Pericardial Inflammation Research Articles

Anakinra-Dependent Recurrent Pericarditis: The Role of the R202Q Variant of the MEFV Gene.

Background: the role of the R202Q (c.605G>A, p.Arg202Gln) missense variant of the MEFV gene has been debated as either a benign polymorphism or a potentially pathogenic mutation. We report and discuss here the case of a young female with corticosteroid-dependent recurrent pericarditis carrying the homozygous R202Q variant, exhibiting distinctive clinical features possibly influenced by this genetic variant. Methods: a 30-year-old woman with a previous diagnosis of cancer and recent respiratory infection presented with severe pleuritic chest pain, hypotension, tachycardia, and fever. Initial diagnostic evaluation indicated cardiac tamponade, and emergent pericardiocentesis was performed. Despite initial treatment with NSAIDs, colchicine, and corticosteroids, the patient experienced multiple recurrences. Genetic testing identified homozygous R202Q variant in the MEFV gene. Given the corticosteroid dependency and recurrent nature of her condition, IL-1 inhibitor anakinra was introduced, leading to significant improvement, although tapering below 150 mg per week failed to prevent recurrences. Results: the introduction of anakinra resulted in rapid symptom relief and resolution of pericardial effusion. However, attempts to taper or discontinue anakinra led to pericarditis recurrences. Ultimately, a maintenance dose of 50 mg every three days was established, which maintained remission for 18 months without recurrence. Despite multiple tapering attempts, further reduction in anakinra dosage was unsuccessful without triggering relapses. Conclusions: the R202Q variant, although typically considered benign, may contribute to an autoinflammatory phenotype resembling familial Mediterranean fever. This case underscores the potential pathogenicity of the homozygous R202Q variant in recurrent pericarditis and its responsiveness to IL-1 inhibition. In patients with corticosteroid-dependent recurrent pericarditis, genetic testing for the R202Q variant should be considered when anti-IL-1 drugs cannot be withdrawn. Further studies are warranted to elucidate the variant's role in pericardial inflammation and guide personalized treatment strategies.

CardioDPi: An explainable deep-learning model for identifying cardiotoxic chemicals targeting hERG, Cav1.2, and Nav1.5 channels

The cardiotoxic effects of various pollutants have been a growing concern in environmental and material science. These effects encompass arrhythmias, myocardial injury, cardiac insufficiency, and pericardial inflammation. Compounds such as organic solvents and air pollutants disrupt the potassium, sodium, and calcium ion channels cardiac cell membranes, leading to the dysregulation of cardiac function. However, current cardiotoxicity models have disadvantages of incomplete data, ion channels, interpretability issues, and inability of toxic structure visualization. Herein, an interpretable deep-learning model known as CardioDPi was developed, which is capable of discriminating cardiotoxicity induced by the human Ether-à-go-go-related gene (hERG) channel, sodium channel (Na_v1.5), and calcium channel (Ca_v1.5) blockade. External validation yielded promising area under the ROC curve (AUC) values of 0.89, 0.89, and 0.94 for the hERG, Na_v1.5, and Ca_v1.5 channels, respectively. The CardioDPi can be freely accessed on the web server CardioDPipredictor (http://cardiodpi.sapredictor.cn/). Furthermore, the structural characteristics of cardiotoxic compounds were analyzed and structural alerts (SAs) can be extracted using the user-friendly CardioDPi-SAdetector web service (http://cardiosa.sapredictor.cn/). CardioDPi is a valuable tool for identifying cardiotoxic chemicals that are environmental and health risks. Moreover, the SA system provides essential insights for mode-of-action studies concerning cardiotoxic compounds.

