A Rare Case of Adult-onset Still's Disease Revealed by Acute Myopericarditis

Background: Still's disease in adults is a systemic inflammatory pathology of unknown aetiology, characterized by clinical manifestations associating feverish peaks, arthritis or arthtralgia, transient rashes and hyperferritinemia. Currently, this disease remains a multisystemic disease with generally poor outcome, poorly described in the literature with very few studies unlike other rheumatic diseases, probably underdiagnosed due to its clinical polymorphism. Cardiac forms are quite rare and among the manifestations described, pericarditis remains the most reported entity, myocardial involvement is exceptional. The aim of our observation is to report an exceptional case of discovery of stille disease following a myo-pericarditis mimicking a coronary Sd initially.

Characteristics of Patients Referred to a Cardiovascular Disease Clinic for Post-Acute Sequelae of SARS-CoV-2 Infection

ABSTRACTThere is limited literature on the cardiovascular manifestations of post-acute sequelae of SARS-CoV-2 infection (PASC). We aimed to describe the characteristics, diagnostic evaluations, and cardiac diagnoses in patients referred to a cardiovascular disease clinic designed for patients with PASC from May 2020 to September 2021. Of 126 patients, average age was 46 years (range 19-81 years), 43 (34%) were male. Patients presented on average five months after COVID-19 diagnosis. 30 (24%) patients were hospitalized for acute COVID-19. Severity of acute COVID-19 was mild in 37%, moderate in 41%, severe in 11%, and critical in 9%. Patients were also followed for PASC by pulmonology (53%), neurology (33%), otolaryngology (11%), and rheumatology (7%). Forty-three patients (34%) did not have significant comorbidities. The most common symptoms were dyspnea (52%), chest pain/pressure (48%), palpitations (44%), and fatigue (42%), commonly associated with exertion or exercise intolerance. The following cardiovascular diagnoses were identified: nonischemic cardiomyopathy (5%); new ischemia (3%); coronary vasospasm (2%); new atrial fibrillation (2%), new supraventricular tachycardia (2%); myocardial involvement (15%) by cardiac MRI, characterized by late gadolinium enhancement (LGE; 60%) or inflammation (48%). The remaining 97 patients (77%) exhibited common symptoms of fatigue, dyspnea on exertion, tachycardia, or chest pain, which we termed “cardiovascular PASC syndrome.” Three of these people met criteria for postural orthostatic tachycardia syndrome. Lower severity of acute COVID-19 was a significant predictor of cardiovascular PASC syndrome. In this cohort of patients referred to cardiology for PASC, 23% had a new diagnosis, but most displayed a pattern of symptoms associated with exercise intolerance.

399 CMR prevalence of subclinical myocardial damage in non-hospitalized COVID-19 patients: are we looking for a needle in a haystack?

Aims Subclinical myocardial damage is not uncommon in COVID-19 patients, likely reflecting a combination of direct viral toxicity with the activation of an uncontrolled autoimmune response usually developing during the cytokine storm phase. Whilst myocardial involvement in hospitalized patients has been extensively described in literature, no data are currently available for non-hospitalized individuals. Present study aimed to explore prevalence and impact on patients’ management of myocardial damage detected with CMR, in a cohort of consecutive non-hospitalized SARS-CoV-2 infection patients. Methods and results We conducted a single centre prospective observational study on 31 consecutive patients with previous COVID-19 who underwent CMR between October 2020 and June 2021 without requiring hospital admission. Myocarditis was defined by CMR according to the revised Lake Louise Criteria (LLC), if at least one criterion was positive: T2-based marker for myocardial oedema and T1-based marker for associated myocardial injury. Our patients’ cohort included 31 individuals with a mean age of 42.5 ± 17.4 years (20 males; 64.5%) with mean follow-up time of 365.8 ± 89 days between first positive PCR and last clinical evaluation. CMR evidence of cardiac involvement was observed in six patients (19.3%)—including two acute (of which one with pericardial inflammation), one subacute and three healed myocarditis. CMR abnormalities were associated with a higher percentage of palpitations (83% vs. 24%, P = 0.013) and chest pain (66% vs. 16%, P = 0.026) during the active phase of COVID-19. In all CMR positive cases, a tailored therapeutic approach was established consisting with the administration of cardioactive therapy with beta-blockers. All cases were uneventful during the follow-up period. Conclusions Our data showed a 19.3% prevalence of unexpected/subclinical myocardial involvement in a cohort of 31 consecutive non-hospitalized patients with previous SARS-CoV-2 infection. CMR findings were retrospectively associated with cardiac symptoms during the acute phase and yielded a change in clinical and therapeutic management in all positive cases. A better knowledge of symptomatic course of COVID-19 could help physicians to adequately select individuals in which CMR may show signs of cardiac damage.

