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BACKGROUND: American Heart Association (AHA) guidelines for management of Kawasaki disease (KD) rely on coronary artery (CA) z-scores from echocardiograms. Compared with echocardiography, cardiac CT (CCT) offers better visualization of distal segments and evaluation for thrombosis and stenosis. Despite increasing use of CCT in KD, CA z-scores for CCT are not available and measurement concordance between imaging modalities is a critical knowledge gap. METHODS: We retrospectively reviewed KD patients with CA aneurysms who had concurrent echocardiography and CCT between 2016-2020. Patients were included if they had history of CA z-scores of ≥ 3 on echocardiography during their clinical course. Agreement between CCT and echocardiography was assessed using Bland-Altman analysis. RESULTS: Paired CCT and echocardiography studies were available in 18 patients (21 studies). The largest CA aneurysms were large/giant (z-score ≥10) in 14 studies, medium (z-score ≥5

Graphical abstract, Highlights • A unique case of S. aureus coronary stent infection occurred 5 years after index PCI. • Prompt diagnosis was made with coronary angiography and echocardiography. • Successful treatment included prolonged antibiotics and surgical intervention. • Delayed coronary stent endothelialization might have contributed to late infection.

Background Although not common, coronary artery aneurysms (CAAs) can develop to over 8 mm in diameter to become giant CAAs. In the context of systemic lupus erythematosus (SLE), autoantibody- and immune complex-mediated atherosclerosis is believed to be the most prevalent cause of aneurysm. Case presentation We report the case of a 53-year-old female SLE patient who presented to our hospital with radiating chest pain. Coronary angiography revealed a giant aneurysm in the middle segment of the left anterior descending artery (LAD) and distal subtotal occlusion in the left circumflex artery (LCX). Laboratory testing also identified risk factors such as an abnormal pulmonary enzyme profile, dyslipidemia, and nephritis parameters.To prevent thromboembolism, anticoagulation and antiplatelet therapy were administered. In addition, one stent was implanted at the distal end of the LCX and repeated coronary angiography verified restoration of TIMI grade III flow.The patient was discharged with resolved chest pain. During 6 months of follow-up, the patient is in good health. Conclusions Our case study, together with 16 recent comparable reports, emphasizes the need for coronary aneurysm screening in SLE patients. It is necessary that thromboembolism, anticoagulation and antiplatelet therapy were administered for CAA.

Introduction Kawasaki disease (KD) with coronary artery aneurysms (complex KD) presents relentless challenges for families. Psychosocial experiences and needs were explored. Method A descriptive, exploratory study of adolescents and parents using a needs survey and psychosocial instruments (anxiety, depression, and functional impairment). Results Fifty-one parents and 38 adolescents participated. Predominant interests were for information sheets, newsletters, Web sites, and phone applications. Gaps in disease-specific knowledge and awareness of coronary artery aneurysms between parents and adolescents were identified. Psychosocial concerns for adolescents included symptoms of anxiety (22%), depression (13%), and functional impairment (22%). Multivariable analyses indicated higher depression scores associated with longer travel distance from specialists (p = .04). Parent-reported social concerns for their adolescent were associated with higher anxiety (p = .005) and functional impairment (p = .005). Written commentary complemented the findings. Discussion Care protocols require psychosocial assessment/referral and the use of virtual platforms. The groundwork was laid for developing patient and family-centered strategies.

Percutaneous transluminal rotational atherectomy (PTRA) is one of the most used techniques to facilitate percutaneous coronary intervention in heavily calcified coronary lesion (CCL). Coronary aneurysms (CAs) are detected in 1.2–4.9% of coronary angiogram. The presence of CA and CCL is infrequent but not rare, where the use of PTRA may be mandatory despite the high risk. After a complex procedure of PTRA in a CCL with CA we decided to investigate about this particular condition. We identified a total of six patients among 174 consecutive percutaneous coronary intervention (3.4%). All the procedures showed good stent expansion in the absence of major complications, such as no-reflow or coronary perforation. Cardiovascular death, rehospitalization for myocardial infarction and target lesion failure were not reported at follow-up (252 ± 152 days).

Sotos syndrome, characterized by cerebral gigantism with neurologic disorders, is an overgrowth syndrome caused by mutations of the NSD1 gene, with an estimated prevalence of 1:10,000-1:50,000. We herein describe the first case of Sotos syndrome complicated by acute coronary syndrome, for which emergency coronary artery bypass grafting was performed. (Level of Difficulty: Intermediate.), Central Illustration

Background:Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation and management was assessed in a large real-world dataset.Methods:Using a combined dataset of patients with acute Kawasaki disease from the Children’s Hospital at Montefiore and the National Heart, Lung, and Blood Institute Kawasaki Disease Study, coronary Z scores and the rate of coronary lesions (Z ≥ 2.0) and aneurysms (Z ≥ 2.5) were determined using four Z score systems. Agreement among Z scores and the effect on Kawasaki management were assessed.Results:Of 333 patients analysed, 136 were from Montefiore and 197 from the Kawasaki Disease Study. Age, sex, body surface area, and rate of coronary lesions did not differ between the samples. Among the four Z score systems, the rate of acute coronary lesions varied from 24 to 55%. The mean left anterior descending Z scores from Pediatric Heart Network and Boston had a large uniform discrepancy of 1.3. Differences in Z scores among the four systems may change anticoagulation management in up to 22% of a Kawasaki population.Conclusions:Choice of Z score system alone may impact Kawasaki disease diagnosis and management. Further research is necessary to determine the ideal coronary Z score system.

