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Objectives To assess the scan time, image quality, and diagnostic performance of self-navigated coronary MR angiography (SN-CMRA) for coronary aneurysm (CAA) detection in Kawasaki disease (KD) patients and compare it with diaphragm-navigated CMRA (DN-CMRA). Materials and methods SN-CMRA and DN-CMRA were performed on 76 pediatric patients with KD (48 males, 6.75 ± 3.59 years). Thirty-three of whom underwent coronary CT angiography (CCTA)/invasive coronary angiography (ICA). The scan time and qualitative and quantitative image quality assessment were compared between the two sequences. The diagnostic performance for CAA detection by the two approaches using CCTA/ICA as the reference standard was compared on per-patient, per-vessel, and per-segment basis. Results The scan time of SN-CMRA was significantly shorter than that of DN-CMRA (7.49 ± 2.31 min vs. 10.03 ± 4.47 min, p  < 0.001). There was no difference in overall and segmental image quality to reach the clinical diagnostic criteria between the two sequences (all p  > 0.05). No significant difference in vessel length of the three main coronary arteries was found between the two approaches (all p  > 0.05). Moreover, SN-CMRA showed no difference from DN-CMRA in contrast ratio of blood-myocardium (1.25 (interquartile range [IQR], 1.06 to 1.51) vs. 1.18 (IQR, 0.95 to 1.64), p  = 0.706). There was no difference in the diagnostic accuracy of SN-CMRA and DN-CMRA for CAA detection on per-patient, per-vessel, or per-segment basis (all p  > 0.05). Conclusion SN-CMRA at 3T showed reliable diagnostic performance and application value for CAA detection in children with KD. Compared with DN-CMRA, SN-CMRA can simplify the scanning procedure and shorten the scan time, achieving comparable image quality and diagnostic accuracy. Clinical relevance statement Coronary aneurysm in children with Kawasaki disease (KD) can be detected by self-navigated coronary MR angiography (CMRA) non-invasively and without radiation, achieving comparable image quality and diagnostic performance as diaphragm-navigated CMRA while shortening scanning time. It can provide reference for risk stratification and treatment management of KD. Key Points • Evaluating the size of coronary aneurysm is important for risk stratification and treatment of Kawasaki disease. • Self-navigated coronary MR angiography (SN-CMRA) shortens scan time and achieves comparable image quality and diagnostic performance compared with diaphragm-navigated coronary MR angiography. • SN-CMRA can evaluate coronary aneurysm non-invasively and without radiation, providing information for risk stratification and treatment.

Background Kawasaki disease shock syndrome (KDSS) is a severe and uncommon complication of Kawasaki disease (KD). Grisel’s syndrome (GS), a rare non-traumatic atlantoaxial subluxation, has been seldom described in KD. To our knowledge, this is the first documented case in which GS was the initial manifestation of KD and preceded the development of KDSS. Case presentation A 4-year-old boy presented with fever, cervical lymphadenopathy, and restricted neck mobility. Cervical computed tomography revealed atlantoaxial subluxation. He was initially diagnosed with cervical lymphadenitis and treated with antibiotics, but his symptoms persisted and rapidly progressed to shock. He was subsequently diagnosed with GS and KDSS. Management with fluid resuscitation, intravenous immunoglobulin, corticosteroids, and aspirin stabilized his hemodynamic status and improved KD-related symptoms. With systemic inflammation controlled and cervical immobilization applied, GS subsequently resolved. At 6 months of follow-up, echocardiography showed no coronary artery abnormalities, and no recurrence of GS or neurological sequelae was observed. Conclusions Clinicians should recognize GS as a potential early manifestation of KD and maintain vigilance for progression to KDSS. Timely recognition and management could prevent severe cardiac and neurological complications in patients with KD complicated by GS and KDSS.