764 Prevalence and clinical implications of cardiac involvement in individuals with paucisymptomatic SARS-CoV-2 infection

Aims Myocardial involvement has been reported in SARS-CoV-2 infection, especially in hospitalized patients during the acute phase of the disease. However, the exact prevalence and the clinical implications of cardiac involvement in young individuals with paucisymptomatic SARS-CoV-2 infection are debated. Methods and results We gathered data on 100 young patients with previous paucisymptomatic SARS-CoV-2 infection, not undergoing hospitalization and without previous diagnosis of structural heart disease, who underwent cardiological evaluation in our clinic at IRCCS ICS Maugeri (Pavia, Italy). Results were validated in an external cohort of 28 patients who underwent cardiac magnetic resonance (MRI) at Humanitas Research Hospital (Rozzano, Italy). The study population included 100 patients with previous paucisymptomatic SARS-CoV-2 infection: 60 (60%) males; median age 36 years (IQR: 22–50 years); median time after SARS-CoV-2 infection 181 days (IQR: 76–218 days). At the cardiological evaluation, 31/100 (31%) of patients referred cardiological symptoms, including dyspnoea, palpitations, chest pain or syncope. Overall, 26/100 (26%) patients showed on or more of the following instrumental alterations at first level assessment: 4/100 (4%) increase of TnI; 7/100 (7%) electrocardiographic abnormalities, 12/100 (12%) ventricular arrhythmias, and 11/100 (11%) echocardiographic abnormalities. Of 32 patients who underwent cardiac MRI, myocardial involvement was detected in 6/32 (19%) patients (Figure 1), similarly to what was observed in the validation cohort [54% males; median age 47 years (IQR: 26–55 years); myocardial involvement at MRI 4/28, 14%]. Furthermore, the proportion of patients with myocardial involvement was significantly higher in patients with first-level cardiac alterations (6/18, 28%) as compared with patients without cardiac alterations at first-level examination (0/14, 0%, P = 0.024). When analysing possible predictors for the occurrence of cardiac involvement at the MRI, documentation of ventricular arrhythmias at Holter ECG or exercise test was associated with an 87-fold higher probability of cardiac involvement at the MRI (OR: 87.3; 95% CI: 4.0–1914.3; P < 0.001). Conclusions Around 15–20% of patients with paucisymptomatic SARS-CoV-2 infection exhibit cardiac involvement documented at the cardiac MRI after a mean of 6 months from the onset of the disease. The presence of instrumental alterations detected with first level diagnostic tests, and in particular the documentation of ventricular arrhythmias at the 24 h-Holter ECG or at the exercise stress test, is a powerful predictor of myocardial involvement.

Unique presentations of the post COVID-19 infection, multisystem inflammatory syndrome in children.

Introduction: The epidemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), causing COVID-19, continuous to affect most of the world's population. In children, the respiratory and systemic involvement appears to have a much more benign course in comparison to adults, with almost no fatalities reported. However, we are encountering a post-infectious immune mediated condition, termed, multisystem inflammatory syndrome in children (MIS-C). In most cases the main features are prolonged fever and elevation of inflammatory markers, many of the patients present with abdominal pain and varying degree of myocardial involvement from mild reduction in cardiac output to the most alarming manifestation of cardiovascular shock. Results: We present two patients with unusual manifestations of MIS-C, related to post COVID-19 infection, an infant born to a mother who was severely ill at the very end of pregnancy, presenting with prolonged fever, rash, pericardial effusion, and evidence of coronary arteries wall thickening as a result of inflammation, and, a teenage girl with severe cardiac tamponade without the more common cardiac manifestations of myocardial involvement. Discussion: Post COVID-19 MIS-C can present in a wide variety of manifestations. The pathophysiologic mechanism underlying these inflammatory responses in infants are yet to be elucidated. Physicians should be aware of such presentations since rapid diagnosis and treatment are key for favorable outcome.