Background Aneurysmal degeneration of the coronary button after aortic root replacement using the button technique is a rare but potentially life-threatening complication. However, the appropriate management of this complication, including the indications for conservative and surgical treatment, is still unknown. Case presentation Here we present a 38-year-old woman who successfully underwent surgical repair of a left coronary button aneurysm using the graft interposition technique 24 years after aortic root replacement. Because follow-up computed tomography after aortic root replacement showed a progressively enlarging left coronary button aneurysm, the patient was judged an acceptable candidate for surgical treatment, considering the potential risk of aneurysmal rupture and subsequent myocardial infarction. The postoperative recovery was uneventful. The patient is doing well 1 year after the surgery. Conclusions We believe that serial follow-up using computed tomography is mandatory for coronary button aneurysms, and surgical intervention may be considered if progressive enlargement of the aneurysm is observed, especially in younger patients.

We clarified the changes in coronary artery diameters based on the degree of coronary artery involvement immediately after acute Kawasaki disease (KD). Two hundred sixteen coronary arteries in 85 patients after KD examined by two-dimensional echocardiography were reviewed from 1995. The maximal internal diameters were measured at 2 months, 1 year, 3 years, 10 years and 15 years after KD. The coronary arteries were divided into five groups based on the absolute diameter at 2 months, as well as six groups based on the Z score at 2 months. The maximum diameters were compared at 2 months with those during follow-up in each group. The numbers of right coronary, left anterior descending, left coronary, and left circumflex arteries were 84, 73, 55 and 4, respectively. There was a significant relationship between the maximum internal diameter at 2 months and subsequent changes in the maximum diameters after KD in the late period in both groups (p

Background We aimed to assess the efficacy of different initial intravenous immunoglobulin (IVIG) regimens in Kawasaki disease (KD) patients to find more cost‐effective therapy options. Methods A multicenter, open‐label, blind‐endpoint randomized controlled trial was conducted from January 2014 to December 2015. Patients with KD, within 10 days of illness, were randomly assigned to receive different IVIG regimens (Group A, 2 g/kg once; Group B, 1 g/kg for 2 consecutive days; Group C, 1 g/kg once) and aspirin 30mg/kg/d. Primary outcomes included hours to defervescence and development of coronary artery lesions during the study period. Major secondary outcomes included total fever days, total dose of IVIG, changes of laboratory data, length of stay, and hospitalization expenses. (ClinicalTrials.gov: NCT02439996). Results A total of 404 patients underwent randomization. No difference was found in the outcomes of defervescence among three groups at 6, 12, 24, and 36 hours after completion of initial IVIG infusion. There were no differences in the incidence of coronary artery lesions during the study period (at week 2, month 1, month 3, and month 6 of illness), changes of laboratory data, total fever days, and length of stay. Group C patients had the lowest total dose of IVIG (mean: 1.2 vs 2.2 vs 2.1 g/kg; P < 0.001) and hospitalization expenses (mean: 8443.8 vs 10798.4 vs 11011.4 Chinese Yuan; P < 0.001) than other two groups. Conclusions A single dose of 1g/kg IVIG is a low‐cost treatment with the same efficacy as 2 g/kg IVIG and can be an option for the initial therapy of KD patients.

A 62-year-old man was diagnosed with a giant coronary artery aneurysm associated with immunoglobulin G4 (IgG4)-related disease. He had previously undergone two thoracic operations with sternotomies and abdominal aortic aneurysm repair for IgG4-related aortopathy. We opted for hybrid open and endovascular repair to reduce risk and avoid complications of a resternotomy and extracorporeal circulation. This first successful case of hybrid repair of a giant coronary artery aneurysm shows that the procedure is safe and feasible in patients with IgG4-related vasculopathy. It is critical to carefully monitor these patients for the occurrence of new IgG4-related aneurysms and other manifestations of vasculopathy.

A 61-year-old man presented with pneumonia and myocardial infarction on a background of diabetes and end-stage renal failure. Methicillin-resistant Staphylococcus aureus bacteremia was confirmed in the setting of ongoing pyrexia. The patient was referred for a 67Ga study, which revealed Ga-avid active disease in the pericardial effusion. This was localized further on CT/MRI and coronary angiography to a dissecting right coronary artery aneurysm, which was treated with surgical repair and long-term antibiotic treatment.

Infected pseudo coronary artery aneurysm (CAA) is extremely rare, and currently, there is no established treatment. We experienced a rare case of an infected pseudo CAA brought on due to a stent fracture. Following prolonged successful antimicrobial administration, which proved effective in successfully treating the patient, we performed coronary stent graft placement. Although a surgical procedure should fundamentally be the first course of action considered in such cases, when there are concerns as to the degree of invasiveness, our strategy represents a viable option.

BackgroundKawasaki disease (KD) as an acute, systemic vasculitis is the leading cause of acquired heart disease in children under the age of 5 years.MethodsA 10-year cross-sectional retrospective study was designed to assess 190 Iranian children with KD during 2008–2018. Demographic data, clinical and laboratory manifestations from the onset of symptoms to diagnosis, clinical signs and symptoms, and subsequent treatments were evaluated to predict hospitalization stay, complications, and response to treatment.ResultsChildren with KD had a male-to-female ratio of 1.18:1 and an average age of 36 months. There was an insignificantly more incidence of KD in cold seasons. The most frequent symptoms were fever (92.6%), oral mucus membrane changes (75.8%), bilateral bulbar conjunctival injection (73.7%), polymorphous skin rash (73.2%), peripheral extremity changes (63.7%), and cervical lymphadenopathy (60.0%). The rate of gastrointestinal, cardiac, joint, and hepatic complications was determined to be 38.4, 27.9, 6.8, and 4.2%, respectively. 89.5% of patients received intravenous immunoglobulin (IVIG) plus aspirin as the first line of treatment, while, 16.3% of them needed an extra second line of treatment. Significantly low serum sodium levels and high platelet counts were detected in KD patients with cardiac complications. Cardiac complications often were more encountered in patients who did not respond to the first line of treatment. Higher platelet count, lower serum sodium amount, and C-reactive protein (CRP) level were significantly associated with a need for an additive second line of treatment. A significant relationship between hospitalization stay and hemoglobin level was found.ConclusionAs most of the clinical manifestations and complications were following other reports released over the past few years, such data can be confidently used to diagnose KD in Iran. Seasonal incidence and a positive history of recent infection in a notable number of patients may provide clues to understand possible etiologies of KD. Laboratory markers can successfully contribute to health practitioners with the clinical judgment of the need for additional treatments, possible complications, and hospitalization duration.

