Coronary artery aneurysms (CAAs) are rare cardiovascular abnormalities often associated with conditions like atherosclerosis, connective tissue disorders, or vasculitis. We report a case of a 39-year-old woman presenting with palpitations and dysphagia, ultimately diagnosed with a giant aneurysmal left circumflex coronary artery using a multimodal imaging approach. Initial echocardiography identified a cystic lesion within the left atrium, prompting further evaluation with cardiac magnetic resonance (CMR), computed tomography (CT), and coronary catheterization. These modalities confirmed a large LCX aneurysm measuring 6.3 × 7.9 cm with left main coronary artery dilation. While surgical intervention was recommended, the patient opted for medical management. This case underscores the critical role of advanced imaging in diagnosing and managing complex cardiac conditions.

Kawasaki disease (KD) is a common pediatric vasculitis, with coronary artery lesions (CALs) representing its most severe complication. Early identification of high-risk patients, including those with disease resistant to first-line treatments, is essential to guide personalized therapeutic approaches. Given the limited reliability of current scoring systems, there has been growing interest in the development of new prognostic models based on machine learning algorithms and artificial intelligence (AI). AI has the potential to revolutionize the management of KD by improving patient stratification and supporting more targeted treatment strategies. This narrative review examines recent applications of AI in stratifying patients with KD, with a particular focus on the ability of models to predict intravenous immunoglobulin resistance and the risk of CALs. We analyzed studies published between January 2019 and April 2024 that incorporated AI-based predictive models. In total, 21 papers met the inclusion criteria and were subject to technical and statistical review; 90% of these were conducted in patients from Asian hospitals. Most of the studies (18/21; 85.7%) were retrospective, and two-thirds included fewer than 1000 patients. Significant heterogeneity in study design and parameter selection was observed across the studies. Resistance to intravenous immunoglobulin emerged as a key factor in AI-based models for predicting CALs. Only five models demonstrated a sensitivity > 80%, and four studies provided access to the underlying algorithms and datasets. Challenges such as small sample sizes, class imbalance, and the need for multicenter validation currently limit the clinical applicability of machine-learning-based predictive models. The effectiveness of AI models is heavily influenced by the quantity and quality of data, labeling accuracy, and the completeness of the training datasets. Additionally, issues such as noise and missing data can negatively affect model performance and generalizability. These limitations highlight the need for rigorous validation and open access to model code to ensure transparency and reproducibility. Collaboration and data sharing will be essential for refining AI algorithms, improving patient stratification, and optimizing treatment strategies.

To describe a single-center experience of "complete aortic repair" consisting of surgical or endovascular total arch replacement/repair (TAR) followed by thoracoabdominal fenestrated-branched endovascular aortic repair (FB-EVAR).

We reviewed 480 consecutive patients who underwent FB-EVAR with physician-modified endografts (PMEGs) or manufactured stent-grafts between 2013 and 2022. From those, we selected only patients treated with open or endovascular arch repair and distal FB-EVAR for aneurysms involving the ascending, arch and thoracoabdominal aortic segments (zones 0-9). Manufactured devices were used under an investigational device exemption protocol. Endpoints included early/in-hospital mortality, mid-term survival, freedom from secondary intervention, and target artery instability.

There were 22 patients, 14 men and 8 women with a median age of 72±7 years. Thirteen postdissection and 9 degenerative aortic aneurysms were repaired with a mean maximum diameter of 67±11 mm. Time from index aortic procedure to aneurysm exclusion was 169 and 270 days in those undergoing 2- and 3-stage repair strategies, respectively. The ascending aorta and aortic arch were treated with 19 surgical and 3 endovascular TAR procedures. Three (16%) surgical arch procedures were performed elsewhere, and perioperative details were unavailable. Mean bypass, cross-clamp, and circulatory arrest times were 295±57, 216±63, and 46±11 minutes, respectively. There were 4 major adverse events (MAEs) in 2 patients: both required postoperative hemodialysis, 1 had postbypass cardiogenic shock necessitating extracorporeal membrane oxygenation, and the other required evacuation of an acute-on-chronic subdural hematoma. Thoracoabdominal aortic aneurysm repair was performed with 17 manufactured endografts and 5 PMEGs. There was no early mortality. Six (27%) patients experienced MAEs. There were 4 (18%) cases of spinal cord injury with 3 (75%) experiencing complete symptom resolution before discharge. Mean follow-up was 30±17 months in which there were 5 patient deaths-0 aortic related. Eight patients required ≥1 secondary intervention, and 6 target arteries demonstrated instability (3 IC, 1 IIIC endoleaks; 2 TA stenoses). Kaplan-Meier 3-year estimates of patient survival, freedom from secondary intervention, and target artery instability were 78±8%, 56±11%, and 68±11%, respectively.

Complete aortic repair with staged surgical or endovascular TAR and distal FB-EVAR is safe and effective with satisfactory morbidity, mid-term survival, and target artery outcomes.Clinical ImpactThe presented study demonstrates that repair of the entirety of the aorta - via total endovascular or hybrid means- is safe and effective with low rates of spinal cord ischemia. Cardiovascular specialists within comprehensive aortic teams at should feel confident that staged repair of the most complex degenerative and post-dissection thoracoabdominal aortic aneurysms can be safely performed in their patients with complication profile similar to that of less extensive repairs. Meticulous and intentional case planning is imperative for immediate and long-term success.

During total arch replacement (TAR) using frozen elephant trunk (FET) technique with Frozenix for true thoracic aortic aneurysm (tTAA), oversized FET tends to be chosen similar to the endovascular devise selection. However, the oversized FET is considered a risk factor for intimal injury. The appropriate size selection of FET remains insufficiently understood.

Between October 2014 and March 2022, a total of 49 patients underwent TAR using Frozenix for tTAA. Out of 49 patients, four patients planned to staged surgery were excluded, 19 patients were operated on with an undersized Frozenix compared with the descending aorta (undersized FET group) and in 26 patients an equal or oversized Frozenix was used (oversized FET group). Clinical outcomes and postoperative diameter changes were investigated.

In-hospital mortality was 0%. The mean diameter of Frozenix and the descending aorta was 30.7 mm and 28.8 mm, respectively, in the oversized FET group, and 26.7 mm and 30.1 mm in the undersized FET group. Postoperative computed tomography (CT) demonstrated no endoleaks not only in the oversized FET group but also in the undersized FET group. CT also revealed that undersized FET had expanded more than the original diameter in all cases except for two, with an average of 2.47 ± 1.53 mm. Additionally, the descending aorta covered with Frozenix shrank in 10 patients (53%). Postoperative adverse aortic events were not observed.

Undersized Frozenix tightly fit the descending aorta and resulted in complete sealing without endoleaks. Oversized FET is not strictly necessary considering the size-related adverse complications.

