Abstract 17059: Ocrelizumab Induced Kounis Syndrome

Kounis syndrome(KS), first described in 1991, is defined as concurrence of acute coronary syndrome and anaphylactic events. Primary mechanism of KS is interaction of mast cells with T-lymphocytes and macrophages via multidirectional stimuli leading to platelets activation. Case presentation: A 35 y.o. tennis coach with multiple sclerosis is admitted to the medical ICU with anaphylaxis after receiving Ocrelizumab infusion. Vital signs on presentation are significant for hypotension with blood pressure of 69/30 mm Hg, sinus tachycardia to 110 bpm and hypoxia with SatO2 88% on room air. Other investigations including chest x-ray, EKG and blood work are unrevealing for secondary pathological process outside of anaphylaxis. She undergoes fluid resuscitation followed by epinephrine drip for persistent hypotension. In addition methylprednisolone, famotidine and diphenhydramine are administered. She requires escalating doses of epinephrine and subsequently develops chest pain with troponin elevation to 0.29 ng/ml and EKG concerning for new ST depression and T wave inversion in II, III, aVF, V2 - V6 leads. Urgent echocardiography revealed normal biventricular function with no wall motion abnormalities and is only significant for moderate MR. Given excellent underlying functional capacity and no underlying cardiac risk factors, she was treated for Kounis syndrome by treating underlying anaphylaxis and weaning epinephrine as able with additional fluid resuscitation. Her chest pain resolved and EKG normalized with eventual discontinuation of epinephrine. Repeat echocardiography revealed preserved left ventricular (LV) function and mild MR. Discussion: KS is not a rare disease but easily overlooked and infrequently diagnosed. Our patient had the type I variant: endothelial dysfunction or microvascular angina in absence of cardiac risk factors. Inflammatory mediators can cause vasospasm and catecholamines used for treatment may potentiate it therefore requiring thoughtful dosing and appropriate duration of treatment. Prompt recognition is crucial for appropriate management of anaphylatic shock followed by that of ACS if LV function declines or risk factors for cardiac disease are present.

Association of Global Coagulation Profiles With Cardiovascular Risk Factors and Atherosclerosis: A Sex Disaggregated Analysis From the BioHEART‐CT Study

Background Although the association between dysregulated coagulation and atherosclerosis is well recognized, individual assays have been of minimal value in understanding disease susceptibility. Here we investigated the association of global coagulation profiles with coronary artery disease with consideration of sex differences. Methods and Results The study included patients from the BioHEART‐CT (The BioHEART Study: Assessing Patients With Suspected Cardiovascular Disease for New Disease Markers and Risk Factors) biobank who had computed tomography coronary angiograms scored for coronary artery calcium score (CACS) and Gensini score. The cohort included 206 adult patients who were referred for clinically indicated computed tomography coronary angiography and had a median of 2 major cardiac risk factors; 50% were women and the average age was 62.6 years (±9.9 years). The overall hemostatic potential (OHP) and calibrated automated thrombography generation assays were performed on platelet‐poor plasma. CACS and Gensini score in men were significantly correlated in bivariate analysis with measures from the OHP assay, and regression models predicting disease severity by CACS or Gensini score were improved by adding the OHP assay variables in men but not in women. The calibrated automated thrombography generation assay demonstrated a more hypercoagulable profile in women than in men. The OHP assay showed hypercoagulable profiles in women with hyperlipidemia and men with obesity. Conclusions The OHP assay identified hypercoagulable profiles associated with different risk factors for each sex and was associated with CACS and Gensini score severity in men, emphasizing the associations between increased fibrin generation and reduced fibrinolysis with cardiac risk factors and early atherosclerosis. Registration Information www.anzctr.org.au . Identifier: ACTRN12618001322224.

Combined cardiac risk factors predict COVID-19 related mortality and the need for mechanical ventilation in Coptic Clergy

Background and aims The clinical adverse events of COVID-19 among clergy worldwide have been found higher than among ordinary communities, probably because of the nature of their work. The aim of this study, was to assess the impact of cardiac risk factors on COVID-19-related mortality and the need for mechanical ventilation in Coptic clergy. Methods Of 1576 Coptic clergy participating in the COVID-19-Clergy study, serving in Egypt, USA and Europe, 213 had the infection and were included in this analysis. Based on the presence of systemic arterial hypertension (AH), participants were divided into two groups: Group-I, Clergy with AH (n=77) and Group-II, without AH (n=136).Participants' demographic indices, cardiovascular risk factors, COVID-19 management details and related mortality were assessed. Results Clergy with AH were older (p<0.001), more obese (p=0.04), had frequent type 2 diabetes (DM) (p=0.001), dyslipidemia (p=0.001) and coronary heart disease (CHD) (p=0.04) compared to those without AH. COVID-19 treatment at home, hospital or in intensive care did not differ between the patient groups (p>0.05 for all). Clergy serving in Northern and Southern Egypt had a higher mortality rate compared to those from Europe and the USA combined (5.22%, 6.38%, 0%; p=0.001). The impact of AH on mortality was significant only in Southern Egypt (10% vs. 3.7%; p=0.01) but not in Northern Egypt (4.88% vs. 5.81%; p=0.43). In multivariate analysis, CHD OR 1.607 [(0.982 to 3.051); p=0.02] and obesity, OR 3.403 [(1.902 to 4.694); p=0.04]predicted COVID-19 related mortality. A model combining cardiac risk factors (systolic blood pressure (SBP) ≥160 mmHg, DM, obesity, dyslipidemia and history of CHD), was the most powerful independent predictor of COVID-19-related mortality, OR 4.813 [(2.011 to 7.017); p=0.008]. The same model also proved the best independent multivariate predictor of mechanical ventilation OR 1.444 [(0.949 to 11.88); p=0.001]. Conclusion In Coptic clergy, the cumulative impact of risk factors is the most powerful predictor of mortality and the need for mechanical ventilation in Coptic clergy. FUNDunding Acknowledgement Type of funding sources: None.