Background Patients with Kawasaki disease (KD) prone to develop coronary artery aneurysm (CAA) with unknown etiology. We aimed to disclose the relationship between vasa vasorum (VV) and intimal thickening using optical coherence tomography (OCT) in KD. Methods Forty-three coronary artery branches of 21 patients with KD were examined by OCT. The coronary arteries were classified into three groups: the CAA group ( n  = 9) in which CAAs remained since the acute phase, the regressed group ( n  = 16) in which CAAs were regressed, and the no CAA group ( n  = 18). The number and distribution of VV, and intimal thickening in coronary arteries were evaluated on OCT. Results Intimal thickening was significantly more severe in the CAA and regressed groups than in the no CAA group (median: 481, 474, and 218 μm, p  = 0.001 and p  < 0.001, respectively). The number of VV in the regressed group was significantly higher than that in the CAA and no CAA groups. The numbers of adventitial VV and internal VV were positively correlated with the intimal thickness ( R  = 0.64, p  < 0.001; R  = 0.62, p  < 0.001, respectively). In the no CAA group, no internal VV were observed. Conclusions VV enhances according to intimal thickening, suggesting that VV may have some link to the healing process, such as CAA regression and intimal thickening. Impact Kawasaki disease (KD) is a vasculitis syndrome developing coronary artery aneurysm, however its etiology still remains unclear. Coronary artery imaging using optical coherence tomography (OCT) can reveal coronary arterial wall pathology, however OCT studies are limited in patients with KD. Using OCT, we disclosed the closed relationship between vasa vasorum enhancement and regressed coronary arterial lesions. Vasa vasorum enhancement is involved in the pathomechanism of the convalescent phase of KD.

BackgroundKawasaki disease (KD) is a vasculitis with formation of coronary artery aneurysms (CAAs) that can lead to myocardial ischemia. Echocardiography is the primary imaging modality for the coronary arteries despite limited visualization. Coronary angiography (CAG) is the gold standard yet invasive with high-radiation exposure. To date however, state-of-the-art CT scanners enable high-quality low-dose coronary computed tomographic angiography (cCTA) imaging. The aim of our study in KD is to report (i) the diagnostic yield of cCTA compared to echocardiography, and (ii) the radiation dose.Methods and resultsWe collected data of KD patients who underwent cCTA. cCTA findings were compared with echocardiography results. In 70 KD patients (median age 15.1 years [0.5–59.5 years]; 78% male; 38% giant CAA), the cCTA identified 61 CAAs, of which 34 (56%, with a Z score > 3, in 22 patients) were not detected by echocardiography. In addition, the left circumflex (aneurysmatic in 6 patients) was always visible upon cCTA and not detected upon echocardiography. Calcifications, plaques, and/or thrombi were visualized by cCTA in 25 coronary arteries (15 patients). Calcifications were seen as early as 2.7 years after onset of disease. In 5 patients, the cCTA findings resulted in an immediate change of treatment. The median effective dose (ED) in millisievert differed significantly (p < 0.01) between third-generation dual-source and other CT scanners (1.5 [0.3–9.4] (n = 56) vs 3.8 [1.7–20.0] (n = 14)).ConclusionsThe diagnostic yield of third-generation dual-source cCTA combined with reduced radiation exposure makes cCTA a favorable diagnostic modality to complete the diagnosis and long-term treatment indications for KD.Key Points• cCTA is a favorable diagnostic modality to complete the diagnosis and long-term treatment indications for Kawasaki disease.• Kawasaki disease patients with proven coronary artery involvement on echocardiography require additional imaging.

Coronary artery (CA) stenosis and occlusion in convalescent Kawasaki disease (KD) is progressive and may result in myocardial infarction. The use of regadenoson, a strong selective CA vasodilator with low side effect profile, for stress cardiac magnetic resonance (CMR) imaging has not been studied in children with KD. The safety, feasibility, and diagnostic utility of regadenoson stress CMR was assessed in children with KD and CA abnormalities. A retrospective review of regadenoson stress CMR in children with convalescent KD was performed. Hemodynamics changes after regadenoson administration and adverse effects were recorded. First-pass perfusion was evaluated at rest and during pharmacologic stress. The results were compared with anatomic CA imaging. Forty-one stress CMR (18 sedated examinations, 44%) were performed successfully in 32 patients. Median age was 11.2 years (range 2.2 to 18.6) and weight 41 kg (range 13 to 93.4). Heart rate increased 66 ± 25% (p <0.005) after regadenoson. Minor adverse events occurred in 6 sedated and 1 unsedated patients. Hypoperfusion during stress occurred in 16 of 41 (39%), including 5 inducible, 9 inducible and fixed, and 2 fixed lesions. Late gadolinium enhancement was present in 10 of 16 with hypoperfusion and in 1 without hypoperfusion. Stress CMR had 100% positive agreement and >90% negative and overall agreement with moderate-to-severe CA stenoses. Four patients with hypoperfusion underwent revascularization for severe CA stenoses. In conclusion, regadenoson stress CMR is hemodynamically safe and feasible in children with KD and CA disease. It has excellent agreement with CA angiography and aided decision-making to proceed with revascularization.