Coronary artery aneurysm (CAA) is an uncommon coronary disease, with a reported incidence in adults ranging from 0.33 to 4.9%.It is usually considered a variant of coronary artery disease (CAD). CAA is associated with thrombus formation due to abnormal laminar flow, as well as abnormal platelet and endothelial-derived pathophysiologic factors within the CAA. CAA identified in the context of acute coronary syndrome (ACS) poses several unique management challenges. Optimal antiplatelet and anticoagulant therapy is the mainstay of therapy. Percutaneous intervention for CAA is associated with complications including distal embolization of thrombus, no-reflow phenomenon, stent malposition, dissection, and rupture. There are currently no accepted guidelines to direct the management of CAA in patients presenting with ACS. Preference for conservative vs. surgical or catheter-based management is controversial. We review the literature and report different treatment strategies for two cases with both CAA and ACS.

Purpose A coronary artery aneurysm (CAA) can result in critical cardiac events such as thromboembolic complications or rupture. A saccular CAA located in the left main trunk (LMT) is the most critical form of this pathology and its surgical repair is challenging. We conducted this single-center study to review the surgical outcomes of patients with a saccular CAA in the LMT. Methods Between May, 2012 and June, 2020, five patients with a saccular CAA in the LMT underwent surgery at our center. The median age at operation was 66.5 (59.7–69) years and the median diameter of the CAA was 13.0 mm (IQR 11–14 mm). Results The CAA was fully excluded by patch closure of the LMT orifice and direct closure of the distal LMT, supplemented by coronary artery bypass grafting with the exclusive use of arterial conduits. There was no in-hospital mortality, although one patient suffered graft spasm-related myocardial infarction with complete recovery. Post-operative angiography showed a fully excluded LMT in all patients. There was no mortality or adverse cardiac events during follow-up. Conclusions Our surgical policy for CAA in the LMT is feasible and safe; however, coronary blood flow is dependent on reliable bypasses.

A 21-year-old man who had an initial misdiagnosis of chest wall cellulitis and sepsis presented to the emergency department with chest pain. Electrocardiogram demonstrated ST-segment elevation in the inferior leads. Cardiac catheterization identified diffuse aneurysmal dilation and thrombosis of the distal right coronary artery. Clinical signs were consistent with acute Kawasaki disease. (Level of Difficulty: Intermediate.), Graphical abstract

Multisystem inflammatory syndrome in children (MIS-C) or paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is an emerging disease in children affected with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and thought to be an immune-mediated post-infectious complication of SARS-CoV-2. The disease presentation is similar to Kawasaki disease but has certain distinguishing features. The exact pathogenesis is still not clear but an aberrant immune response, antibody-mediated vascular damage and virus-mediated abnormal type I and III interferon-gamma response are thought to be responsible. Most children who are previously healthy present after 2-4 weeks of SARS-CoV-2 infections with febrile illness of short duration with prominent gastrointestinal, cardiac and hematologic manifestations, progressing to vasoplegic shock, requiring vasopressor therapy. Cardiovascular involvement is prominently marked by acute myocardial injury/myocarditis and the development of coronary artery aneurysms. Laboratory markers of inflammation are elevated uniformly. Most children require intensive care, and few need invasive ventilation. The treatment mainly consists of anti-inflammatory and immunomodulatory therapy like intravenous immunoglobulins and steroids. The overall prognosis is good and reported mortality rates are 0-4%.

Background Coronary artery ectasia (CAE) is a rare anomaly that can present at any age. Predisposing risk factors include Kawasaki disease in a younger population and atherosclerosis in the older generation. We present a unique case of the management of a young woman diagnosed with multivessel CAE with aneurysmal changes in the setting of acute coronary syndrome and subsequently during pregnancy. Case summary A 23-year-old woman presented with acute onset chest pain. Electrocardiogram revealed no ischaemic changes; however, troponin I peaked at 16 ng/mL (reference range 0–0.04 ng/mL). Echocardiogram showed apical dyskinesis with preserved left ventricular ejection fraction. Coronary angiography showed multivessel CAE along with significant thrombus burden in an ectatic lesion of the left anterior descending artery. Since the patient was haemodynamically stable, conservative management with dual antiplatelet therapy and anticoagulation was started. On follow-up, coronary computed tomographic angiogram illustrated resolution of the coronary thrombi and echocardiogram showed improvement to the apical dyskinesis. It was presumed that Kawasaki disease was the most likely aetiology of her disease. Subsequently the patient reported that, contrary to medical advice, she was pregnant, adding another layer of complexity to her case. Discussion Coronary artery ectasia can be discovered as an incidental finding or can present with an acute coronary syndrome. Management is challenging in the absence of randomized trials and large-scale data. Treatment options include medications, percutaneous intervention, and surgical revascularization. Close surveillance is required in these patients to assess progression of disease. Here we discuss treatment options during acute coronary syndrome and pregnancy.

Objective Kawasaki disease (KD) is a vasculitis of unknown aetiology with a high risk of coronary aneurysms if untreated. Timely treatment with intravenous immunoglobulin decreases the risk for coronary artery aneurysms (CAA). In this study, we set out to elucidate the factors associated with the risk of developing CAA. Methods Records of all KD-diagnosed children in Skåne between 2004 and 2014 were collected and clinical and demographic data were compiled. KD is defined according to the revised American Heart Association diagnostic criteria and classified as either complete KD (cKD) or incomplete KD (iKD). Results KD was diagnosed in 77 children and CAA was found in 31% (n = 24). Children with CAA were younger compared with children without (median; 20 vs 34 months) and intravenous immunoglobulin treatment within 10 days was less likely to be received (75% vs 91%). In children presenting with iKD, 47% developed CAA compared with 21% in cKD patients. Using multivariate analysis, an association between the risk of CAA with low age in children with iKD was observed. Conclusion The risk of CAA development is disturbingly high in young children with iKD. This highlights the importance of rapid intense treatment and vigilance in infants, who are the most difficult to diagnose, in order to reduce the frequency of CAA.