Early Post-operative ECG Changes as a Predictor of Post-pericardiotomy Syndrome Following Atrial Septal Defect Repair

To identify risk factors associated with post-pericardiotomy syndrome (PPS) in patients undergoing surgical repair of atrial septal defects (ASD). A single-center retrospective study. Tertiary academic hospital. Included were patients of all ages who underwent surgical ASD repair, while exclusion criteria included the absence of post-operative electrocardiogram (ECG), lack of follow-up post-discharge and factors hindering ECG interpretation. Demographic and clinical data, including ECG changes indicative of pericardial inflammation, were collected. The primary outcome measure was the development of PPS, determined based on the standardized European Society of Cardiology (ESC) criteria. Among 190 patients who underwent surgical ASD repair, 154 (81%) met the inclusion criteria. Of these, 25 (16%)in total developed PPS, of which 60% were ≥ 18 years of age and 56% female. Significant associations relating both early ECG changes and pre-discharge pericardial effusion with subsequent occurrence of PPS were found in both univariate and multivariate analyses. The study establishes correlations of both early post-operative ECG changes indicative of inflammation and pre-discharge pericardial effusion with subsequent occurrence of PPS in patients undergoing surgical ASD repair. Both utilizing the standardized ESC definition of PPS and incorporating a physician-validated ECG evaluation strengthened the methodologic approach in establishing these relationships. The results also highlight the importance of considering age as a potential risk factor for PPS. Further research is needed to validate these findings and explore additional risk factors predicting early identification and management of patients at high risk for PPS following surgical ASD repairs.

Novel role of cardiovascular MRI to contextualise tuberculous pericardial inflammation and oedema as predictors of constrictive pericarditis.

Tuberculosis (TB) and human immunodeficiency virus/acquired immunodeficiency syndrome have reached epidemic proportions, particularly affecting vulnerable populations in low- and middle-income countries of sub-Saharan Africa. TB pericarditis is the commonest cardiac manifestation of TB and is the leading cause of constrictive pericarditis, a reversible (by surgical pericardiectomy) cause of diastolic heart failure in endemic areas. Unpacking the complex mechanisms underpinning constrictive haemodynamics in TB pericarditis has proven challenging, leaving various basic and clinical research questions unanswered. Subsequently, risk stratification strategies for constrictive outcomes have remained unsatisfactory. Unique pericardial tissue characteristics, as identified on cardiovascular magnetic resonance imaging, enable us to stage and quantify pericardial inflammation and may assist in identifying patients at higher risk of tissue remodelling and pericardial constriction, as well as predict the degree of disease reversibility, tailor medical therapy, and determine the ideal timing for surgical pericardiectomy.

INFLA-score: A new diagnostic paradigm to identify pericarditis

BackgroundDiagnosis of pericarditis may be challenging because not all patients meet the conventional criteria. An overlooked diagnosis implies a longer course of symptoms and an increased risk of recurrences. C-reactive protein (CRP), widely used as an inflammation marker, has some limitations. This study aimed to assess the usefulness and prognostic value of INFLA-score, a validated index assessing low-grade inflammation, in the definite diagnosis of pericarditis. MethodsPatients with suspected pericarditis were included. The INFLA-score was computed based on white blood cells and platelet count, neutrophil-to-lymphocyte ratio, and CRP, ranging from −16 to +16. An INFLA-score > 0 was considered positive for the presence of pericardial inflammation. The primary end point was the association of INFLA-score with diagnosis of pericarditis according to conventional criteria. The recurrence of pericarditis at 6 months was the secondary end point. ResultsA total of 202 patients were included, aged 47 ± 17 years, and 57% were females. Among 72 (36%) patients with a diagnosis of pericarditis, an INFLA-score > 0 was observed in 86% (vs. 36%, p < 0.001), abnormal CRP in 42% (vs. 10%, p < 0.001), pericardial effusion in 44% (vs. 19%, p < 0.001), abnormal electrocardiogram in 56% (vs. 24%, p < 0.001), and rubs in 5% (vs. 0.1%, p = 0.072). INFLA-score > 0 had the strongest predictive value for the diagnosis of pericarditis (hazard ratio 8.48, 95% confidence interval [CI] 3.39–21.21), with 86% sensitivity and 64% specificity, as opposed to CRP (hazard ratio 1.72, non-significant 95% CI 3.39–21.20). Recurrent pericarditis at 6 months was more frequent in patients with a positive INFLA-score (37% vs. 8%, p < 0.001, rate ratio 4.15, 95% CI 2.81–6.12). In patients with normal CRP, INFLA-score–confirmed ongoing inflammation in 78% of the cases. Compared with the conventional criteria, the INFLA-score had the highest accuracy (area under the curve = 0.82). Different cutoffs were valuable to rule out (INFLA-score > 0, sensitivity 86%, and negative LR 0.22) or rule in (INFLA-score ≥ 10, specificity 97%, and positive LR 13) the diagnosis. ConclusionsThe INFLA-score is a useful diagnostic tool to assess the probability of pericarditis, with a strong prognostic value for further recurrences, outperforming CRP.