Larry A. Allen, MD, MHS, FAHA, FACC; Mats Börjesson, MD, PhD, FACC; Alan C. Braverman, MD, FACC; Julie A. Brothers, MD; Silvia Castelletti, MD, MSc, FESC; Eugene H. Chung, MD, MPH, FHRS, FAHA, FACC; Timothy W. Churchill, MD, FACC; Guido Claessen, MD, PhD; Flavio D'Ascenzi, MD, PhD; Douglas Darden, MD; Peter N. Dean, MD, FACC; Neal W. Dickert, MD, PhD, FACC; Jonathan A. Drezner, MD; Katherine E. Economy, MD, MPH; Thijs M.H. Eijsvogels, PhD; Michael S. Emery, MD, MS, FACC; Susan P. Etheridge, MD, FHRS, FAHA, FACC; Sabiha Gati, BSc (Hons), MBBS, PhD, MRCP, FESC; Belinda Gray, BSc (Med), MBBS, PhD; Martin Halle, MD; Kimberly G. Harmon, MD; Jeffrey J. Hsu, MD, PhD, FAHA, FACC; Richard J. Kovacs, MD, FAHA, MACC; Sheela Krishnan, MD, FACC; Mark S. Link, MD, FHRS, FAHA, FACC; Martin Maron, MD; Silvana Molossi, MD, PhD, FACC; Antonio Pelliccia, MD; Jack C. Salerno, MD, FACC, FHRS; Ankit B. Shah, MD, MPH, FACC; Sanjay Sharma, BSc (Hons), MBChB, MRCP (UK), MD; Tamanna K. Singh, MD, FACC; Katie M. Stewart, NP, MS; Paul D. Thompson, MD, FAHA, FACC; Meagan M. Wasfy, MD, MPH, FACC; Matthias Wilhelm, MD.

This American Heart Association/American College of Cardiology scientific statement on clinical considerations for competitive sports participation for athletes with cardiovascular abnormalities or diseases is organized into 11 distinct sections focused on sports-specific topics or disease processes that are relevant when considering the potential risks of adverse cardiovascular events, including sudden cardiac arrest, during competitive sports participation. Task forces comprising international experts in sports cardiology and the respective topics covered were assigned to each section and prepared specific clinical considerations tables for practitioners to reference. Comprehensive literature review and an emphasis on shared decision-making were integral in the writing of all clinical considerations presented.

This American Heart Association/American College of Cardiology scientific statement on clinical considerations for competitive sports participation for athletes with cardiovascular abnormalities or diseases is organized into 11 distinct sections focused on sports-specific topics or disease processes that are relevant when considering the potential risks of adverse cardiovascular events, including sudden cardiac arrest, during competitive sports participation. Task forces comprising international experts in sports cardiology and the respective topics covered were assigned to each section and prepared specific clinical considerations tables for practitioners to reference. Comprehensive literature review and an emphasis on shared decision-making were integral in the writing of all clinical considerations presented.

Accumulating evidence indicates associations between ambient air pollution and Kawasaki disease (KD), but the results remain inconsistent.

This systematic review and meta-analysis aimed to comprehensively summarize the current evidence on the effects of ambient air pollutants on KD.

The PubMed, Web of Science, Embase, and Scopus databases were searched up to January 18, 2025 for studies investigating the effects of ambient air pollution on KD. A fixed- or random-effects model was used to calculate pooled ORs with 95% CIs for an increase in ambient air pollutant concentration of 10 μg/m3. The risk of bias was assessed using the Risk of Bias In Nonrandomized Studies of Exposures tool, and the quality of evidence was assessed by the Grading of Recommendations, Assessment, Development, and Evaluations framework. The protocol was registered with PROSPERO (CRD42024545321).

Thirteen studies with 124,857 participants were included. Seven studies were at high risk of bias. The meta-analysis revealed an increased risk of KD after short-term postnatal exposure to PM2.5 (OR: 1.011; 95% CI: 1.003-1.019; I2 = 0%; high-quality evidence) and PM10 (OR: 1.004; 95% CI: 1.000-1.008; I2 = 38%; high-quality evidence), as well as long-term postnatal exposure to PM2.5 (OR: 1.415; 95% CI: 1.179-1.697; I2 = 41%; high-quality evidence). Prenatal exposure to carbon monoxide, nitric oxide, nitrogen oxides, and sulfur dioxide; short-term postnatal exposure to nitric oxide; and long-term postnatal exposure to carbon monoxide and nitrogen oxides were also associated with KD occurrence.

Both prenatal and postnatal exposure to several ambient pollutants are associated with the risk of KD.

Macrophage activation syndrome (MAS) is a hyperinflammatory and potentially fatal complication associated with rheumatologic disorders. In Kawasaki disease (KD), MAS is a rare and poorly described condition, making its differentiation from a severe, treatment-resistant presentation of KD particularly challenging.

We aimed to describe MAS in KD by analyzing its epidemiological, clinical, and laboratory characteristics, complications, therapeutic strategies, and outcomes.

A comprehensive literature review of PubMed, Embase, Scopus, and Cochrane Library was conducted to identify English-language studies on KD complicated by MAS, including case reports and case series, until 15 November 2024.

A total of 176 pediatric patients (60 females; median age 4 years, range 0.13-17) from 48 articles were included. MAS occurred after or simultaneously with KD diagnosis in 174/176 cases (99%). Common features included fever (100%), splenomegaly (49.4%), and hyperferritinemia (98.2%). Cardiac involvement was reported in 37% of children. The HLH-2004 criteria were met in 63% of cases, while the 2016 Ravelli criteria for MAS complicating systemic juvenile idiopathic arthritis were met in 94%. Treatment included additional doses of IVIG (36.2%), GCs (82.8%), cyclosporine A (28.7%), and biologics (13.8%), with complete MAS resolution in 93% of cases.

MAS in KD is a rare but severe complication, with overlapping features that make its differentiation from severe and resistant KD challenging. Persistent fever despite initial IVIG administration, along with splenomegaly and hyperferritinemia, emerge as key warning signs. Ravelli criteria provide stronger diagnostic support compared to the HLH-2004 criteria. Moreover, MAS is associated with increased cardiac involvement.

Aneurysmal Coronary Artery Disease (ACAD) can occur as localized dilations of a segment of one or more coronary arteries or diffuse ectasia-type dilatations of one or more coronaries. Atherosclerosis remains the most common cause of these aneurysms, with Kawasaki Disease being implicated in the Asian population. We present a case of a 62-year-old Asian woman who dies suddenly with no prior symptoms. and underwent an autopsy. Her heart showed diffuse aneurysmal dilatations of the epicardial coronary arteries, with a giant saccular aneurysm of the left main coronary artery. Histopathology revealed medial degeneration of the aneurysmal wall with no evidence of atheroma or vasculitis. Following a review of the pathological causes of coronary aneurysms, it is likely that the cause of such diffuse dilatations of the coronaries could be previous vasculitis such as Kawasaki disease.