Background A small subset of pediatric patients develop a rare syndrome associated with Coronavirus Disease 2019 (COVID-19) infection called multisystem inflammatory syndrome in children (MIS-C). This syndrome shares characteristics with Kawasaki disease. Case Report A 15-year-old girl presented to our Emergency Department (ED) with fevers and malaise. She was diagnosed on her initial visit with an acute viral syndrome and discharged with a COVID polymerase chain reaction test pending, which was subsequently negative. She returned 3 days later with persistent fever, conjunctivitis, and a symmetric targetoid rash over her palms. She had no adenopathy, but her erythrocyte sedimentation rate and C-reactive protein were both significantly elevated at 90 mm/h and 19.61 mg/dL, respectively. The patient was then transferred to the regional children's hospital due to a clinical suspicion for MIS-C, and subsequent COVID-19 immunoglobulin G testing was positive. She had been empirically started on intravenous immunoglobulin in addition to 81 mg aspirin daily. Initial echocardiograms showed mild dilatation of the left main coronary artery, and on repeat echocardiogram, a right coronary artery aneurysm was also identified. Oral prednisone therapy (5 mg) was initiated and the patient was discharged on a continued prednisone taper. Why Should an Emergency Physician Be Aware of This? We present a case of a 15-year-old girl who presented to the ED with MIS-C who developed coronary aneurysms despite early therapy, to increase awareness among emergency physicians of this emerging condition.

Background Infected coronary artery aneurysms (ICAA) represent a rare but potentially fatal complication of pre-existent atherosclerotic or non-atherosclerotic coronary artery disease, percutaneous coronary artery intervention, endocarditis or extracardiac infection. Methods A retrospective analysis of four cases in addition to 51 infected coronary artery aneurysms from the literature, for a total of 55 ICAA was performed. Clinical and morphological information including age, sex, clinical presentation, microbial cultures, size, location and associated abnormalities as well as patient outcome was reviewed. Results 83% of affected patients were adult males, with an average age of 55.24 years. The right coronary artery was the most commonly affected vessel (40%). In nearly 80% of the time, the responsible organism was either Staphylococcus aureus (53.3%), or Streptococcus (20%) infection. ICAA are typically large, on average 3.4 cm in diameter and can measure up to 9 cm. On contrast enhanced CT, imaging features include lobulated contour or saccular shape (54.2%) with thick wall or mural thrombus (87.5%). Associated abnormal appearance of the pericardium with either pericardial fluid, thickening or loculation is common (79.2%). Conclusion ICAA are typically large, and characterized by a thick wall with a lobulated or saccular shape. Association with mediastinal, chest wall or pericardial abnormalities are common. This combination of findings, in the setting of fever, known infection, or recent coronary intervention should raise concern for ICAA.

Coronary artery fistula (CAF) is a rare congenital anomaly with a reported incidence of 0.2%-0.6%. There is a wide variation in the clinical presentation depending on the size and the site of the fistula. Transcatheter closure is currently the treatment of choice in these patients. We report a case of CAF with an unusually large fistulous sac within the interventricular septum. The fistula had connections with all the three major coronary arteries, namely, left anterior descending (LAD), left circumflex, and right coronary arteries but did not have any exit resulting in to and fro movement of blood within the sac and the feeding vessels. The patient was managed successfully by transcatheter coil embolization.

Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications. Early diagnosis and treatment is mandatory in incomplete KD to reduce the risk of coronary involvement. Between 2002 and 2018, 124 patients have been diagnosed suffering from KD at the University Children’s Hospital Regensburg (KUNO). We assessed luminal diameters of both CAs normalized as Z-scores by 2D-echocardiography. A total of 94 patients were analyzed. Of them, 31 (33%) were affected by an incomplete form of KD. In 24 children (26%), serial echocardiography was necessary in order to confirm diagnosis. Mean Z-scores for the left main coronary artery (LMCA), right main coronary artery (RMCA), and left anterior descending artery increased significantly between the initial (LMCA 0.79z, RMCA 0.15z, LAD 0.49z) and second (LMCA 1.69z, RMCA 0.99z, LAD 1.69z) examination (p Conclusion:To confirm diagnosis of KD, it might not be necessary to detect dilation or aneurysms. Our observation suggests that patients suspected having KD should be monitored with serial echocardiography in order to detect a possible enlargement of the CA diameters, even if Z-scores are within the normal range. What is Known:• Kawasaki disease (KD) is an acute vasculitis predominantly affecting the small arteries of young children. Up to 25% of untreated patients suffer from coronary artery (CA) complications.• Due to less classic clinical criteria in patients with incomplete KD, the risk for CA pathology is even higher. What is New:• A significant progression of patients’ CA Z-scores in serial echocardiographic measurements may be helpful to ensure diagnosis of KD early even if Z-scores are within the normal range.• Twenty-seven patients (90%) with incomplete KD could be diagnosed within 10 days of fever, early enough to prevent significantly higher rates of CA aneurysm.

ObjectivePatients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outcomes in patients with CAA at diagnosis.DesignSingle-centre, retrospective review.PatientsPatients with KD diagnosed within 10 days of fever onset and with baseline CA z-score ≥2.5.InterventionsPrimary treatment with IVIG (n=162) versus IVIG plus corticosteroids (n=48).Main outcome measuresTreatment resistance (persistent fever >36 hours after initial treatment), CAA regression rate.ResultsOf the 92 patients with KD who received corticosteroids at our institution from 2012 to 2019, 48 met the inclusion criteria for primary adjunctive therapy. The corticosteroid group was younger and had larger baseline CAAs compared with historical controls. Demographics and laboratory values were otherwise similar between groups. The corticosteroid group had a less treatment resistance (4% vs 30%, p=0.003) and a greater improvement in C reactive protein. After adjusting for baseline CA z-score, age and baseline bilateral versus unilateral CAA, the corticosteroid group had a higher odds of (OR 2.77 (1.04, 7.42), p=0.042) and a shorter time to CAA regression (HR 1.94 (1.27, 2.96), p=0.002).ConclusionPrimary adjunctive corticosteroid therapy is associated with decreased initial treatment resistance, greater improvement in inflammatory markers and higher likelihood of CAA regression in patients who have CAA at diagnosis. Multi-centre, randomised controlled trials are needed to confirm the benefits of corticosteroids in patients with CAA at diagnosis and to compare corticosteroids with other adjunctive therapies.