Novel Therapeutic Insights Into the Treatment of Pericarditis: Targeting the Innate Immune System.

Acute pericarditis is characterized by pericardial inflammation that can be treated with anti-inflammatory drugs. A considerable percentage of patients develops recurrent pericarditis with several relapses. In developed countries, the idiopathic form is the most frequent and has a high risk of recurrences. Two pathophysiological mechanisms have been described for idiopathic recurrent pericarditis: autoimmune and autoinflammatory. The autoimmune mechanism is more frequently encountered in patients with rheumatologic disorders, especially systemic lupus erythematosus. The innate immune system plays a central role in the pathophysiology of pericarditis, especially in the autoinflammatory phenotype. Current evidence highlights the central role played by interleukin 1 and NLRP3 (NACHT, leucine-rich repeat, and pyrin domain-containing protein 3) in idiopathic recurrent pericarditis. Accordingly, interleukin 1 blockers have been approved for the treatment of this condition. Neutrophils are likely to be important in such setting; however, their role has only been partially investigated. In the present review, we have collected the current knowledge on the role of innate immune system in pericarditis pathophysiology and how this can be used to provide targeted treatments for patients with recurrent pericarditis.

Anakinra in idiopathic recurrent pericarditis: acomprehensive case series and literature review.

Idiopathic recurrent pericarditis (IRP) is defined by recurring episodes of pericardial inflammation without aknown cause. This study investigates the safety and efficacy of anakinra, an interleukin‑1 inhibitor, as asuccessful therapy for IRP in cases resistant to conventional treatment. Aretrospective evaluation of patients treated at our autoinflammatory center between 2011 and 2023 was conducted. Patient files were examined for demographic, clinical, and treatment response data, including nonsteroid anti-inflammatory drugs (NSAIDs), corticosteroids, and colchicine. Monogenic autoinflammatory disease screening was performed for Mediterranean Fever (MEFV), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase (MVK), nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3), and nucleotide-binding oligomerization domain-containing protein 2 (NOD2). Patients who experienced multiple episodes of pericarditis were diagnosed with recurrent pericarditis. The study evaluated anakinra treatment in IRP patients unresponsive to conventional therapy. The study included 21participants, 9(42.9%) female and 12(57.1%) male. The average age of the participants was 43.1 ± 16.5years. The MEFV mutation analysis revealed that 2(9.5%) had amutation in exon10 and4 (19.0%) had one in exon2. Out of the 16cases, 15successfully discontinued steroid treatment. Four patients (19.0%) experienced injection site reactions. C‑reactive protein (CRP) levels were measured at an average of 196 ± 67.8 mg/l before and 2.6 ± 3.15 mg/l after anakinra treatment. In conclusion, the study adds to the growing evidence for the efficacy of interleukin-1inhibitors, such as anakinra, as apromising treatment modality for IRP in cases resistant to conventional treatment.

Post-cardiac injury syndrome and pericardial effusion recurrence after pericardial effusion drainage in chronic idiopathic pericardial effusion