Kawasaki Disease (KD) is a multisystemic vasculitis of medium- and small-sized arteries. Abnormal intimal thickening may develop in the involved arterial area after regression of coronary artery aneurysm (CAA). Intimal dysfunction may induce local stenosis or arteriosclerosis in the future. In this case–control study, we investigated 29 consecutive KD patients [20 male, median current age, 7.9 years; median follow-up duration, 5.7 years] and a group of 29 healthy matched controls (CON) [19 male, median current age, 10.8 years]. They were assesed and compared for CAA, LVFS, GCS, GLS, coronary artery (CA) Z scores, carotid intima-media thickness (IMT) and coronary artery IMT by high-resolution transthoracic echocardiography (hrTTE). Coronary artery IMT (caIMT) was significantly higher in patients with a maximal CA Z score > 2.5 in acute KD than in CON: KD caIMT: 0.62 mm [IQR, 0.57–0.72 mm] vs. 0.53 mm [0.51–0.60 mm], p  = 0.043. CAAs were found in 15 (51.7%) patients with acute KD. The maximal median LCA Z score in acute KD was 2.57z [IQR, 1.93—3.2z] and in follow-up −0.39z [IQR, −1.25 to −0.36z]. There was no significant difference in carotid IMT between KD patients and CON. Signs of CA intima-media thickening were detected by hrTTE in patients with a maximal CA Z score > 2.5 in acute KD. These data indicate that these patients may be at risk for cardiovascular sequale even in the absence of permanent CA luminal abnormalities. Therefore long-term follow-up of this group of KD patients may be required.

Background Kawasaki disease (KD) is a primary cause of acquired heart disease in children, often leading to significant complications like coronary artery lesions and vasculitis. Despite the administration of standard treatments, 10–20% of patients remain unresponsive, emphasizing the necessity for the development of improved diagnostic and treatment modalities. Given the limitations of conventional imaging techniques, this study investigates the potential of molecular imaging using targeted nanoparticles. Here, the role of osteopontin (OPN) in macrophage-dependent vascular inflammation and cardiovascular pathology is examined, as it may be crucial in the context of KD. Methods Gold nanoparticles (Au NPs) were synthesized through an amidation reaction to create OPN-Au NPs and mIgG-Au NPs. These were characterized using transmission electron microscopy and UV-vis spectra data, and their cytotoxicity was evaluated via cell viability and flow cytometry. The targeting efficacy of OPN-Au NPs was tested in vivo using CT imaging in a Kawasaki disease animal model. Furthermore, the role of BMDM-derived OPN in primary cardiac fibroblast activation was investigated through gene knockout and molecular mechanism analysis. The action mechanism of STAT3 on the epigenetic regulation of Col1 induced by OPN was studied using dual-luciferase reporter assays and ChIP-PCR. Results The Au NPs exhibited favorable optical properties and low cytotoxicity. In vivo CT imaging demonstrated the capacity of these NPs to enhance the imaging of coronary artery lesions. Furthermore, BMDM-derived OPN was demonstrated to regulate fibroblast function via STAT3 signaling. Dual-luciferase reporter assays and ChIP-PCR revealed that OPN-mediated STAT3 regulates the transcriptional expression of Col1 at the epigenetic level. Conclusion The findings indicate the potential of molecular imaging using Au NPs for the early detection and diagnosis of KD. Of note, the research demonstrates the role of OPN in modulating fibroblast activation through STAT3 signaling and its influence on the epigenetic regulation of Col1. This provides critical insights into the immune cell-fibroblast interaction in cardiovascular diseases, offering prospects for more targeted and effective diagnostic and therapeutic strategies for KD. Graphical Abstract

Background Kawasaki disease (KD) causes coronary artery lesions (CAL) and is the leading cause of acquired heart disease in children. The aim of this study is to evaluate the risk factors and set-up a scoring system for predicting CAL of KD. Methods We retrospectively reviewed a total of 478 patients diagnosed with KD. We compared age, gender, laboratory data, and treatment response in two groups and developed a scoring system for predicting CAL. Results During the study period, 365 of these patients had complete medical records of coronary surveys by echocardiography. Anemia, hypoalbuminemia, C reactive protein (CRP), alanine aminotransferase, neutrophil count, and neutrophil/lymphocyte ratio (NLR) showed significant differences with CAL formation. We determined the cut-off value using a receiver-operating-characteristic (ROC) curve, and following multivariate logistic regression analysis, four independent risk factors demonstrated a significant difference with CAL formation, including CRP > 103 mg/L, NLR > 3.5, male gender, and intravenous immunoglobulin (IVIG) resistance. We established a score system based on the above evaluation, for which a ROC curve was performed, and a total score of ≥ 2 points showed a sensitivity of 60.8% and a specificity of 70.6%, with an area under the ROC curve of 0.696. Conclusions Identifying children at risk is important in order to prevent CAL from developing. Four independent risk factors that can predict CAL formation were CRP > 103 mg/L, NLR > 3.5, male gender, and IVIG resistance. This first report incorporated NLR into score systems to predict CAL reinforces previously well-known risk factors for the CAL formation among KD patients.

