular failure with Swan-Ganz Catheter. With Concern for impending fulminant liver failure, transfer to a tertiary care center for emergent liver transplant was initiated. Decision-making The dichotomy of requirement for anti-coagulation for LV thrombus with cardiogenic shock and worsening coagulopathy due to liver failure was a challenge. Decision was made to transfuse blood products as needed with goal fibrinogen of 150 mg/dl, later changed to 100-120 mg/dl with heparin. Liver enzymes were down-trending, but it was difficult to determine if this was due to recovery or worsening of liver failure with stabilization of hemodynamics. While awaiting transfer, he developed acute cerebrovascular accident requiring emergent mechanical thrombectomy of a left MCA occlusion with suspension of heparin complicated by acute large intraventricular and intraparenchymal hemorrhage with rapid decline in neurological function. The family declined decompressive craniotomy with evacuation of parenchymal hemorrhage and the patient was transitioned to comfort care measures. Conclusion There are no clear guidelines for transfusion of plasma-based blood products in the setting of cardiogenic shock and liver disease. Expert opinion recommends maintaining fibrinogen levels above 100-200 mg/dl, however, this is in the setting of acute blood loss and is not studied in patients with liver disease. Further studies are needed.Copyright © 2023 American College of Cardiology Foundation

Multisystem Inflammatory Syndrome in Children (MIS-C) Associated With Covid-19 - Single-Center Experience

OBJECTIVES: To describe the clinical presentation, phenotype and outcome of multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (Covid-19) from a tertiary care center in southern India. METHOD(S): 257 children fulfilling the inclusion criteria of MIS-C were prospectively enrolled from June, 2020 to March, 2022. RESULT(S): Median (range) age at presentation was 6 year (35 day to 12 years). Presenting features were fever (98%), vomiting (75.8%), red eyes (63%), rashes (49%), pain abdomen (49%), shock (45.9%), lymphopenia (73%, thrombocytopenia (58.3%) and anemia (45%). 103 (39.7%) children required intensive care admission. Shock phenotype, Kawasaki-like phenotype and no specific phenotype were diagnosed in 45.9%, 44.4%, and 36.6% children, respectively. Left ventricular dysfunction (30.3%), acute kidney injury (13%), acute liver failure (17.4%), and hemophagolymphohistiocytosis (HLH) (13.6%) were the major system involvement in MIS-C. Mitral regurgitation (P=0.029), hyperechogenic coronaries (P=0.006), Left ventricular dysfunction (P=0.001) and low ejection fraction (P=0.007) were significantly associated with shock. Overall mortality was 11.7%. CONCLUSION(S): Kawasaki-like and shock-like presentation were common in MIS-C. Coronary abnormalities were seen in 118 (45.9%) children. Children with acute kidney injury, HLH, need for mechanical ventilation, and echocardiogram evidence of mitral regurgitation in MIS-C have a poor outcome.

Characteristics of patients with acute coronary syndrome in the COVID-19 pandemic: Acute coronary syndrome characteristics in patients with COVID-19

Aim: Coronavirus disease 2019 (COVID-19) has caused thrombotic disease. In this study, we aimed to determine the demographic and clinical characteristics of acute coronary syndrome (ACS) patients infected with COVID-19 and to investigate whether they differ from patients with ACS without COVID-19 in terms of these characteristics. Material(s) and Method(s): The study was designed as a single-center retrospective study. Thirty-three COVID-19 infected ACS patients (Group 1) and 100 ACS patients without COVID-19 infection (Group 2) were included in the study. Result(s): The groups were compared in terms of coronary angiographic data. Twenty-eight (84.8%) patients in Group 1 and 74 (74%) patients in Group 2 were presented as non-ST elevation myocardial infarctus. Patients were compared in terms of baseline Thrombolysis in Myocardial Infarctus (TIMI) flow, thrombus stage, myocardial blush (end), using of thrombus aspiration catheter, stent thrombosis, and TIMI flow after percutaneous coronary intervention, and it was observed that there was no statistical difference between the groups (p> 0.05). Discussion(s): COVID-19 infection can cause plaque rupture, myocardial damage, coronary spasm and cytokine storm by triggering the coagulation and inflammation process. The fact is that we did not encounter an increased thrombus load in this study.Copyright © 2022, Derman Medical Publishing. All rights reserved.