IntroductionThe management of even large pericardial effusions in asymptomatic patients is still a matter of debate. Aim of the present study is to explore, in a multicenter setting, the rate of post-cardiac injury syndromes (PCIS) and pericardial effusion recurrence after pericardial effusion drainage procedure. Material and MethodsThis is a multicenter international retrospective study including a consecutive cohort of patients diagnosed with large, chronic and idiopathic pericardial effusions, prospectively evaluated from January 2003 to December 2021 who underwent a clinically indicated pericardial drainage procedure. Two separate end-points were recorded: 1) recurrence of pericardial effusion after drainage without any sign of pericardial inflammation 2) occurrence of PCIS, defined as the new onset of pericarditis 1 to 6 weeks after pericardial intervention. Results124 patients were enrolled (50 % female, mean age 64 years old). A mean follow-up of 29.6 ± 25.6 months was obtained in 110 patients (88 %). 110 patients were treated with pericardiocentesis (89 %), 25 with pleuro-pericardial windows (20 %), and 1 with pericardiectomy (1 %). PCIS occurred in 21 out of 124 patients followed for at least 6 weeks (16.9%). Recurrence of pericardial effusion after drainage without any sign of pericardial inflammation occurred in 68 out of 110 patients at a longer follow-up (61.8 %). At multivariate analysis only inflammatory cells in pericardial fluid was associated with PCIS and pericardiocentesis with pericardial effusion recurrency. ConclusionOur data support the need of caution with the use of pericardiocentesis in asymptomatic patients with large pericardial effusion as it is often associated with pericardial effusion recurrence. Of interest the presence of inflammatory cells in the pericardial fluid is associated with PCIS after pericardial drainage procedures.

Delayed Diagnosis of Constrictive Pericarditis Resulting in Recurrent Heart Failure: A Case Report

Constrictive pericarditis can lead to compromised diastolic ventricular filling due to pericardial inflammation and fibrosis. A diagnosis of constrictive pericarditis was established by identifying structural and hemodynamic features through echocardiography. We present a case of constrictive pericarditis, which manifested in the form of gradually worsening dyspnea and lower-extremity edema over a 7 years period. The patient was diagnosed with constrictive pericarditis using echocardiography, and underwent a pericardiectomy.

Abstract 16555: Neutrophil to Lymphocyte Ratio: A Marker of Inflammation in Pericardial Disease

Introduction: Neutrophil-to-lymphocyte ratio (NLR) reflects the intricate relationship between the innate and adaptive immune systems during inflammatory states. With NLR known to be a sensitive indicator of inflammation, its role as a marker in acute pericarditis is yet to be investigated. Here we describe a rare case of acute pericarditis with an elevated NLR highlighting the potential impact this marker can have within the diagnostic evaluation of pericardial disease. Clinical Case: 46-year-old male with a past medical history of long QT syndrome, crohn's disease, ankylosing spondylitis, psoriatic arthritis, and histoplasmosis who presented with a four-day history of pleuritic chest pain, fever, and chills. The patient had a recent admission two months prior for pericardial effusion s/p emergent pericardial window. Cultures then showed acute and chronic inflammatory granulation tissue and necrosis with no evidence of malignancy and negative stains for fungal organisms. He was discharged home with ibuprofen without improvement. Upon arrival, the patient was hemodynamically stable with a workup notable for leukocytosis of 13.82x 10 9 /l, elevated CRP of 22.5 mg/dL and ESR of 65 mm/hr. NLR was 7.0. Echocardiography showed preserved ejection fraction with moderate circumferential pericardial effusion (figure A). The patient was started on colchicine in addition to ibuprofen. The cardiac MRI obtained showed evidence of moderate active pericarditis (figure B, C). A decision was ultimately made to start inpatient anakinra with a plan to discharge on triple therapy with colchicine, ibuprofen, and rilonacept. Discussion: NLR has the potential to be a novel inflammatory marker in pericardial disease associated with pericardial inflammation and edema. A normal range of NLR is between 1-2, with values higher than 3.0 and below 0.7 considered pathological. Our case highlights the importance of NLR as a diagnostic and prognostic marker of pericardial disease.

A rare case of obstructive shock due to cardiac tamponade in a term pregnancy

Background Obstructive shock due to cardiac tamponade is a rare, life-threatening occurrence in the peripartum period. Etiologies include preeclampsia, infection, autoimmune conditions, and malignancy. Early recognition of the underlying disease process allows for multidisciplinary treatment and a favorable outcome. Case A 33 year-old presented for cardiac tamponade identified in the peripartum period. She was diagnosed with preeclampsia with severe features immediately prior to her repeat Cesarean delivery and received magnesium prophylaxis. Postoperatively, she developed hypotension, tachycardia, and shortness of breath and was found to have a pericardial effusion with tamponade physiology. She underwent pericardial drain placement which was initially successful. However, she had recurrent symptomatic tamponade and thus a pericardial window was performed resulting in improvement of her symptoms. Workup revealed pericardial inflammation possibly secondary to a viral source, and she was successfully treated with anti-inflammatory therapy. Conclusion We hypothesize that this patient’s cardiac tamponade was caused by inflammatory pericarditis exacerbated by severe preeclampsia. Preeclampsia is a disease characterized by cardiovascular remodeling and fluid shifts in other compartments and thus is theorized to have contributed to this patient’s effusion. Cardiac tamponade should be considered in the differential for any parturient presenting with hypotension and shortness of breath.

