Incidence Of Coronary Artery Aneurysms Research Articles

The Effectiveness of No or Low-Dose versus High-Dose Aspirin in Treating Acute Kawasaki Disease: A Systematic Review and Meta-Analysis.

This systematic review and meta-analysis assesses the effectiveness of no or low-dose versus high-dose aspirin on the incidence of coronary artery aneurysms (CAAs), intravenous immunoglobulin (IVIG) resistance, hospital stay length, and fever duration during the acute phase of Kawasaki disease. Our review adheres to the Preferred Reporting Items for Systematic Reviews guidelines. The PubMed and Google Scholar databases were comprehensively searched to identify relevant studies in the literature, including observational studies and randomized controlled trials (RCTs). The primary outcome was the incidence of CAAs. The secondary outcomes were the hospital stay length, fever duration, and IVIG resistance. The risk of bias was assessed using the Newcastle-Ottawa scale for cohort studies and Cochrane's Risk of Bias Tool for RCTs. The data were analyzed using the Review Manager software. Twelve studies with a total of 68,495 participants met the inclusion criteria. The incidences of CAAs (odds ratio [OR] = 0.93; 95% confidence interval [CI] = 0.64-1.34) and IVIG resistance (OR = 1.46; 95% CI = 1.00-2.12) did not differ significantly between no or low-dose versus high-dose aspirin in treating acute KD. Moreover, the fever durations (mean difference [MD] = 3.55 h; 95% CI = -7.99-15.10) and hospital stay lengths (MD = -0.54 days; 95% CI = -2.50-1.41) were similar in the no and low-dose aspirin group compared to the high-dose aspirin group. Our review indicates that there are no significant differences in the incidences of CAA and IVIG resistance, fever durations, and hospital stay lengths between no or low-dose versus high-dose aspirin in treating the acute phase of KD.

Post Percutaneous Transluminal Coronary Angioplasty (PTCA) Coronary Aneurysm- A Case Report

Introduction Coronary aneurysm defined angiographically as luminal dilation 50% larger than that of the adjacent reference segment. (1) Drug-eluting stents (DES), which locally elute antiproliferative drugs, can dramatically inhibit neointimal growth. However, several pathological studies have indicated that DES may delay healing after vascular injury, and DES implantation may be theoretically associated with a risk of coronary artery aneurysm formation. Coronary aneurysms have been reported from 3 days to up to 4 years after DES implantation procedures, with varying clinical presentations. The incidence of coronary artery aneurysms after DES implantation is low within the first 9 months, with a reported incidence of 0.2% to 2.3%, a rate similar to that reported after bare-metal stent (BMS) implantation (0.3% to 3.9%) in the DES versus BMS randomized trials.(2) However, the true incidence of coronary aneurysms in an unselected patient population is still largely unknown. It can be congenital, or secondary to vasculitis (Kawasaki disease) or after percutaneous coronary intervention. Drug-eluting stents (DES), which locally elute antiproliferative drugs, can dramatically inhibit neointimal growth has become standard of care for routine coronary angioplasty. However, several pathological studies have indicated that DES may delay endothelial healing after vascular injury, and DES implantation may be theoretically associated with a risk of coronary artery aneurysm formation (3)

Intermediate and long-term outcomes of transcatheter closure of congenital coronary cameral fistulas in 66 children

Objective: To evaluate the intermediate and long-term outcomes and technical aspects of transcatheter closure (TCC) of coronary cameral fistulas (CCF) in pediatric patients. Methods: This was a case-control study. All pediatric patients with CCF who underwent TCC between January 2005 and December 2019 were retrospectively reviewed. Data was collected from medical records, including demographic characteristics, procedural details, intraoperative and postoperative serious adverse events, follow-up results and prognosis. Patients with serious adverse events and without serious adverse events were compared regarding their clinical features and CCF characteristics. Comparisons between groups were performed with independent sample t test, chi-square test or Fisher exact test. Results: A total of 66 CCF patients (34 boys, 32 girls, 3.9 (1.9, 6.2) years old, 15 (11, 20) kg) underwent attempted TCC. All of the CCF were all medium or large fistulas including 55 proximal fistulas (83%) and 11 distal fistulas (17%). The CCF originated more frequently from the right coronary artery (38 cases (58%)), followed by the left coronary artery (28 cases (42%)). The incidence of coronary artery aneurysms (CAA) was 61% (40/66).Procedural treatment was achieved in 64 patients and procedural success was achieved in 59 patients (92%). Six (9%) serious adverse events occurred in 5 patients during the perioperative period. Acute complications included procedure-related death in one patient and acute myocardial infarction in one patient. Periprocedural complications occurred in 3 patients at one day postoperatively including acute myocardial infarction (2 cases), occluder detachment (1 case), and tricuspid chordae tendinae rupture (1 case). Clinical follow-up data were available in 58 of the 62 patients who underwent initial successful TCC with a follow-up period of 9.3 (6.5, 13.4) years. Ten adverse events occurred in 9 patients including 5 complications consisted of aortic valve perforation (1 case), coronary thrombosis (1 case), progressive aneurysmal dilation after reintervention (1 case), and new-onset tricuspid valve prolapse with significant regurgitation (2 cases) and large residual shunts due to fistula recanalization (5 cases). Therefore, the incidence of intermediate and long-term adverse events was 17% (10/58). During the periprocedural and follow-up period, 16 adverse events occurred in 13 patients, whereas no adverse events occurred in 51 patients. Patients with seriovs adverse events presented with larger proportion of large CCF (11/13 vs. 39% (20/51), P=0.005), giant CAA (10/13 vs.14% (7/51), P=0.030), and higher mean pulmonary artery pressure ((20±9) vs.(16±6) mmHg, 1 mmHg=0.133 kPa, t=2.02, P=0.048) compared to patients without serious adverse events. Conclusions: TCC in CCF children appears to be effective with favorable intermediate and long-term outcomes. Strict indication of TCC is mandatory.