Objectives: Kawasaki disease (KD) is the most frequent cause of acquired heart disease in children in high-income countries because of coronary artery involvement. Risk factors for coronary lesions can vary in consideration of different genetic background and environmental factors. Methods: Multicenter retrospective and prospective study including 372 consecutive children (58% boys; mean age 34.3 ± 30.3 months, Caucasian 85%) was diagnosed with KD. We divided the cohort into 2 groups according to the presence of coronary anomalies (CAA) and aneurysms. We compared the groups and studied the risk factors for CAA and for aneurysms, the most severe lesions. Results: Children with CAA were 91/372 (24.46%, aneurysms 20/372, 5.37%). Children with CAA were more likely to have a longer duration of fever (p < 0.001), later day of treatment (p < 0.001), to be IVIG non-responders and late treated (p < 0.001), while age, clinical presentation, and seasonality were not different. They also had significantly higher WBC and neutrophils, lower lymphocytes, Hb and Na during the acute stage, and slower resolution of inflammation. Age, IVIG unresponsiveness, and presence of non-coronary cardiac findings were independent risk factors for CAA and for aneurysms, while neutrophils just for CAA. Age under 6 months was a risk factor for the aneurysm. Aneurysms occurred more frequently in the first quartile of the age of KD onset (under 14 months). Conclusion: Very young children with non-coronary cardiac findings are at increased risk for a more severe form of KD with aneurysms. These children could benefit from adjunctive therapy beside IVIG, especially if they have higher markers of inflammation, particularly neutrophils.Key points• Risk factors for coronary lesions can vary in consideration of different genetic background and environmental factors.• Risk factors for coronary involvement have been extensively studied in the Asian population, and others have been validated in cohorts with mixed ethnicities.• In our predominantly Caucasian population, non-coronary cardiac findings, age younger than 6 months, and IVIG unresponsiveness are independent risk factors for a more severe form of KD with aneurysms.

Kawasaki disease (KD) is a systemic vasculitis targeting medium-sized and small arteries. It manifests in young children. If not appropriately treated, KD leads to the development of coronary artery aneurysms inabout 20% of patients. No local evidence-based clinical and epidemiological data on KD are currently available in Kazakhstan. The awareness of KD among physicians is inadequate, resulting in underdiagnosis of the disease in the country. Given the high priority of cardiovascular morbidity and mortality in Central Asian countries, clinical and epidemiological studies on KD along with measures for early diagnosis and management of the patients with cardiovascular affections are warranted.

Kawasaki disease (KD) patients' resistance to treatment with intravenous immunoglobulins (IVIG) places them at high risk for an unfavorable progression of the disease. In these patients, there has been little evidence that alternative treatments are effective. Nevertheless, biologicals such as an interleukin-1-receptor blocker and tumor-necrosis-factor-α inhibitors increasingly have been used. If the patient does not respond to one of these therapeutics, a combination of 2 biologicals might be an alternative, but this is not yet generally accepted due to the potentially increased risk of infection. Here we report on a 3-month-old boy suffering from severe refractory KD. KD diagnosis was delayed due to the misinterpretation of a urinary tract infection and to the short and nonsimultaneous presence of classical KD symptoms. After complete KD later was able to be diagnosed, treatment with intravenous immunoglobulins was administered. However, the disease proved resistant to 2 courses of IVIG, as well as to corticosteroids. The patient developed giant coronary artery aneurysms early during the course of disease. Anakinra was initiated, but even with stepwise higher anakinra dosages, he remained febrile and coronary artery dimensions increased. Therefore, etanercept was added as a second biological. Only under combination treatment with anakinra and etanercept were his inflammation and fever able to be completely resolved. Coronary artery dimensions improved over time.

Background Previous studies demonstrated that coronary artery lesions (CALs) resulting from Kawasaki disease (KD) can improve over time. However, limited information is available on sub-acute outcomes of CALs detected at admission during KD illness. Methods The nationwide Japanese KD survey contained substantial information on KD patients with CALs detected at admission and who received standard IVIG treatment within 10 days of disease onset. Coronary outcomes were evaluated by changes in CALs from admission to the first assessment at 30 days from disease onset in three categories: improved, unchanged, and progressed. Ordinal logistic regression analysis was performed to evaluate factors associated with the outcomes. Results Of 2024 patients with CALs detected at admission, improved, unchanged, and progressed outcomes were found in 1548 (76.5%), 390 (19.3%), and 86 (4.2%), respectively. Over 80% of patients with coronary artery (CA) dilatations had improved outcome. Independent factors associated with worse outcomes were larger-size CALs (adjusted ORs [95% CIs]: CA aneurysm = 5.13 [3.65-7.22] and giant CA aneurysms = 7.49 [3.56-15.72] compared with CA dilatation, respectively), age ≥ 60 months (1.45 [1.08-1.94] compared with 12-59 months), recurrent KD (1.57 [1.07-2.29]), parental history of KD (2.23 [1.02-4.85]), and delayed admission (8-10 days from disease onset: 1.76 [1.21-2.57] compared with 1-4 days). Conclusions KD patients with larger CALs, ≥60 months old, and with recurrent status or parental history may require more rigorous treatment. In addition, delayed admission may result in worse coronary outcome, indicating that prompt diagnosis and treatment are required.