FRI271 A Case Of Autoimmune Polyglandular Syndrome Type 2 Presenting With Cardiac Symptoms

Abstract Disclosure: J. Lin: None. C. Lee: None. A.R. Crawford: None. C. Taub: None. Autoimmune polyglandular syndrome type 2 (APS 2) is characterized by the combination of autoimmune adrenal insufficiency with autoimmune primary hypothyroidism, and/or type 1 diabetes mellitus, as well as other autoimmune conditions. The estimated prevalence is reported to be 1.4-4.5 cases per 100,000 persons. Presenting symptoms are often nonspecific, leading to delayed diagnosis; however, manifestations can sometimes become rapidly life-threatening. Cardiac symptoms, rare but clinically significant, can be the first presentation for APS 2. 34 year old male with history of asthma presents with nausea and chest pain, found to have pericarditis that was complicated by pericardial tamponade and cardiogenic shock requiring emergent pericardiocentesis and mechanical support with veno-arterial extracorporeal membrane oxygenation. Stress dose steroids were given due to concern for fulminant myocarditis. His condition improved with glucocorticoid treatment, but the etiology of his acute decompensation was unclear. He completed the prednisone taper before discharge and was started on amiodarone for atrial flutter. He presented with recurrent symptoms a week later. Cardiac MRI showed pericarditis without myocarditis. PET scan showed pericardial inflammation without evidence of malignancy or vasculitis. Broad infectious workup including viral studies was negative. An extensive biochemical workup showed low serum aldosterone and high renin activity while on prednisone. Autoimmune workup was positive for 21-hydroxylase antibody, thyroid peroxidase antibody, and homozygous for celiac HLA DQ8, suggestive of APS 2. Patient was started on levothyroxine for subclinical hypothyroidism with TSH &amp;gt;20. Interleukin blockade with anakinra was initiated, and patient was discharged with 25mg of daily prednisone pending insurance authorization for anakinra. A month later, patient re-presented with pericarditis. Patient received stress dose steroids, and was started on fludrocortisone. Levothyroxine was stopped because of amiodarone induced thyrotoxicosis. Patient was discharged on a prednisone taper regimen, and anakinra was approved a month later. Few case reports have described the association between APS 2 with pericardial tamponade. In healthy patients presenting with unexplained pericarditis, autoimmunity should be considered as a differential diagnosis as it can rapidly become life-threatening requiring urgent intervention. It is important to recognize for these patients, they are at risk for recurrent pericarditis and cardiac tamponade. Presentation: Friday, June 16, 2023

Protocolo diagnóstico y terapéutico de la pericarditis aguda y el taponamiento cardíaco

Both acute pericarditis and cardiac tamponade are encompassed within acute pericardial syndromes. Acute pericarditis is an uncommon disease in the emergency department, representing just 5% of patients who consult due to chest pain, of which only 0.1% require hospitalization. Both acute pericarditis and cardiac tamponade as well as constrictive pericarditis can be considered a single inflammatory process and therefore can be classified into three groups according to the clinical manifestations.In the first group are symptoms arising from pericardial inflammation, in which pain and fever are predominant. In the second group are symptoms arising from pericardial effusion due to pericardial inflammation that has been ongoing for some time and the production of serous fluid, whose most extreme manifestation is cardiac tamponade. In the third group are symptoms arising from chronic inflammation in the form of pericardial thickening, retraction, and calcification.

A Contemporary Approach to the Diagnosis and Management of Constrictive Pericarditis.