The Influence of Deterioration of Kidney Function on the Diagnostic Power of Laboratory Parameters Used in the Prognostic Classification of AL Amyloidosis

There is a possibility that renal dysfunction may potentially reduce the diagnostic power of the laboratory parameters Tn, NT-proBNP and sFLC levels, used in the current prognostic classification of AL amyloidosis and the diagnosis of heart involvement by amyloid. In this study, the impact of lowering the eGFR value on the usefulness of these parameters in the prognosis and diagnosis of the presence of amyloid in the myocardium was assessed in a group of 71 patients with newly diagnosed primary amyloidosis. The assessment of diagnostic power of laboratory parameters was performed on the entire study group, and in the ranges of eGFR ≥ 60 and < 60 mL/min/1.73 m2. It has been proven that, with a decrease in the eGFR value, the concentrations of NT-proBNP and the κ uninvolved light chains increase significantly (p < 0.001). To assess the diagnostic power of laboratory parameters used in the diagnosis of myocardial involvement in patients with AL amyloidosis, an ROC analysis was performed. The highest values of AUC were obtained for the NT-proBNP concentration (AUC = 0.906). The lowest values of the AUC and Youden’s index were obtained for the dFLC values (AUC = 0.723), and involved κ FLC concentration (AUC = 0.613). For all compared parameters, the smallest values of the AUC were obtained for eGFR (<60 mL/min/1.73 m2). It seems that the most suitable cardiac parameter used in the prognostic classification of AL amyloidosis, independent of renal function, is TnI. It should be noted that a concentration of involved λ chains hada higher diagnostic power to assess the heart involvement, compared to the routinely used “cardiac parameters”, TnI and NT-proBNP. It can therefore be an additional parameter used to assess the presence of amyloid in the myocardium. A decrease in eGFR value influenced the change in the diagnostic cut-off points of the most analyzed laboratory parameters. Finally, it is concluded that lowering the eGFR value reduces the utility of laboratory parameters used in the prognostic classification of AL amyloidosis.

The elevation of Creatine kinase in acute pancreatitis: a case report

Creatine Kinase (CK/CK-MB) testing is an essential lab test approaching patients with chest or epigastric pain. we report a 38-year-old man with acute pancreatitis and elevated CK/CK-MB level without myocardial involvement. Acute pancreatitis may be considered as a false positive cause of CK/CK-MB test in patients presenting with chest pain.

Global, segmental and layer specific analysis of myocardial involvement in Duchenne muscular dystrophy by cardiovascular magnetic resonance native T1 mapping

Background Progressive cardiomyopathy accounts for almost all mortality among Duchenne muscular dystrophy (DMD) patients.‍ Thus, our aim was to comprehensively characterize myocardial involvement by investigating the heterogeneity of native T1 mapping in DMD patients using global and regional (including segmental and layer-specific) analysis across a large cohort. Methods We prospectively enrolled 99 DMD patients (8.8 ± 2.5 years) and 25 matched male healthy controls (9.5 ± 2.5 years). All subjects underwent cardiovascular magnetic resonance (CMR) with cine, T1 mapping and late gadolinium enhancement (LGE) sequences. Native T1 values based on the global and regional myocardium were measured, and LGE was defined. Results LGE was present in 49 (49%) DMD patients. Global native T1 values were significantly longer in LGE-positive (LGE +) patients than in healthy controls, both in basal slices (1304 ± 55 vs. 1246 ± 27 ms, p < 0.001) and in mid-level slices (1305 ± 57 vs. 1245 ± 37 ms, p < 0.001). No significant difference in global native T1 was found between healthy controls and LGE-negative (LGE−) patients. In segmental analysis, LGE + patients had significantly increased native T1 in all analyzed segments compared to the healthy control group. Meanwhile, the comparison between LGE− patients and healthy controls showed significantly elevated values only in the basal anterolateral segment (1273 ± 62 vs. 1234 ± 40 ms, p = 0.034). Interestingly, the epicardial layer had a significantly higher native T1 in LGE− patients than in healthy controls (p < 0.05), whereas no such pattern was noticed in the global myocardium. Epicardial layer native T1 resulted in the highest diagnostic performance for distinguishing between healthy controls and DMD patients in receiver operating curve analyses (area under the curve [AUC] 0.84 for basal level and 0.85 for middle level) when compared to global native T1 and endocardial layer native T1. Conclusions Myocardial regional native T1, particularly epicardial native T1, seems to have potential as a novel robust marker of very early cardiac involvement in DMD patients. Trial registration: Chinese Clinical Trial Registry (http://www.chictr.org.cn/index.aspx) ChiCTR1800018340, 09/12/2018, Retrospectively registered.