Pediatric Kawasaki disease (KD) patients showing resistance to intravenous immunoglobulin (IVIG) are at risk of coronary artery lesions; thus, early prediction of IVIG resistance is particularly important. Herein, we aimed to develop and verify a novel predictive risk model for IVIG resistance in KD based on meta-analyses.

PubMed, Embase, and Web of Science databases were searched for cohort studies on the risk factors for IVIG resistance from January 2006 to December 2022. Data were extracted from the screened literature, followed by quality assessment using the Newcastle-Ottawa scale. meta-analyses used Stata 17.0 software to extract the risk factors with significant combined effect sizes and combined risk values, followed by logistic regression prediction model construction. The model was prospective validated using data from 1007 pediatric KD cases attending the Children's Hospital of Soochow University. The model's predictive ability was assessed using the Hosmer-Lemeshow test and area under the receiver operating characteristic curve (AUC) and the clinical utility was assessed using decision curve analysis(DCA).

Fifteen cohort studies reporting 4273 patients with IVIG resistance were included. The incidence of IVIG resistance was 16.2%. Six risk factors were reported ≥ 3 times with significant results for the combined effect size: male sex, rash, cervical lymphadenopathy, % neutrophils ≥ 80%, Age ≤ 12 months and platelet count ≤ 300 × 109/L. The logistic scoring model had 83.8% specificity, 70.4% sensitivity, and an optimal cut-off value of 23.500.

The risk prediction model for IVIG resistance in KD showed a good predictive performance, and pediatricians should pay high attention to these high-risk patients and develop an appropriate individual regimens to prevent coronary complications.

Coronary artery lesions constitute a significant complication of Kawasaki disease (KD) and represents one of the primary etiologies of acquired cardiovascular disease in pediatric populations. In the present study, we observed a downregulation of MEF2C expression in the whole blood of KD patients and in human coronary artery endothelial cells (HCAECs) during the pathophysiological progression of KD. Furthermore, transcriptomic data analysis, in conjunction with observations from HCAECs stimulated with KD serum, indicates that the downregulation of MEF2C in KD is correlated with increased inflammatory levels and the activation of inflammatory pathways. Overexpression of MEF2C has the potential to mitigate inflammation and apoptosis in HCAECs, whereas MEF2C knockdown exhibits contrary effects. Furthermore, MEF2C overexpression may alleviate inflammation and apoptosis in the coronary endothelium, attenuate abdominal aortic dilation, and prevent the decline of cardiac function in a CAWS-induced KD murine model. Mechanistically, MEF2C overexpression safeguards against KD-induced endothelial barrier disruption and the downregulation of endothelial junction proteins in coronary injury associated with KD. Additionally, through RNA sequencing, we identified that KLF2 might be involved in the MEF2C-mediated protection against coronary endothelial injury. Employing a gene interference methodology, we substantiated that MEF2C mitigates coronary artery injury in KD via KLF2-regulated endothelial barrier protection in HCAECs. These findings suggest that MEF2C could serve as a potential therapeutic target for the prevention and treatment of coronary lesions in KD.

Giant coronary artery aneurysms and myocardial fibrosis after Kawasaki disease may lead to devastating cardiovascular outcomes. We characterised the vascular and myocardial outcomes in five selected Kawasaki disease patients with a history of giant coronary artery aneurysms that completely regressed.

Five patients were selected who had giant coronary artery aneurysm in early childhood that regressed when studied 12-33 years after Kawasaki disease onset. Coronary arteries were imaged by coronary CT angiography, and coronary artery calcium volume scores were determined. We used endocardial strain measurements from CT imaging to assess myocardial regional wall function. Calprotectin and galectin-3 (gal-3) as biomarkers of inflammation and myocardial fibrosis were measured by enzyme-linked immunosorbent assay.

The five selected patients with regressed giant coronary artery aneurysms had calcium scores of zero, normal levels of calprotectin and gal-3, and normal appearance of the coronary arteries by coronary computed tomography angiography. CT strain demonstrated normal peak systolic and diastolic strain patterns in four of five patients. In one patient with a myocardial infarction at the time of Kawasaki disease diagnosis at the age of 10 months, CT strain showed altered global longitudinal strain, reduced segmental peak strain, and reduced diastolic relaxation patterns in multiple left ventricle segments.

These patients illustrate that regression of giant aneurysms after Kawasaki disease is possible with no detectable calcium, normal biomarkers of inflammation and fibrosis, and normal myocardial function. Individuals with regressed giant coronary artery aneurysm still require longitudinal surveillance to assess the durability of this favourable outcome.

The objective of this study was to investigate the role of infections in the pathogenesis of Kawasaki disease.

The investigation was a nationwide epidemiological case-control study, comprising all cases of Kawasaki disease diagnosed in Sweden 1987-2018. Controls were randomly sampled from the general population, matched on sex, age, and area of residency. Data on infections were obtained from the Swedish National Patient Register, which prospectively collects data on all Swedish residents. Infections were classified by organ system, infectious agent and by temporal proximity to Kawasaki disease diagnosis date. Prescription of antibiotics and infections in family members were also considered in separate analyses.

The study comprised n=1774 (61% male) cases and n=17 731 controls. Overall, a history of infections was associated with Kawasaki disease with an OR of 2.3 (95% CI 2.0 to 2.5). Respiratory, skin, urogenital and gastrointestinal tract infections were all associated with Kawasaki disease. Temporal stratification revealed a prominent clustering of infections during the weeks before a Kawasaki diagnosis, but also higher frequencies of infections several months preceding Kawasaki disease with OR ranging from 5.1 (95% CI 3.6 to 7.1) 15-28 days to 1.3 (95% CI 1.1 to 1.6) 181-365 days prior Kawasaki disease. A dose-response relationship was observed, with repeated infections associating with higher ORs of Kawasaki.

The findings suggest that infections are closely linked with Kawasaki disease, and with a wider temporal association than previously known. Further, the data imply that many different agents may induce the disease.

Kawasaki disease (KD) primarily affects the pediatric population and exhibits a notable incidence of drug resistance, resulting in coronary artery damage and thrombosis. This study aimed to identify innovative therapeutic targets for KD treatment. By harnessing single-cell data derived from peripheral blood mononuclear cells, we identified differentially expressed genes. Through the integration of eQTL data and Mendelian randomization analysis, we identified FCGR3B and S100A12 were causally linked to KD. The DrugBank database showed their potential as drug target candidates. GSEA further elucidated their roles on coronary artery damage and thrombosis. Furthermore, we have confirmed that the ligand-FCGR3B complex enhances the intracellular calcium concentration (Ca2+) within the cytoplasm, which in turn accelerates the secretion of S100A12, a pro-inflammatory cytokine that targets endothelial cells, from neutrophils. By integrating existing research, we proposed a synergistic effect that FCGR3B-S100A12 pathway positively modulates the development of coronary artery damage and thrombus formation, suggesting their perspectives in clinical treatment.