CMR provides valuable anatomical information, including pericardial thickness and the presence of pericardial effusion, enables a comprehensive cardiac function assessment, hemodynamics, myocardial characteristics, and above all, assessment for pericardial inflammation.(2, 4) Ventricular interdependence is demonstrated by abnormal diastolic septal motion observed on steady-state free precession images (81% sensitive and 100% specific for constriction)(supplemental-video-1)(3, 4), while pericardial-myocardial tethering can be demonstrated using myocardial tagging sequences.

Monogenic autoinflammatory disease-associated cardiac damage.

Autoinflammatory diseases (AIDs) constitute several disorders that are characterized by the presence of recurrent episodes of unprovoked inflammation due to dysregulated innate immune system in the absence of autoantibodies or infections. Most of them have a strong genetic background, with mutations in single genes involved in inflammation referred to monogenic AIDs. In this article, we will review the cardiac manifestations in various monogenic AIDs. Various cardiac manifestations can be seen in various monogenic AIDs, including pericarditis, valvular diseases, coronary diseases, cardiomyopathies, and pulmonary hypertension, especially in Familial Mediterranean fever (FMF). Monogenic AIDs can manifest a variety of cardiac lesions, the most common of which is pericardial effusion, which may be local pericardial inflammation secondary to systemic inflammatory responses. While, the pathogenesis and incidence are still unclear. More research is still needed to explore the relationship between monogenic AIDs and cardiac damage for better understanding these diseases.

The contemporary role of cardiac computed tomography and cardiac magnetic resonance imaging in the diagnosis and management of pericardial diseases.

Pericardial syndromes encompass different clinical conditions from acute pericarditis to idiopathic chronic pericardial effusion. Transthoracic echocardiogram is the first and most important initial diagnostic imaging modality in most patients affected by pericardial disease. However, cardiac computed tomography and cardiac magnetic resonance (CMR) have recently gained a pivotal role in cardiology and recent reports supported the role of both these advanced techniques in the evaluation and guiding therapy of pericardial disease. The most promising tool is the capability of CMR to identify the presence of pericardial inflammation carrying both diagnostic and prognostic value in the setting of recurrent and chronic pericarditis. On the other side cardiac CT permits accurate evaluation of the presence and extension of pericardial calcification providing important information in confirming the diagnosis of constrictive pericarditis and during the preprocedural planning for patients undergoing pericardiectomy. Both cardiac CT and CMR correct indication and proper evaluation need specific expertise, especially for the evaluation of pericardial disease, thus the aim of the present review is to provide physicians an updated overview on the CCT and CMR role in pericardial disease focusing on technical issues, recent research findings and potential clinical applications.

Cardiac magnetic resonance imaging of pericardial diseases: a comprehensive guide.

Cardiac magnetic resonance (CMR) imaging has been established as a valuable diagnostic tool in the assessment of pericardial diseases by providing information on cardiac anatomy and function, surrounding extra-cardiac structures, pericardial thickening and effusion, characterization of pericardial effusion, and the presence of active pericardial inflammation from the same scan. In addition, CMR imaging has excellent diagnostic accuracy for the non-invasive detection of constrictive physiology evading the need for invasive catheterization in most instances. Growing evidence in the field suggests that pericardial enhancement on CMR is not only diagnostic of pericarditis but also has prognostic value for pericarditis recurrence, although such evidence is derived from small patient cohorts. CMR findings could also be used to guide treatment de-escalation or up-titration in recurrent pericarditis and selecting patients most likely to benefit from novel treatments such as anakinra and rilonacept. This article is an overview of the CMR applications in pericardial syndromes as a primer for reporting physicians. We sought to provide a summary of the clinical protocols used and an interpretation of the major CMR findings in the setting of pericardial diseases. We also discuss points that are less well clear and delineate the strengths and weak points of CMR in pericardial diseases.

The incidence and clinical characteristics of myocarditis and pericarditis following mRNA-based COVID-19 vaccination in Republic of Korea adolescents from July 2021 to September 2022.