Looks Like a Vegetation but It Is Not Endocarditis

Background Myxomas are the second most common primary cardiac tumor (PCT) but overall have a low incidence rate. They usually arise from the interatrial septum whereas infective endocarditis (IE) vegetations frequently develop where there is turbulent blood flow, i.e., on the atrial side of the atrioventricular valves. Case A 75 year old male presented with fatigue, shortness of breath (SOB), myalgias and lower extremity edema for 2 weeks. His vital signs were stable and he was afebrile. Blood cultures were negative, WBC was normal, COVID-19 test was negative, and troponin was mildly elevated. TEE showed an ejection fraction of 20% with a large mitral valve (MV) mass (Figure 1A,B). Decision-making The mass was surgically resected and the MV was replaced (Figure 1C). On pathologic evaluation, the mass was confirmed to be a myxoma. The patient was later discharged without complication. Conclusion Clinical features of myxoma can overlap with IE including fever, malaise, SOB, and other signs of valvular obstruction or embolization. About 5% of myxomas originate from the MV and the differential diagnosis for an intra-atrial mass should include IE, PCT, metastatic tumors, and intracardiac thrombus. On echocardiography, myxomas appear irregularly frond-like or grape-cluster in shape. They are typically nonhomogeneous and can have areas of calcifications (Figure 1A). Both TEE and TTE are the mainstay for diagnosis of intracardiac masses and TEE specifically assists in guiding surgical excision. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

An Underestimated Cause of Reversible Cardiomyopathy. A Case of Premature Atrial Contractions Requiring Ablation in a Patient with a Structurally Normal Heart

Background Premature atrial contractions (PACs) are usually seen as a relatively benign condition. Data about PACs induced cardiomyopathy and requiring ablation are limited. Case After recovery of COVID-19 infection, an otherwise healthy 39 year old patient presented with palpitations lasting for several months. His blood lab-work was non-significant. Electrocardiogram showed frequent premature beats with both narrow and wide QRS complex. Medical treatment was ineffective. Holter monitoring showed 21% burden of premature beats over 2 days. Transthoracic echocardiogram (TTE) showed ejection fraction (EF) 45% with dilated LV dimension. MRI confirmed a structurally normal heart. Decision-making The patient was diagnosed with arrhythmia-induced cardiomyopathy, so he was referred for electrophysiological study. There was A-V activation pattern confirming atrial origin of all of the premature beats. Intermittent bundle branch block during conduction of the beats with the shortest A-V time caused wide QRS complex. With 3D CARTO mapping system, activation mapping of the right atrium and direct mapping of tricuspid annulus, we were able to reach the origin site of the PACs and apply ablation lesions there. After a month, TTE showed EF 56% with normal LV dimensions. Symptoms resolved and there was no evidence of PACs. Conclusion follow up of patients with frequent PACs is very important for early detection of induced cardiomyopathy. Catheter ablation in these cases leads to excellent results. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