Kawasaki disease (KD) is a leading cause of acquired heart disease in children, often resulting in coronary artery complications such as dilation, aneurysms, and stenosis. While intravenous immunoglobulin (IVIG) is effective in reducing immunologic inflammation, 10-15% of patients do not respond to initial therapy, and some show resistance even after two consecutive treatments. Predicting which patients will not respond to these two IVIG treatments is crucial for guiding treatment strategies and improving outcomes. This study aimed to forecast resistance to two consecutive IVIG treatments using advanced machine learning models based on clinical and laboratory data. Data from the 9th National Kawasaki Disease Patient Survey by the Korean Kawasaki Disease Society encompassing 15,378 patients (mean age 33.0 ± 24.8 months; sex ratio 1.4:1) were used. Clinical and laboratory findings included white blood cell count, absolute neutrophil count (ANC), platelet count, erythrocyte sedimentation rate, serum protein, aspartate aminotransferase, alanine aminotransferase, total bilirubin, N-terminal pro-brain natriuretic peptide, and presence of pyuria. Machine learning models, including Logistic Regression (LR), Multi-Layer Perceptron (MLP), Random Forest (RF), CATBoost, Explainable Boosting Machine (EBM), and Gradient Boosting Machine (GBM), were applied to predict treatment resistance. The machine learning models achieved Area Under the Receiver Operating Characteristic Curve (AUROC) values between 0.664 and 0.791, with the GBM model exhibiting the highest AUROC of 0.791. Analysis of feature importance revealed that ANC, serum protein, platelet count, and C-reactive protein (CRP) levels were the most significant predictors of treatment resistance. The cutoff values for these predictors were 7,860/mm³ for ANC, 7.0 g/dL for serum protein, 519,000/mm³ for platelet count, and 10.4 mg/dL for CRP. Among the patients, 12.2% were refractory to the first IVIG infusion, and 2.8% did not respond to the second IVIG treatment. Additionally, 13.1% of these patients had confirmed coronary artery dilatation (CAD) in the acute phase, and 4.7% developed CAD after the acute phase. Machine learning models effectively predict resistance to consecutive IVIG treatments, allowing for early identification of high-risk patients. Key predictors include ANC, serum protein, platelet count, and CRP levels. These findings can guide personalized treatment strategies and improve outcomes for Kawasaki Disease.

The impact of coronary artery aneurysms (CAAs) caused by Kawasaki disease (KD) on long-term health-related quality of life (HRQOL) in children has not been well documented.

This study investigated long-term HRQOL in a large sample of children diagnosed with KD-related CAAs. A case-control, retrospective study included 66 patients with KD-related CAAs. A total of 98 hospitalized patients were matched as controls based on age and sex: 49 patients were allocated to a group with pneumonia and 49 patients were allocated to a group with arterio-arterial fistula. Both child-reported and parent-proxy-reported Pediatric Quality of Life Inventory surveys were collected.

The median (IQR) follow-up period was 5.64 (3.81-7.47) years (range, 1.03-10.67 years). The mean (SD) age at diagnosis was 3.73 (1.93) years. At baseline, children and parents as their proxies reported similar HRQOL scores for KD-related CAAs and arterio-arterial fistula that were considerably lower than for pneumonia, respectively. At long-term follow-up, children in the small and medium-sized aneurysms group reported a mean (SD) score of 81.61 (19.50), which was comparable to the arterio-arterial fistula group (83.32 [18.24]), 9.51 points lower than that of the pneumonia group (P = .014), and 9.70 points higher than that of the giant aneurysms group (P = .012). Parents also reported a comparable mean (SD) score of 81.03 (12.57) vs 83.30 (15.17) in the small and medium-sized aneurysms group and arterio-arterial fistula group, both of which had statistically significantly lower scores than the pneumonia group (P = .010) and higher scores than the giant aneurysms group (P = .009).

Despite improvement in HRQOL scores, children with documented KD-related CAAs without complete recovery often encountered issues that disrupted their well-being during long-term follow-up. Routine outpatient HRQOL screening could be instituted to help eliminate the risk of long-term disability following initial clinical improvement.

To evaluate the use of noncontrast-enhanced coronary magnetic resonance angiography (NC-CMRA) for long-term follow-up in patients with Kawasaki disease (KD). In total, 40 (77 aneurysms) patients with KD who underwent NC-CMRA were retrospectively analyzed. Coronary artery aneurysms and dilations observed during the acute phase were classified into three groups according to their diameter based on the American Heart Association criteria. The prevalence of coronary artery stenotic lesions was evaluated using the Kaplan-Meier method (log-rank test). The agreement in the coronary artery stenosis rates between NC-CMRA and coronary angiography (CAG) was examined via Brand-Altman analysis and intraclass correlation coefficients (ICC). In patients with large or giant aneurysms, the prevalence of coronary artery stenotic lesions was 26.3% at 10 years, 53.2% at 15 years, and 71.9% at 20 years. In patients with medium aneurysms, the prevalence of coronary artery stenotic lesions was 8.4% at 10 and 15 years and 23.7% at 20 years. Patients with small aneurysms did not exhibit stenotic lesions. Patients with large or giant aneurysms had significantly higher rate of coronary stenotic lesions than those with medium and small aneurysms (p < 0.05). All 16 stenotic lesions detected on NC-CMRA were consistent with those observed on CAG, and the coronary artery stenotic rate had moderate consistency (ICC 0.65). In KD, the detection of coronary artery stenosis using NC-CMRA was consistent with that using CAG. Therefore, NC-CMRA can be a better alternative following echocardiography for long-term coronary artery evaluation in patients with KD.

Kawasaki disease (KD) manifests as an acute febrile condition and systemic vasculitis, the etiology of which remains elusive. Primarily affecting children under 5 years of age, if untreated KD can lead to a significant risk of coronary artery aneurysms and subsequent long-term cardiovascular sequelae, including myocardial ischemia and myocardial infarction. Intravenous immunoglobulin therapy mitigates the risk of aneurysm formation, but a subset of patients exhibit resistance to this treatment, increasing the susceptibility of coronary artery lesions. Furthermore, the absence of a KD-specific diagnostic test or biomarkers complicates early detection and appropriate treatment. Experimental murine models of KD vasculitis have substantially improved our understanding of the disease pathophysiology, revealing the key roles of the NLRP3 inflammasome and interleukin-1 (IL-1) signalling pathway. This review aims to delineate the pathophysiologic findings of KD while summarising the findings for the emerging key role of IL-1β in its pathogenesis, derived from both human data and experimental murine models, and the translational potential of these findings for anti-IL-1 therapies for children with KD.

To explore the clinical characteristics, related factors, and prognosis of Kawasaki disease (KD) combined with acute febrile cholestasis and improve the understanding of the liver complications of KD to avoid misdiagnosis and missed diagnosis.