The left ventricular (LV) systolic dyssynchrony index (SDI) is an important prognostic indicator for many cardiovascular diseases; however, the characteristics of the SDI in patients with Kawasaki disease (KD) are unknown. In this study, we aimed to identify and quantify the SDI using real-time three-dimensional echocardiography (RT3DE) in KD patients during different phases. In addition, we intended to explore whether the SDI is associated with systolic dysfunction. Seventy consecutive KD patients and seventy age- and sex-matched controls were enrolled. The SDIs (percent of cardiac cycle) of 16 segments (16-SDI%) and 12 segments (12-SDI%) were calculated based on the defined standard deviation of each segment time from end diastole to the minimal systolic volume according to the 17-segment model (apex excluded). In the acute phase, the 16-SDI% and 12-SDI% were significantly higher in KD patients than in controls (4.40 ± 0.14 vs. 1.98 ± 0.12, P = 0.000; 3.55 ± 1.21 vs. 1.67 ± 0.93, P = 0.009, respectively), and patients with coronary artery aneurysm (CAA) exhibited higher 16-SDI% (P = 0.021) and 12-SDI% (P = 0.034) than patients without CAA. In the convalescent phase, patients with CAA still had higher 16-SDI% (P = 0.002) and 12-SDI% (P = 0.031) than controls, while the SDI in patients without CAA recovered to normal. The 16-SDI% was negatively correlated with the LV ejection fraction obtained from RT3DE (r = - 0.845, P = 0.000). Mechanical dyssynchrony is prevalent in KD patients during the acute phase and transient in patients without CAA, while patients with CAA still have impaired synchrony even in the convalescent phase. LV systolic dysfunction is associated with increased dyssynchrony. RT3DE is a valuable modality for identifying and quantifying dyssynchrony in KD patients.

BackgroundKawasaki disease (KD) is the leading cause of acquired heart disease in children, and is steadily increasing in prevalence in East Asia. KD is often complicated by coronary artery damage, including dilatation and/or aneurysms. Aspirin is used with intravenous immunoglobulin (IVIG) to prevent coronary artery abnormalities in KD. However, the role and optimal dose of aspirin remain controversial. Identifying the dose of aspirin in the acute phase will facilitate development of a more appropriate treatment strategy in improving the outcome of KD.MethodsA total of 2369 patients with KD were retrospectively analyzed and divided into three groups according to the aspirin dose: 510 in group 1 (20–29 mg/kg/day), 1487 in group 2 (30–39 mg/kg/day), and 372 in group 3 (40–50 mg/kg/day). The differences in laboratory data, rate of IVIG resistance and coronary artery damage were compared among the groups.ResultsThere was no difference in the incidence of coronary artery aneurysms (CAAs) in group 1 compared with groups 2 and 3 (2 weeks of illness: 2.94% vs. 1.90% vs. 3.36%; 3–4 weeks of illness: 1.94% vs. 2.32% vs. 2.65%). The risk for developing CAA was not reduced at 2 weeks of illness onset in groups 2 and 3 compared with group 1 (adjusted OR = 1.05, 95% confidence interval: 0.34–3.18; aOR = 1.81, 95% CI: 0.42–7.83). Furthermore, the risk for developing CAA was not reduced at 3–4 weeks of illness onset in groups 2 and 3 (aOR = 2.63, 95% CI: 0.61–11.28; aOR = 0.52, 95% CI: 0.03–9.54). There was no significant difference in the rate of IVIG resistance among the groups. Platelet levels after IVIG treatment in group 1 were significantly lower than those in groups 2 and 3 (522.29 × 109/L, 544.69 × 109/L, and 557.77 × 109/L,p = 0.013). C reactive protein of the 30-40 mg/kg*day group was slightly higher than the other two groups. (7.76, 8.00, and 7.01 mg/L,p = 0.028).ConclusionsAspirin at the dose of 20–29 mg/kg/day dose not increase the risk of coronary artery damage and IVIG resistance compared with the dose of 30–50 mg/kg/day. This low dose may have a lower risk for a potential effect on liver function.

Kawasaki disease (KD) is a systemic inflammatory condition primarily affecting young children. We present an adolescent male with two episodes of complete KD between the age of 2 and 14 years. At age 14, he presented with findings suggestive of a retropharyngeal abscess. This was later determined to be a recurrence of KD, diagnosed after the development of coronary artery aneurysms. Our case reinforces the role of maintaining a high index of suspicion for KD, both in patients with prior KD episodes and in those with persistent fever who do not fulfill the diagnostic criteria for typical KD. This is particularly important for patients presenting with atypical symptoms not commonly associated with KD, such as inflammation of the retropharyngeal and parapharyngeal spaces.

Thrombosis within an occluded coronary arterial fistula (CAF) may cause angina and myocardial infarction. This study aims to estimate how the occlusion position of CAFs with terminal aneurysm affects the risk stratification of thrombosis in the fistula in terms of hemodynamics. Twelve CAF models were reconstructed based on patient-specific computed tomography angiogram (CTA) images. They were classified into three groups: preserved group (untreated fistula), aneurysm-reserved group (occluded at the fistula terminal: distal occlusion) and aneurysm-removed group (occluded before the aneurysm: proximal occlusion). Hemodynamics results were analyzed and compared with the clinical follow-up results. The results showed that: (1) Hemodynamic patterns within the fistula before and after treatment were significantly different among patients. (2) Aneurysm-removed occlusions showed better improvements with respect to the CAF blood-stealing phenomena. (3) Irrespective of whether aneurysms were removed or not, a disturbed flow pattern was observed. Areas having high OSI and low TAWSS were present in the post-occluded CAFs. The removal of the aneurysm, however, would alleviate the flow disturbance, and decrease the proportion of the area of OSI > 0.3. (4) The thrombosis region spotted in the follow-up patient CTAs was consistent with the computed high OSI area. A proximal occlusion, namely, removing the aneurysm of the CAF, may help in reducing the risk of thrombosis after surgery. However, follow-up studies with a larger cohort should be carried out to test and verify this speculation in the future.