PurposeCoronary artery involvement remains the primary focus in the long-term management of Kawasaki disease (KD). However, previous studies suggest that myocardial abnormalities frequently persist beyond coronary artery involvement in KD patients. Yet, their temporal evolution and clinical implications remain poorly characterised. To address this gap, we established the Kawasaki disease cardiac imaging (KDCI) cohort, integrating cardiac magnetic resonance (CMR) with echocardiography, coronary CT angiography (CCTA) and invasive angiography. These multimodal imaging approaches enable comprehensive assessment of cardiac abnormalities and elucidate the role of cardiac imaging in optimising long-term KD management.ParticipantsThe KDCI cohort is a prospective study aiming to enrol 400–500 KD patients diagnosed at West China Second University Hospital from September 2018 to September 2035. To date, 207 participants have been recruited. Participants will perform the multimodal cardiac imaging including echocardiography, CMR, CCTA, invasive angiography and comprehensive laboratory testing under a scheduled protocol in the follow-up.Findings to dateThe KDCI cohort has established baseline characteristics for 207 KD patients. Of those included to date, 72.0% (149/207) received intravenous immunoglobulin (IVIG) treatment, with 26.1% (54/207) demonstrating IVIG resistance, and 37.7% (78/207) exhibiting coronary artery dilatation. Longitudinal follow-up data are available for 80.7% (167/207) of participants, with a median follow-up duration of 2.7 years and a follow-up patient-years of 594 patient-years. Of the 207 patients, 16.9% (35/207) patients experienced endpoint events, encompassing coronary artery thrombosis (8.2%, 17/207), coronary stenosis/obstruction (5.3% 11/207) and clinical myocardial infarction (1.9%, 4/207). Based on the data collected, we have demonstrated the cardiac abnormalities beyond coronary artery involvement in KD by CMR and CCTA.Future planThe KDCI cohort will maintain ongoing recruitment and longitudinal follow-up, with a projected enrolment exceeding 400 participants by 2035. This expansion will yield a median follow-up duration of 10 years, providing robust long-term outcome data. We have implemented standardised protocols for scheduled follow-up assessments and data collection in newly enrolled patients. Furthermore, planned genomic analyses will be incorporated to investigate the molecular pathogenesis and prognostic determinants of KD.

Introduction Given the evidence that brachial artery flow-mediated dilation (FMD) is declined in children later after the onset of Kawasaki disease (KD), we hypothesized that indicators that detect the situation of the endothelium are useful parameters that can accurately reflect subclinical dysfunction in resistant patients and assist in differentiating patients with KD at a higher risk of IVIG resistance, which may be valuable in better understanding how to protect patients from endothelial and thrombotic complications. Methods Fifty IVIG-resistant KD children, 120 IVIG-responsive KD children, 35 febrile children with acute upper respiratory infection, and 50 healthy controls were recruited, and indicators reflecting endothelial inflammation, including flow-mediated dilation (FMD), were measured. Receiver operating characteristic (ROC) curve analysis was utilized to determine the threshold values of these indicators of IVIG resistance. Multiple logistic regression analysis was performed to determine whether FMD was an independent predictor of IVIG-resistant patients. Results In comparison with the lab data, PCT, Na + , and FMD exhibited AUCs of 0.727, 0.653, and 0.698 ( P  < 0.05), respectively, in predicting IVIG resistance in KD through ROC analysis. PCT > 1.69 ng/ml, Na +  < 133.2 mmol/l, and FMD < 5.79% were independent predictors of IVIG resistance in KD (OR 4.257, 3.516, 3.563, 95% CI 1.549 ~ 11.700, 1.277 ~ 9.680, 1.299 ~ 9.772, P  < 0.05). Conclusion More severe endothelial dysfunction, especially lower FMD, was present in IVIG-resistant patients than in IVIG-responsive patients. It is a helpful diagnostic tool that provides supportive criteria to detect KD patients at a higher risk of IVIG resistance when FMD < 5.79% in children. Key Points • IVIG-resistant KD patients have more severe endothelial dysfunction than IVIG-sensitive patients. • FMD  <  5.79% may indicate an increased risk of IVIG resistance in children with Kawasaki disease .