We retrospectively analyzed the demographic, clinical, and laboratory data of 1803 patients with KD between January 2019 and July 2023 in our hospital. Based on the presence of cholestasis, patients were divided into the cholestatic and control groups. Logistic regression analysis was performed for the statistically significant indicators between the two groups to examine the risk factors for KD with coronary artery abnormalities (CAA) and intravenous immunoglobulin (IVIG) unresponsiveness. Additionally, patients with KD and cholestasis were compared with patients with acute febrile cholestasis due to other causes during the same period.

Compared to the control group (n = 1720), patients in the cholestatic group (n = 83) were older, had higher levels of white blood cell count (WBC), C-reactive protein (CRP), D-dimer, N-terminal pro-brain natriuretic peptide (NT-proBNP), a shorter fever duration, and high incidences of IVIG unresponsiveness and CAA. KD was the leading cause of acute febrile cholestasis in children (72.6%). In the multivariate logistic regression analysis, younger age, cholestasis, hypoalbuminemia, and a high NT-proBNP level were risk factors for IVIG unresponsiveness, whereas male sex, longer fever duration before treatment, and high alanine aminotransferase (ALT), and CRP levels were risk factors for CAA.

KD with cholestasis was associated with a higher risk of IVIG unresponsiveness and coronary artery abnormalities. KD was the leading cause of acute febrile cholestasis in children. Attention to the possibility of KD is warranted in children with acute febrile cholestatic jaundice, especially if associated with elevated WBC, CRP, and D-dimer levels, or hypoalbuminemia.

Patients with Kawasaki disease (KD) with coronary artery involvement require long-term cardiac care. Although respective evidence-based recommendations are missing, cardiac catheterization is still considered the gold standard for diagnosing detailed coronary pathology. Therefore, to better understand coronary artery pathology development, we conducted a survey to document and evaluate cardiac catheterization data in a European population.

We retrospectively analyzed cardiac catheterization data from KD children from the year 2010 until April 2023. This registry covers basic acute-phase clinical data, and more importantly, detailed information on morphology, distribution, and the development of coronary artery pathologies. A total of 164 mainly White patients (65% boys) were included. A relevant number of patients had no coronary artery aneurysm (CAA) at the cardiac catheterization, indicating that distal CAAs were almost exclusively detected alongside proximal CAAs. Patients with multiple CAAs revealed a significant positive correlation between the number of CAAs and their dimensions in diameter and in length. Location of the CAA within the coronary artery, age at onset of KD, or natal sex did not significantly influence CAA diameters, but CAAs were longer in older children and in boys.

That distal CAAs were only present together with proximal ones will hopefully reduce diagnostic CCs in patients with KD without echocardiographically detected proximal CAAs. Furthermore, this study gives valuable insights into dimensional specifics of CAAs in patients with KD. As an ongoing registry, future analyses will further explore long-term outcomes and performed treatments, helping to refine clinical long-term strategies for patients with KD.

URL: https://drks.de/; Unique Identifier: DRKS00031022.

This study is to investigate whether angiotensin type 1 receptor blockers (ARBs) or angiotensin-converting enzyme inhibitors (ACEis) can regress coronary artery aneurysm (CAA) in patients with Kawasaki disease (KD).

This multicenter, prospective, observational study was conducted at 53 institutions throughout Japan. We enrolled patients who were diagnosed with KD after January 2015 and had a medium or large CAA (maximum luminal diameter ≥ 4 mm or z score ≥  + 5) 30 days or later after KD onset.

Of the 209 patients, 47 (22%) were taking ARBs/ ACEis. Compared with those in the non-ARB/ACEi group, the baseline CAA diameter was significantly greater (6.7 mm vs. 5.5 mm, p < 0.01), and bilateral CAA (70% vs. 59%, p = 0.01) and giant CAA (32% vs. 20%, p = 0.08) were more frequently observed in the ARB/ACEi group. Although the overall regression rates did not differ between the groups (67% vs. 65%), the regression rates of giant CAA were approximately 1.6 times greater in the ARB/ACEi group than in the non-ARB/ACEi group (36% vs. 23%). Multivariate Cox regression analysis after adjustment for other clinical variables suggested that ARBs/ACEis may be a factor in CAA regression (hazard ratio [HR]: 1.5, 95% confidence interval [CI]: 0.91-2.46).

Although ARBs/ ACEis were used more frequently in patients with severe CAA, these patients had similar CAA regression rates to patients not taking ARBs/ACEis. ARBs/ACEis may be beneficial agents aimed at inducing CAA regression in KD patients.

• Large CAAs are less likely to regress and are always at risk of life-threatening cardiac events. • Moderate CAA, age less than 1 year, and female sex have been reported to be factors that promote the regression of CAA.

• Although ARBs/ACEis were used more frequently in patients with severe CAA, these patients had a similar rate of CAA regression to patients who did not take ARBs/ACEis. • The regression rates of giant CAA were approximately 1.6 times greater in the ARB/ACEi group than in the non-ARB/ACEi group.

The giant coronary artery aneurysm is a very rare finding (0.02% of the general population), in which the right coronary artery is most often affected by aneurysms. Herein, we present a rare case of a giant left coronary artery aneurysm involving multiple major coronary arteries and compressing the cardiac cavity. The giant coronary artery aneurysm was opened and explored under cardiopulmonary bypass, and many mixed thrombi and calcified tissue were exposed. The patient was discharged uneventfully 7 days after surgery. The best management strategy at present is based on case reports, small case series, and personal experience. Treatment must be individualized according to the aetiology, location, symptoms, size, disease progression, the existence of infection, and the degree of any coexisting atherosclerosis. Surgery is a good alternative, particularly if a giant coronary artery aneurysm has a high risk of rupture and compressing the cardiac cavity. Even today, the treatment strategy is still open to debate and a clear evidence-based management strategy has not been established.

Giant coronary artery aneurysms are rare but potentially fatal complications of Kawasaki disease. The lack of evidence-based recommendations on their management and treatment cause guidelines and practices to differ. We aimed to assess these variations.

An anonymous online survey regarding surveillance, imaging, pharmacological management, and interventional practices was distributed among 134 physicians attending to Kawasaki disease patients worldwide. A p-value of <0.05 was deemed significant.

The majority (60%) of respondents were general paediatric cardiologists, and 29% interventional specialists. The average years in practice was 15 ± 9.6. Physicians from Asia had the most experience with giant coronary artery aneurysms. American practitioners preferred combining anticoagulants with aspirin. Beta-blockers and statins were more likely used in teenagers versus younger children. Cardiac catheterisation was most (52%) chosen for coronary surveillance in patients with echocardiogram anomalies, followed by Coronary CT-angiography. The indications for coronary intervention were split among respondents, regardless of geographic region or experience. The preferred treatment of coronary stenosis was percutaneous intervention (69%) versus bypass surgery. For thrombosis, thrombolytics (50%) were preferred over percutaneous (39%) and surgical (11%) interventions. Most (92%) preferred intervening in young children in a paediatric facility but were split between a paediatric and adult facility for older children. Most chose combined management by adult and paediatric specialists for either age-scenarios (70, 82%).