Characteristics and outcomes of acute COVID-19 infection in paediatric and young adult patients with underlying cardiac disease

Objective To describe outcomes of acute coronavirus disease 2019 in paediatric and young adult patients with underlying cardiac disease and evaluate the association between cardiac risk factors and hospitalisation. Study design We conducted a retrospective single-institution review of patients with known cardiac disease and positive severe acute respiratory syndrome coronavirus 2 RT-PCR from 1 March, 2020 to 30 November, 2020. Extracardiac comorbidities and cardiac risk factors were compared between those admitted for coronavirus disease 2019 illness and the rest of the cohort using univariate analysis. Results Forty-two patients with a mean age of 7.7 ± 6.7 years were identified. Six were 18 years of age or more with the oldest being 22 years of age. Seventy-six percent were Hispanic. The most common cardiac diagnoses were repaired cyanotic (n = 7, 16.6%) and palliated single ventricle (n = 7, 16.6%) congenital heart disease. Fourteen patients (33.3%) had underlying syndromes or chromosomal anomalies, nine (21%) had chronic pulmonary disease and eight (19%) were immunosuppressed. Nineteen patients (47.6%) reported no symptoms. Sixteen (38.1%) reported only mild symptoms. Six patients (14.3%) were admitted to the hospital for acute coronavirus disease 2019 illness. Noncardiac comorbidities were associated with an increased risk of hospitalisation (p = 0.02), particularly chronic pulmonary disease (p = 0.01) and baseline supplemental oxygen requirement (p = 0.007). None of the single ventricle patients who tested positive required admission. Conclusions Hospitalisations for coronavirus disease 2019 were rare among children and young adults with underlying cardiac disease. Extracardiac comorbidities like pulmonary disease were associated with increased risk of hospitalisation while cardiac risk factors were not.

Cardiovascular disease in systemic lupus erythematosus

There is a well-known increased risk for cardiovascular disease that contributes to morbidity and mortality in systemic lupus erythematosus (SLE). Major adverse cardiovascular events and subclinical atherosclerosis are both increased in this patient population. While traditional cardiac risk factors do contribute to the increased risk that is seen, lupus disease-related factors, medications, and genetic factors also impact the overall risk. SLE-specific inflammation, including oxidized lipids, cytokines, and altered immune cell subtypes all are likely to play a role in the pathogenesis of atherosclerotic plaques. Research is ongoing to identify biomarkers that can help clinicians to predict which SLE patients are at the greatest risk for cardiovascular disease (CVD). While SLE-specific treatment regimens for the prevention of cardiovascular events have not been identified, current strategies include minimization of traditional cardiac risk factors and lowering of overall lupus disease activity.

Cognition and disease characteristics in adult onset versus late onset multiple sclerosis

Background: Cognitive impairment is common sequelae of multiple sclerosis (MS); however, relatively little is known about cognitive impairment in late-onset multiple sclerosis (LOMS). Objective: To investigate differences in disease characteristics and cognition in LOMS and adult-onset multiple sclerosis (AOMS) patients. Methods: Archival medical records and neuropsychological evaluations from an MS specialty center were reviewed. Differences in disease characteristics between 53 LOMS and 124 AOMS were compared using chi-square or analysis of variance (ANOVA). To investigate differences in cognitive functioning, age-adjusted standardized scores were compared via analysis of covariance (ANCOVA), using cardiac risk factors and disease duration as covariates. Results: Compared to AOMS, LOMS patients had significantly more cardiac risk factors, shorter disease duration, and shorter time to diagnosis. LOMS patients had similar Expanded Disability Status Scale scores as AOMS patients. LOMS patients demonstrated significantly more impairment on tasks of visual learning and memory, and working memory than AOMS patients. Conclusion: Despite a shorter disease duration, LOMS and AOMS patients had similar levels of physical impairment. However, even after accounting for differences in disease duration and cardiac risk, LOMS patients showed a greater burden of cognitive impairment than AOMS patients, suggesting MS diagnosed later in life may progress faster due to the interaction between MS neuropathology and aging.