Age-specific information regarding myocarditis/pericarditis in adolescents following mRNA-based coronavirus disease 2019 (COVID-19) vaccination in Asia remains insufficient. This study investigated the incidence and clinical characteristics of myocarditis/pericarditis in Republic of Korea adolescents after mRNA-based COVID-19 vaccination. This retrospective descriptive study utilized patient data from the Korea Immunization Management System. Incidence rates were calculated according to age and sex. Clinical characteristics (symptoms/signs, laboratory values, and imaging results) were compared between mild and severe cases. Between July 19, 2021 and September 30, 2022, 3,728,224 individuals aged 12 to 19 years received 6,484,165 mRNA-based COVID-19 vaccines, and 173 cases met the case definition for myocarditis/pericarditis: 151 mild (87.3%) and 22 severe (12.7%). The incidence was 3.8-fold higher in males than in females. Troponin I/ troponin T was elevated in 96% of myocarditis cases, demonstrating higher sensitivity than creatine kinase-myocardial band (67.6%) or C-reactive protein (75.2%). ST-segment or Twave on electrography abnormalities were found in 60.3% (85/141). Paroxysmal/sustained atrial/ventricular arrhythmias were more common in severe than in mild cases (45.5% vs. 16.8%, p=0.008). Edema on T2-weighted magnetic imaging occurred in 21.6% (8/37) and 62.5% (5/8) of mild and severe cases, respectively (p=0.03). Abnormal pericardial fluid collection or pericardial inflammation was found in 75.4% of pericarditis cases (49/65). Myocarditis/pericarditis occurred in rare cases following mRNA-based COVID-19 vaccination. Most cases were mild, but the incidence was higher in adolescent males and after the second dose. As bivalent severe acute respiratory syndrome coronavirus 2 mRNA vaccination started in Republic of Korea in October 2022, the post-vaccination incidence of myocarditis/pericarditis should be closely monitored, considering clinical characteristics.

Management of acute pericarditis.

Provide an update on current management and most recent evidence in the treatment of pediatric pericarditis. While treatment of acute pericarditis has not significantly changed over the last decade, management of recurrent acute pericarditis, with increased attention to autoinflammation as a causal mechanism, has evolved substantially. This includes clinical trial evidence that newer medications targeting interleukin-1 receptors are effective in recurrent forms of pericarditis. In addition, advanced imaging utilizing cardiac magnetic resonance has emerged as a particularly effective way to detect ongoing pericardial inflammation in support of more difficult-to-treat patients. Recent advances in acute and recurrent pericarditis management have allowed for a more tailored approach to the individual patient. Yet, unresolved questions require further research.

Novel Pathophysiological, Diagnostic and Therapeutic Concepts in Acute and Recurrent Pericarditis.

Acute pericarditis is the most frequent pericardial disease characterized by inflammation of the pericardial layers resulting in pain, dyspnea and fatigue. Often limited to an isolated event, up to 30% of patients experience one or more recurrences. There is limited knowledge about the pathophysiology of this disease, possibly due to the limited availability of animal models. More recently, following seminal clinical trials with colchicine and interleukin-1 (IL-1) blockers and a novel murine model of acute pericarditis using zymosan A, it has become clear that the NLRP3 (NACHT, leucine-rich repeat, and pyrin domain-containing protein 3) inflammasome/IL-1 axis plays a central role in driving acute pericardial inflammation and in sustaining this process during recurrences. Diagnostic management of pericarditis has been implemented with multimodality imaging including echocardiography, cardiac computed tomography, and cardiac magnetic resonance. These imaging modalities provide essential diagnostic and pathogenetic information, and are able to characterize pericardial inflammation, allowing to refine risk stratification and personalize treatment. Recent acquisitions yield relevant implications with regard to the therapeutic management of acute and recurrent pericarditis. Non-steroidal anti-inflammatory drugs (NSAIDs) and colchicine are cornerstone therapies either for acute and recurrent pericarditis. However, the benefits of targeted agents, such as anakinra - a recombinant human IL-1 receptor antagonist - and rilonacept - an IL-1 /IL-1 trap, are being increasingly recognized. To this end, phenotyping patients with pericarditis and addressing such therapies to those presenting with auto-inflammatory features (elevated C-reactive protein, sustained pericardial and systemic inflammation, multiple recurrences) is of utmost importance to identify patients who might be more likely to benefit from NLRP3 inflammasome/IL-1 pathway blockade.