As identified by our study, the lack of large studies and evidence-based recommendations cause uncertainty and ambivalence towards certain treatments. International collaborative efforts are needed to provide more robust evidence in the management of these patients.

To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAAs) at a single US center.

Medical records for all patients with KD and giant CAAs at a pediatric academic institution were reviewed. Primary outcomes included major adverse cardiovascular events (MACE) and normalization of CA luminal diameter, using Kaplan-Meier analyses.

There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with a median age at diagnosis of 0.9 years (range, 0.2-13.3 years). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 patients (21.7%) at a median of 1.4 years (range, 0.04-22.6 years) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z scores of ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to a normal luminal diameter at a median of 3.6 years (range, 0.6-12.0 years). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%.

Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z scores of ≥20. Despite regression to a normal luminal diameter in >25% of CAAs, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.

Kawasaki disease (KD) is the most common vasculitis in children, and can result in the development of coronary artery aneurysms (CAAs) if not properly managed. While intravenous immunoglobulin (IVIG) and aspirin are standard first-line treatments, refractory KD may develop, increasing the risk of coronary complications. Herein, we report the case of a young girl with KD who initially responded to IVIG, but later developed a giant CAA, despite additional treatments. Infliximab stabilized her condition, and one year later, the CAA remained stable without thrombus formation. This case demonstrates that clinical appearance, particularly fever, may not fully reflect the patient's condition, as fever can subside immediately after treatment, but may relapse days later, underscoring the need for vigilant monitoring.

The frozen elephant trunk (FET) technique is effective for treating extended aortic arch aneurysms. This study compares hand-made and factory-made devices in this context.

A retrospective case-control study was conducted on 68 patients who underwent FET for distal aortic arch aneurysm at our institution. We used two different types of devices: hand-made stent graft in group Z (17 cases, 25.0%) and a commercialized stent graft in group J (51 cases, 75.0%). The study compared demographic characteristics and the outcomes between the two groups.

In-hospital mortality was equivalent in both groups (5.9%). Spinal cord injury rates were 5.9% in group Z and 3.9% in group J. Group Z had a higher rate of aortic events (55.9% vs 96.9%, p < 0.001) and more frequent stent migration. The number of cases with aneurysm diameter shrinkage was lower in group Z. The landing zone angle at insertion influenced aneurysm changes, being 17.6 degrees in shrink cases and 26.7 degrees in unchanged or enlarged cases (p = 0.045).

FROZENIX device notably reduced incidences of postoperative complications compared to hand-made prostheses. Factors such as insertion angle and stent size, rather than changes in the landing zone angle, appeared to influence aneurysm shrinkage. The use of FROZENIX in TARFET procedures has shown benefits in reducing complications and improving long-term prognosis, taking into account the landing zone angle.

Kawasaki disease (KD) causes vascular injury and lifelong remodeling. Excessive intimal proliferation has been observed, resulting in coronary artery lesions (CALs). However, the mechanisms underlying vascular remodeling in CAL and statin treatment have not been comprehensively elucidated. This study aimed to investigate the effects of statins on vascular remodeling using a KD mouse model. Candida albicans water-soluble substance (CAWS) was intraperitoneally injected in 5-week-old male apolipoprotein-E-deficient mice. They were categorized as follows (n = 4): control, CAWS, CAWS+statin, and late-statin groups. The mice were euthanized at 6 or 10 weeks after injection. Statins (atorvastatin) were initiated after CAWS injection, except for the late-statin group, for which statins were internally administered 6 weeks after injection. Elastica van Gieson staining and immunostaining were performed for evaluation. Statins substantially suppressed the marked neointimal hyperplasia induced by CAWS. Additionally, CAWS induced TGFβ receptor II and MAC-2 expression around the coronary arteries, which was suppressed by the statins. KD-like vasculitis might promote the formation of aneurysm by destroying elastic laminae and inducing vascular stenosis by neointimal proliferation. The anti-inflammatory effects of statins might inhibit neointimal proliferation. Therefore, statin therapy might be effective in adult patients with KD with CAL by inhibiting vascular remodeling.

Kawasaki disease (KD) is a pediatric vasculitis, leading to coronary artery aneurysms (CAAs) in ~4-14%. Attention to the etiology and course of KD was generated by the close mimic of a SARS-CoV-2-induced phenotype, called multisystem inflammatory syndrome in children (MIS-C).

A total of 1179 cases were collected from 2012 with ~50% of cases retrospectively included. Clinical characteristics were described and risk factors for CAA (persistence) were investigated. Phenotypic patterns of the prospectively included KD patients were evaluated. These patterns were also compared to the seronegative KD and seropositive MIS-C cases identified during the SARS-CoV-2 pandemic.

KD mostly affected boys and children < 5 years. IVIG resistance, CAAs, and giant CAAs occurred in 24.5%, 21.4%, and 6.6%, respectively. Giant CAAs were significantly more likely to normalize to a normal Z score in patients that were younger than 2.5 years old at the time of initial giant CAA (χ2 test p = 0.02). In our prospective (SARS-CoV-2-seronegative) KD series, there was a diminishing male predominance over time, whereas the proportions of incomplete presentations (p < 0.001) and patients with circulatory shock (p = 0.04) increased since the COVID-19 pandemic. Pre- and post-pandemic KD cases presented with different levels of C-reactive protein, thrombocyte counts, and hemoglobin levels over the years. Compared to pandemic KD, SARS-CoV-2-seropositive MIS-C patients were older (p < 0.001), and more often required intensive care admission (p < 0.001), with a gradual decrease over time between 2020 and 2022 (p = 0.04). KD carried a substantial risk of CAA development in contrast to MIS-C.

the phenotypic changes seen over the last twelve years of our prospective follow-up study suggest a spectrum of hyperinflammatory states with potentially different triggering events within this clinical entity.

Kawasaki disease (KD) is an acute vasculitis that primarily affects children under age 5 years. Approximately 20-25% of untreated children with KD and 3-5% of those treated with intravenous immunoglobulin therapy develop coronary artery aneurysms (CAAs). The prevalence of CAAs is much higher in male than in female patients with KD, but the underlying factors contributing to susceptibility to CAAs in patients with KD remain unclear. This study aimed to identify sex-specific susceptibility loci associated with CAAs in KD patients.

A sex-stratified genome-wide association study (GWAS) was performed using previously obtained GWAS data from 296 KD patients and a new replication study in an independent set of 976 KD patients by comparing KD patients without CAA (controls) and KD patients with aneurysms (internal diameter ≥5 mm) (cases).

Six male-specific susceptibility loci, PDE1C, NOS3, DLG2, CPNE8, FUNDC1, and GABRQ (odds ratios [ORs], 2.25-9.98; p=0.00204-1.96×10-6), and 2 female-specific susceptibility loci, SMAD3 (OR, 4.59; p=0.00016) and IL1RAPL1 (OR, 4.35; p=0.00026), were significantly associated with CAAs in patients with KD. In addition, the numbers of CAA risk alleles additively contributed to the development of CAAs in patients with KD.