Kawasaki disease is an acute febrile illness and systemic vasculitis of unknown aetiology that predominantly afflicts young children, causes coronary artery aneurysms and can result in long-term cardiovascular sequelae. Kawasaki disease is the leading cause of acquired heart disease among children in the USA. Coronary artery aneurysms develop in some untreated children with Kawasaki disease, leading to ischaemic heart disease and myocardial infarction. Although intravenous immunoglobulin (IVIG) treatment reduces the risk of development of coronary artery aneurysms, some children have IVIG-resistant Kawasaki disease and are at increased risk of developing coronary artery damage. In addition, the lack of specific diagnostic tests and biomarkers for Kawasaki disease make early diagnosis and treatment challenging. The use of experimental mouse models of Kawasaki disease vasculitis has considerably improved our understanding of the pathology of the disease and helped characterize the cellular and molecular immune mechanisms contributing to cardiovascular complications, in turn leading to the development of innovative therapeutic approaches. Here, we outline the pathophysiology of Kawasaki disease and summarize and discuss the progress gained from experimental mouse models and their potential therapeutic translation to human disease., This Review outlines the pathophysiology of Kawasaki disease and discusses the progress gained from experimental mouse models and their potential therapeutic translation to human disease., Key points Kawasaki disease is a childhood systemic vasculitis leading to the development of coronary artery aneurysms; it is the leading cause of acquired heart disease in children in developed countries.The cause of Kawasaki disease is unknown, although it is suspected to be triggered by an unidentified infectious pathogen in genetically predisposed children.Kawasaki disease might not be a normal immune response to an unusual environmental stimulus, but rather a genetically determined unusual and uncontrolled immune response to a common stimulus.Although the aetiological agent in humans is unknown, mouse models of Kawasaki disease vasculitis demonstrate similar pathological features and have substantially accelerated discoveries in the field.Genetic and transcriptomic analysis of blood samples from patients with Kawasaki disease and experimental evidence generated using mouse models have demonstrated the critical role of IL-1β in the pathogenesis of this disease and the therapeutic potential of targeting this pathway (currently under investigation in clinical trials).

A coronary pseudoaneurysm is a rare complication of chest trauma. In this report, we describe the case of a 65-year-old man with a mediastinal lesion. On admission, he complained of chest tightness and dry cough, and a pseudoaneurysm was confirmed in the left anterior descending branch of the coronary artery on chest computerized tomography, angiography, and coronary angiography. The patient had experienced chest trauma 5 years previously. With the help of extracorporeal bypass surgery, the pseudoaneurysm was resected under direct observation. The patient recovered well after surgery. Traumatic coronary artery pseudoaneurysms usually are asymptomatic and often misdiagnosed. Preoperative coronary angiography is a crucial diagnostic used for deciding appropriate surgical management.

Background Kawasaki disease (KD) is an acute and systemic vasculitis whose etiology remains unclear. The most crucial complication is the formation of coronary artery aneurysm (CAA). Annexin A1 (ANXA1) is an endogenous anti-inflammatory agent and pro-resolving mediator involved in inflammation-related diseases. This study sought to investigate the serum ANXA1 levels in KD patients and further explore the relationship between ANXA1 and CAA, as well as additional clinical parameters. Methods Serum samples were collected from 95 KD patients and 39 healthy controls (HCs). KD patients were further divided into two groups: KD with CAAs (KD-CAAs) and KD non-CAAs (KD-NCAAs). Serum levels of ANXA1 and interleukin-6 (IL-6) were determined using enzyme-linked immunosorbent assays. Results Serum ANXA1 levels in the KD group were significantly lower than in the HC group. In particular, serum ANXA1 levels were substantially lower in the KD-CAA groups. Moreover, serum ANXA1 levels were positively correlated with N%, C-reactive protein (CRP), and IL-6 but negatively correlated with L% in the KD group. Positive correlations between serum ANXA1 levels and erythrocyte sedimentation rate (ESR), IL-6, and D-dimer (DD) were observed in the KD-CAA group. Conclusions ANXA1 may be involved in the development of KD, and downregulation of ANXA1 may lead to the hypercoagulability seen in KD. Impact For the first time, it was demonstrated that serum ANXA1 levels were significantly decreased in Kawasaki disease with coronary artery aneurysms.ANXA1 might be involved in the acute phase of Kawasaki disease.Low serum concentrations of ANXA1 might lead to the hypercoagulability stage in Kawasaki disease.ANXA1 might be a potential therapeutic target for patients with Kawasaki disease.

Background The calcineurin and nuclear factor of activated T-cells (CaN-NFAT) signaling pathway had been found to be associated with Kawasaki disease (KD) susceptibility and coronary artery aneurysm formation as a contributor. To evaluate serum calcineurin (CaN) and nuclear factor of activated T-cells 1(NFAT1) levels in patients with Kawasaki disease (KD). Methods Serum levels of CaN and NFAT1 were measured by enzyme-linked immunosorbent assay method in 66 healthy children and 74 KD patients at acute, afebrile and subacute stage. Results The serum levels of CaN and NFAT1 increased significantly in the acute stage, and decreased progressively in the afebrile and subacute stage, along with the reduction of C-reactive protein, white blood cells and neutrophil counts. And in the acute stage, the afebrile stage and the subacute stage, the expression of CaN and NFAT1 was upregulated significantly in KD patients compared to that in the healthy control. After the IVIG treatment, the serum levels of CaN and NFAT1 declined significantly in IVIG responders. However, the CaN and NTAT1 levels in the IVIG non-responders declined slowly. And in the afebrile stage, the NFAT1 levels were lower in KD patients with coronary artery lesions (CALs) (268.82 ± 11.96 ng/ml) than those without CALs (285.84 ± 25.13 ng/ml). However, the serum levels of CaN in KD patients with CALs had no significant difference with those in KD patients without CALs. Conclusions The specific regulation of CaN and NFAT1 serum levels in the course of KD was suggested that both of them were related in the development of KD.