Spotting the Needle Amongst the Haystack - When Asthma Isn't Asthma

Introduction/Background: Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss Syndrome, is a rare, small to medium vessel ANCA associated vasculitis. Hallmarks of EGPA include asthma, chronic rhinosinusitis, and peripheral neuropathy. EGPA is characterized by a prodrome of asthma and allergic rhinitis, followed by peripheral blood hyper-eosinophilia and accumulation of extravascular eosinophils, and finally systemic vasculitis. Extrapulmonary involvement is common, sometimes with fatal outcomes. The onset of EPGA is typically between 25-50 years;however, EGPA also occurs during childhood and has a significant morbidity and mortality. Description/Method: Our patient presented to the emergency department with a 2-week history of lethargy, wheeze and left sided neck swelling. After testing COVID-19 positive eight months prior to this, she developed wheezy episodes and was subsequently diagnosed with asthma which was managed with bronchodilators as required. She was reviewed by an allergist who confirmed a dust mite allergy and prescribed Montelukast. She remained well during the summer months however during winter she had 3 distinctive episodes of wheeze and cough which were managed by antibiotics and prednisolone. In the emergency department, an echocardiogram was performed which showed a cardiac tamponade. She was transferred to CICU where she had a pericardial drain inserted. The fluid was abundant with inflammatory cells. Multiple investigations were performed as follows: Hb: 135g/L, wbc: 20.30 x 10 9/L, Eosinophils: 12.77 x 10 9/L, CRP: 51 mg/L, ESR: 75 mm/hr, LDH: 1188 IU/L, IgE: 8000 UI/ml, ANA, ANCA: negative. CT chest showed mediastinal lymphadenopathy and patchy bilateral infiltrate and cardiac MRI showed myopericarditis and LV fibrosis. BMA showed no malignant cells and sinusitis was confirmed by CT. On examination, she was underweight. Her nasal mucosa looked inflamed. Otherwise systemic examination was unremarkable. In the context of poor ejection fraction (20%) with LV fibrosis, urgent MDT was arranged and concluded that our working diagnosis was EGPA. The decision was made to start IV methylprednisolone 10mg/kg/day for 3 days and Ivermectin. That night our patient had a VF arrest which required a single shock conversion 4J/kg. There was 7-minute downtime. Treatment was escalated to include cyclophosphamide, rituximab and plasmapheresis. The patient made a remarkable recovery, extubated and transferred to a normal ward. Her eosinophils count and inflammatory markers improved dramatically following treatment. However, she developed severe neuropathic left leg pain and NCS confirmed peripheral neuropathy Discussion/Results: EGPA is a very rare disease and diagnosis can be challenging especially with the absence of histopathology diagnosis. Early empirical treatment especially in a very ill child in intensive care unit can save lives and divert the progress of the disease. This patient has fulfilled the American College of Rheumatology criteria to diagnose EGPA including asthma, eosinophil count > 10% of upper normal, peripheral neuropathy, pulmonary infiltrates on CT thorax and paranasal sinuses abnormalities. Cardiac biopsy of the fibrotic mass may be a useful tool for diagnosis;however, this invasive procedure may expose this patient with high risk of fatal arrhythmias. Since other causes of eosinophilia were ruled out including parasitic infections, lymphoproliferative disorders, and rare primary immunodeficiency syndromes (hyper-IgE syndrome due to STAT3 or DOCK8 deficiency and Omenn syndrome) and the patient responded well to treatment, the diagnosis of EGPA was supported. Key learning points/Conclusion: Asthma not responding to bronchodilator could be another diagnosis Eosinophilia should be interpreted with caution. Defer the need for histopathology diagnosis in critically ill children Cardiac involvement is a life-threatening marker Early diagnosis prevents life threatening complications.

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).

Multisystem inflammatory syndrome: A case report

Background: Multisystem inflammatory syndrome is a rare but severe complication of Coronavirus 19 infection (COVID-19) occurring about 2-12 weeks after the initial infection. It was initially reported in children (MIS-C) but later identified in adults (MIS-A). We report a case of MIS-A in a patient presenting with myocarditis.\ Method: Case report Results: A 36 years old female admitted due to 3 days history of fever and severe epigastric pain. She had exposure to a relative with COVID-19 3 weeks prior to symptoms and antigen test done was negative. On the day of admission, she started to experience shortness of breath and easy fatigability during activities of daily living. Upon examination, she was hypotensive requiring vasopressors. 12 lead ECG showed acute anteroseptal wall myocardial infarction and 2D echocardiography revealed hypokinesia of the entire interventricular septum and inferior left ventricular free wall, and reduced ejection fraction (EF) at 43.3%. Coronary angiogram showed normal findings. On work-up, she had mild normochromic, normocytic anemia, normal serum lipase, elevated AST 222 (<34 U/L), ALT 250 (<49 U/L), Troponin T > 2000 ng/L, CPK-Total 669 (<192 U/L), Ferritin 456.90 (<204 ng/ml), ESR 13 mm/hr, CRP 24 (<6 mg/L) and procalcitonin 0.35 (<0.5 ng/ml). Infectious workup revealed negative results and PCR for COVID-19 was negative. However, further workup revealed COVID-19 Enzyme Linked Fluorescent Assay (ELFA) IgG positive at 44.75 (>1.00) and IgM negative. The patient was managed as a case of MIS-A and was started on intravenous immunoglobulin (IVIg) and short course steroids with significant improvement in symptoms. On the 10th day of hospitalization, follow-up 2D echocardiography showed improvement in previously noted ventricular wall hypokinesia and normalization of EF to 55.8%. The patient was discharged well and improved. Conclusion(s): Diagnosis of MIS-A is challenging in patients without a previously known COVID-19. A positive serology result is required to fulfill the case definition of MIS-A. Determining a history of COVID-19 and its relationship to a patient's clinical course is important for making diagnosis and determining subsequent management.