A sex-stratified GWAS identified 6 male-specific (PDE1C, NOS3, DLG2, CPNE8, FUNDC1, and GABRQ) and 2 female-specific (SMAD3 and IL1RAPL1) CAA susceptibility loci in patients with KD.

Kawasaki disease (KD), a self-limiting vasculitis, can present with a broader spectrum of vascular involvements, necessitating early recognition and prompt management. This case exemplifies the importance of involving multiple teams on board in managing complex presentations of KD. It also highlights the importance of close monitoring for the progression of the disease spectrum as well as family education to ensure favorable outcomes. The case also emphasizes the importance of long-term follow-up and further research to understand the prognosis, early screening tools, and possible complications due to multi-organ involvement in KD along with their management strategies.

Kawasaki disease (KD) is a multisystem vascular inflammatory syndrome, which predominantly affects the small and medium vessels in children within the age group of less than 5 years. The most threatened complication is the development of coronary artery aneurysms (CAAs). We present an extremely rare case of KD in a 2-month, 21-day-old male infant with extensive vascular involvement, expanding the disease spectrum beyond the involvement of coronary arteries. These included aneurysmal dilatations of both internal carotid arteries, the descending aorta, bilateral multilevel intercostal arteries, coeliac artery, superior mesenteric artery, and both renal arteries. Implementing a multidisciplinary approach with early treatment via intravenous immunoglobulin (IVIG) and dexamethasone proved to be most effective in the patient's management. Despite unique challenges such as severe coronary dilation and pseudomonas sepsis during the special care, the patient was stabilized and discharged after 11 days of hospital stay, highlighting the importance of early prompt management and a centered approach to evaluate in a broader spectrum. This case emphasizes the importance of long-term follow-up and further research to understand the prognosis, early screening tools, and possible complications due to multi-organ involvement in KD along with their management strategies.

This study aimed to develop a novel scoring system utilizing circulating interleukin (IL) levels to predict resistance to intravenous immunoglobulin (IVIG) in Chinese patients with Kawasaki disease (KD). We further compared this scoring system against six previously established scoring methods to evaluate its predictive performance.

A retrospective analysis was conducted on KD patients who were treated at the cardiovascular medical ward of our institution from January 2020 to December 2022. Six scoring systems (Egami, Formosa, Harada, Kobayashi, Lan and Yang) were analyzed, and a new scoring system was developed based on our data.

In our study, 521 KD patients were recruited, 42 of whom (8.06%) were identified as resistant to IVIG. Our study indicated that IVIG-resistant KD patients were at an increased risk for the development of coronary arterial lesions (CALs) (P = 0.001). The evaluation of IVIG resistance using various scoring systems revealed differing levels of sensitivity and specificity, as follows: Egami (38.10% and 88.52%), Formosa (95.24% and 41.13%), Harada (78.57% and 43.22%), Kobayashi (66.67% and 74.95%), Lan (66.67% and 73.49%), and Yang (69.05% and 77.24%). Our novel scoring system utilizing sIL-2R demonstrated the highest sensitivity and specificity of 69.29% and 83.91%, respectively, and calibration curves indicated a favorable predictive accuracy of the model.

Our newly developed scoring system utilizing sIL-2R demonstrated superior predictive performance in identifying IVIG resistance among Chinese patients with KD.

It is important to maintain a vigilant approach towards incidental, rare findings like coronary artery aneurysms (CAAs) presenting with arrhythmia, particularly in patients with a history of myocardial infarction and cardiovascular risk factors. Diagnosis needs thorough evaluation. Tailoring management strategies to individual patient characteristics is crucial for optimizing outcomes.

Kawasaki disease, a systemic vasculitis that affects young children and can result in coronary artery aneurysms, is the leading cause of acquired heart disease among children. A hallmark of Kawasaki disease is increased blood platelet counts and platelet activation, which is associated with an increased risk of developing resistance to intravenous immunoglobulin and coronary artery aneurysms. Platelets and their releasate, including granules, microparticles, microRNAs and transcription factors, can influence innate immunity, enhance inflammation and contribute to vascular remodelling. Growing evidence indicates that platelets also interact with immune and non-immune cells to regulate inflammation. Platelets boost NLRP3 inflammasome activation and IL-1β production by human immune cells by releasing soluble mediators. Activated platelets form aggregates with leukocytes, such as monocytes and neutrophils, enhancing numerous functions of these cells and promoting thrombosis and inflammation. Leukocyte-platelet aggregates are increased in children with Kawasaki disease during the acute phase of the disease and can be used as biomarkers for disease severity. Here we review the role of platelets in Kawasaki disease and discuss progress in understanding the immune-effector role of platelets in amplifying inflammation related to Kawasaki disease vasculitis and therapeutic strategies targeting platelets or platelet-derived molecules.

Congenital heart diseases (CHDs) represent a heterogeneous group of congenital defects, with high prevalence worldwide. Non-invasive imaging is essential to guide medical and surgical planning, to follow the patient over time in the evolution of the disease, and to reveal potential complications of the chosen treatment. The application of cardiac magnetic resonance imaging (CMRI) in this population allows for obtaining detailed information on the defects without the necessity of ionizing radiations. This review emphasizes the central role of CMR in the overall assessment of CHDs, considering also the limitations and challenges of this imaging technique. CMR, with the application of two-dimensional (2D) and tri-dimensional (3D) steady-state free precession (SSFP), permits the obtaining of very detailed and accurate images about the cardiac anatomy, global function, and volumes' chambers, giving essential information in the intervention planning and optimal awareness of the postoperative anatomy. Nevertheless, CMR supplies tissue characterization, identifying the presence of fat, fibrosis, or oedema in the myocardial tissue. Using a contrast agent for angiography sequences or 2D/four-dimensional (4D) flows offers information about the vascular, valvular blood flow, and, in general, the cardiovascular system hemodynamics. Furthermore, 3D SSFP CMR acquisitions allow the identification of coronary artery abnormalities as an alternative to invasive angiography and cardiovascular computed tomography (CCT). However, CMR requires expertise in CHDs, and it can be contraindicated in patients with non-conditional devices. Furthermore, its relatively longer acquisition time and the necessity of breath-holding may limit its use, particularly in children under eight years old, sometimes requiring anesthesia. The purpose of this review is to elucidate the application of CMR during the pediatric age.

Kawasaki disease (KD) is the most common cause of acquired heart disease in childhood. Coronary artery lesions (CALs) are serious complications of KD that can result in stenosis and thrombosis, but the specific underlying pathogenic mechanisms have not been elucidated. Therefore, exploring biomarkers to help predict early CALs is urgently needed for clinical treatment.