A 71-year-old man attended the emergency department with an episode of transient loss of consciousness. He had collapsed while standing from a sitting position soon after a hot bath. There was no antecedent chest pain; however, following this episode, he reported new onset exertional dyspnoea while walking around his home. Electrocardiography on presentation demonstrated intermittent left bundle branch block (QRS duration 145 ms), and an echocardiogram confirmed a normal size left ventricle with normal left ventricular systolic …

Coronary artery aneurysm is a rare disease. It occurs in the epicardial space, mostly along the course of major coronary arteries. Here, we report a case of a giant incidental aneurysm embedded in the basal posterior wall of the left ventricle. A 43-year-old woman was referred to our institution for the evaluation of cardiac palpitations that had been present from the previous 2 months. She reported no medical history (such as Kawasaki’s disease or hypertension) or previous operative history. Echocardiogram and subsequent cardiac CT revealed a giant aneurysm in the left ventricle, with a direct fistulous connection to a dilated and tortuous left circumflex artery, which showed direct communication with the straight right coronary artery.

Introduction: Coronary aneurysms are rare and are linked to drug abuse; symptomatology depends on the coronary anatomy. This is a case of acute coronary syndrome associated with a giant right coronary aneurysm. Case description: A 40-year-old male, with history of heroin and crack use since age 20, attended consultation due to dyspnea, stable angina and diaphoresis. An electrocardiogram showed ST segment overlay on the underside and troponin problems. A coronary catheterization was performed, which revealed apparent inconclusive aorta-to-right atrium fistula. Based on the findings, angiotomography and magnetic resonance imaging were performed, finding a giant right coronary aneurysm. The aneurysm was resected using extracorporeal circuit, femoral cannulation, moderate hypothermia, aortic cross-clamping and cardioplegia, and the right coronary artery was revascularized with the left internal saphenous vein. The patient had a satisfactory postoperative period and was discharged after 7 days. Conclusion: There is an important association between drug use and the development of coronary aneurysms. Aneurysm size makes diagnosis difficult, so complementary studies are necessary to establish a differential diagnosis. An appropriate surgical approach allows for a complete resection of the aneurysm and optimal coronary revascularization. RESUMEN Introducción. La presentación de aneurismas coronarios es rara, sin embargo se asocian al abuso de drogas; su sintomatología depende de la anatomía coronaria. Se presenta el caso de un síndrome coronario agudo asociado a un aneurisma gigante de la arteria coronaria derecha. Presentación del caso. Paciente masculino de 40 años con antecedente de consumo de heroína y crack desde los 20 años, quien consultó por disnea, angina estable y diaforesis. El electrocardiograma evidenció supradesnivel del segmento ST en cara inferior y elevación de troponinas, por lo que se realizó cateterismo coronario que reveló aparente fístula aorto-atrial derecha no concluyente. Dados los hallazgos, se decidió realizar angiotomografía y resonancia magnética que mostraron aneurisma gigante de arteria coronaria derecha. Se realizó resección de aneurisma con circulación extracorpórea, canulación femoral, hipotermia moderada, pinzamiento aórtico y cardioplejia, y se revascularizó la arteria coronaria derecha con vena safena interna izquierda. El paciente tuvo posoperatorio satisfactorio y se le dio de alta a los 7 días. Conclusiones. El tamaño del aneurisma puede dificultar su diagnóstico, por lo que estudios complementarios son útiles para establecer un diagnóstico diferencial. El abordaje quirúrgico adecuado permite realizar una resección completa del aneurisma y una revascularización coronaria óptima.

Background Coronary artery aneurysm is a rare condition which constitutes a small proportion of coronary artery disease. Such condition mimicking an intra-cardiac mass is extremely rare and poorly understood. Case presentation We present an unusual case of a 53-year-old female with decreased exercise tolerance and lower extremity edema for 3 months. The echocardiography showed moderate tricuspid regurgitation and a right intra-ventricular mass below the tricuspid valve. No ventricular wall akinesia or ST segment change was found on echocardiography or electrocardiogram. Coronary computed tomographic angiography confirmed the diagnosis of intra-ventricular mass with feeding vessel originated from the right coronary artery. The patient was scheduled for tumor resection, and the mass turned out to be a thrombosed giant right coronary artery aneurysm. The patient received successful aneurysm resection and had an uneventful postoperative recovery. Unfortunately, a fistula between right coronary artery and right ventricle was detected on follow-up three months later by echocardiography. Conclusions Coronary artery aneurysms presenting as intra-cardiac masses are extremely rare. Comprehensive preoperative evaluation is highly recommended because the surgical strategies for tumor and aneurysm are completely different. Aneurysm resection with bypass surgery is preferred rather than aneurysm repair. To our best knowledge, coronary artery aneurysms presenting as intra-ventricular masses have seldom been reported.

A man diagnosed with immunoglobulin G4 (IgG4)-related disease at the age of 65 years underwent abdominal aortic replacement due to an abdominal aortic aneurysm. In the same hospitalization period, a small coronary artery aneurysm was noticed. He was treated with corticosteroids and his serum IgG levels returned to normal. After experiencing sudden chest pain at age 74 years, coronary angiography showed that the size of the aneurysm had increased dramatically. He underwent coronary artery bypass graft and coronary artery resection without using cardiopulmonary bypass. Thus, we conclude that observation of aneurysms in patients with IgG4-related disease is important, even under corticosteroid therapy.

Although covered stents have been available for percutaneous treatment of coronary aneurysms, patients with longer aneurysmal segments have been difficult to treat with covered stents. We describe a case of a right coronary artery aneurysm with an angiographically estimated length exceeding 30 mm treated percutaneously using covered stents and conventionally available hardware. (Level of Difficulty: Advanced.), Graphical abstract, Although covered stents have been available for percutaneous treatment of coronary aneurysms, patients with longer aneurysmal segments have been…