Patients were recruited from three independent cohorts. In the discovery cohort, Data-Independent Acquisition Mass Spectrometry (DIA-MS) was performed to screen plasma proteins from healthy controls (HCs), KD patients prior to intravenous immunoglobulin (IVIG) treatment, and KD patients post-IVIG treatment. KD patients were further divided into KD patients without CALs (nCAL) and with CALs (CALs) groups. Bioinformatic analysis was carried out for the differentially expressed proteins (DEPs) and hub proteins. Candidate proteins were quantified by enzyme-linked immunosorbent assay (ELISA) in the validation cohort 1 and 2. Furthermore, candida albicans cell wall extract (CAWS)-induced KD vasculitis mice and cell models were established to investigate the expression of biomarkers identified in the aforementioned clinical cohort.

According to the quantitative proteomics analysis, SERPINE1 was significantly increased in KD patients with CALs. Receiver operating characteristic curves (ROC) revealed that plasma SERPINE1 exhibited greater ability in predicting CALs (AUC = 0.824, P < 0.0001). After IVIG treatment, the concentrations of SERPINE1 in the nCALs group significantly decreased. However, the concentration of SERPINE1 remained persistently elevated in the CALs group. Moreover, the expression of SERPINE1 was significantly upregulated in the heart tissue of KD mice, KD plasma, or tumor necrosis factor-α (TNF-α)-stimulated human coronary artery endothelial cells (HCAECs).

Overall, our results suggest that the plasma concentration of SERPINE1 might serve as a new potential predictive biomarker for CALs in KD patients.

Kawasaki disease (KD) is a self-limited febrile disease predominantly affecting infants and children under 5 years old. Coronary artery lesions (CAL) are a prevalent complication, highlighting the necessity for swift diagnosis and treatment. A comprehensive review of biomarkers applicable for the diagnosis and treatment of Kawasaki disease (KD) in clinical settings is imperative. To provide a comprehensive review and analysis of biomarkers for diagnosis of KD, incidence of CAL, and intravenous immunoglobulin (IVIG) resistance. The data included in our study were sourced from searches conducted in PubMed/MEDLINE, Embase, EBSCO, and Google Scholar until March 15, 2024. Studies investigating the association with KD or evaluating diagnostic value were included in our study. Eligibility was independently assessed by two authors, with conflicts resolved through discussion. Data extraction was performed by 2 independent authors, following Meta-analyses Of Observational Studies in Epidemiology (MOOSE) guideline. Data were pooled using a random-effects model. We assess biomarkers relevant to KD, categorizing them into three groups: diagnostic, associated with CAL incidence, and linked to IVIG resistance. For studies focusing solely on association, we present standardized mean differences (SMD). For those reporting sensitivity and specificity as diagnostic measures, we calculate the diagnostic odds ratio (DOR) to compare their efficacy. We identified 14 meta-analyses on biomarkers related to KD. 11 biomarkers exhibited diagnostic value for KD, while 21 were associated with its progression. Four biomarkers, including non-coding RNAs (DOR, 19.35 [95% CI, 13.58-27.56]), Serum ferritin (DOR, 24.90 [11.67-53.12]), N terminal proBNP (DOR, 21.03 [9.03-49.00]), and micro RNAs (DOR, 45.28 [6.30-325.52]), have significant diagnostic value for the diagnosis of KD. Seven biomarkers showed significant association with the incidence of CAL. Twenty biomarkers were for the prediction of IVIG resistance, including prognostic nutritional index (DOR, 7.72 [95% CI, 2.37-25.09]), non-coding RNAs (DOR, 14.63 [3.24-66.14]), neutrophil to lymphocyte ratio (DOR, 6.62 [4.05-10.81]), platelet to lymphocyte ratio (DOR, 3.30 [2.10-5.19]), and C reactive protein (DOR, 6.58 [3.69-11.74]). Based on the evidence, we have proposed various biomarkers associated with KD. Our aim is for these biomarkers to have wide applicability in both diagnostic and therapeutic settings.

Kawasaki disease (KD) is an acute febrile illness of childhood that can lead to coronary artery aneurysms (CAAs) and myocardial infarction. Intravenous immunoglobulin reduces the prevalence of CAA when given to patients with KD within 10 days of fever onset. Children with KD may undergo evaluation for other diagnoses before treatment, particularly those with incomplete KD criteria. If KD outcomes are improved with early treatment, a delay in treatment while evaluating for other causes might place these patients at risk.

We performed a retrospective cohort study of children treated for KD within the first 10 days of illness at our KD center from 2014 to 2021 to determine the prevalence of CAA by day of treatment.

A total of 290 patients met the study criteria. No statistically significant difference was found in the odds of developing a maximum z score ≥2.5 for each day of delayed treatment within 10 days of fever onset (adjusted odds ratio, 0.87; 95% CI, .72-1.05; P = .13). Subgroup analyses by age, sex, and year of treatment did not reveal a significant association between treatment day and maximum z score ≥2.5, although the number of patients <6 months of age was small.

Our study supports current recommendations. We found similar odds of developing adverse coronary outcomes regardless of treatment day within 10 days from fever onset.

Coronary microvascular disease (CMD) and impaired coronary blood flow control are defects that occur early in the pathogenesis of heart failure in cardiometabolic conditions, prior to the onset of atherosclerosis. In fact, recent studies have shown that CMD is an independent predictor of cardiac morbidity and mortality in patients with obesity and metabolic disease. CMD is comprised of functional, structural, and mechanical impairments that synergize and ultimately reduce coronary blood flow in metabolic disease and in other co-morbid conditions, including transplant, autoimmune disorders, chemotherapy-induced cardiotoxicity, and remote injury-induced CMD. This review summarizes the contemporary state-of-the-field related to CMD in metabolic and these other co-morbid conditions based on mechanistic data derived mostly from preclinical small- and large-animal models in light of available clinical evidence and given the limitations of studying these mechanisms in humans. In addition, we also discuss gaps in current understanding, emerging areas of interest, and opportunities for future investigations in this field.

Kawasaki disease (KD) is an acute systemic vasculitis that preferentially involves coronary arteries in young children, and predominantly affects young children. Cardiovascular lesions are the most severe complications of this disease. Even though giant aneurysms are rare, they can complicate thrombus formation, leading to myocardial ischemia, myocardial infarction, and even cardiac death. Later in life, it can lead to steno-occlusive lesions. Follow-up led to coronary artery stenosis. In this article, we report a case of a pediatric patient with KD who presented with a large thrombus within a giant coronary aneurysm as a consequence of delayed treatment with intravenous immunoglobulin (IVIG) and IVIG resistance, which contributed to the formation of coronary artery lesions. Transthoracic echocardiography is a valuable tool for detecting coronary artery abnormalities; however, computed tomography coronary angiography is valuable for precisely delineating coronary anatomy and complications. It is important to maintain a slightly higher international normalized ratio to decrease the risk of thrombosis in coronary artery aneurysms.