EE61 Cost Effectiveness of Treating Ivig-Resistant Kawasaki Disease Children with Second Round IVIG Versus Infliximab

Intravenous immunoglobulin (IVIG) is a known effective treatment to reduce the incidence of coronary artery aneurysms in children with Kawasaki Disease (KD). However, there are IVIG-resistant patients and a lack of clinical trial data to determine the best second round treatment. Using recent effectiveness findings from the KIDCARE study, this research aimed to determine if second round IVIG or infliximab is cost effective in KD patients with persistent or recrudescent fever.

Complete and incomplete Kawasaki disease: Clinical differences and coronary artery outcome from a national prospective surveillance study in Switzerland.

The aim of this national prospective surveillance study was to compare the clinical presentation, laboratory findings, treatment, and coronary artery outcome in patients with incomplete and complete Kawasaki disease (KD). Between March 2013 and February 2019, children with a diagnosis of complete and incomplete KD were reported by the Swiss Paediatric Surveillance Unit and prospectively enrolled. Clinical data, laboratory values, treatment, and echocardiographic features were collected at diagnosis and 1 year of follow-up. Data were compared between children with complete or incomplete KD. A total of 351 questionnaires were registered from children with a diagnosis of KD. Of them, 219 (62.4%) children had complete KD, and 132 (37.6%) children had incomplete KD. Children with incomplete KD were younger and had a longer-lasting fever; however, there were no differences in the level of C-reactive protein. All but four children received intravenous immunoglobulin treatment, whereas 14% of children were treated with corticosteroids. Children with incomplete KD were more often treated with corticosteroids than children with incomplete KD (p = 0.01). At diagnosis, 39 (11.1%) patients had only coronary artery dilation and 57 (16.2%) had at least one coronary artery aneurysm. There were no differences in coronary artery involvement between the two groups. At follow-up, 273 of 294 (92.8%) patients had no coronary artery involvement, with no difference between the two groups (p = 0.609). The overall incidence of coronary artery aneurysms at diagnosis was 16.2%. At follow-up, most coronary artery aneurysms had regressed, and coronary artery aneurysms were present in only 5.8% of the patients. Coronary artery aneurysms were slightly more frequent in patients with incomplete KD at follow-up (p = 0.039) but not at diagnosis (p = 0.208). Although the clinical presentation in children with incomplete and complete KD differs, the absence of coronary artery involvement does not. The use of corticosteroids appears to be preventive against the development of coronary artery aneurysms in these patients. However, the results of this study suggest a lower rate of coronary artery aneurysm regression in patients with incomplete KD. Further studies on a larger scale are needed to assess the risk of non-regression of coronary artery aneurysms in this particular group of patients.

Infliximab as a second-line therapy for children with refractory Kawasaki disease: A systematic review and meta-analysis of randomized controlled trials.

Infliximab is a tumour necrosis factor-alpha inhibitor that is used to treat children with refractory Kawasaki disease (KD). Our purpose was to evaluate the safety and impact of infliximab versus intravenous immunoglobulins on the incidence of coronary artery aneurysms (CAAs) and treatment resistance in children with refractory KD. The Medline/PubMed, Embase, CINAHL, Cochrane Central Register of Controlled Trials and clinical trials registries were searched to December 2021. Randomized controlled trials (RCTs) comparing infliximab as second-line therapy to a second dose of intravenous immunoglobulin (IVIG) in children with refractory KD, reported in abstract or full text, were included. Studies were selected and assessed for risk of bias by two reviewers. Data were extracted and pooled using conventional random-effects meta-analysis. The certainty of evidence was assessed using the GRADE system. A total of 199 participants from four RCTs were included. The pooled risk ratio (RR) for the incidence of treatment resistance in patients treated with infliximab was 0.40 (95% confidence interval [CI] 0.25-0.64). For incidence of CAAs RR was 1.20 (95% CI 0.54-2.63), the incidence of adverse effect "infusion reactions" RR was 0.48, (95% CI 0.12-1.92) and for "infections" RR was 0.55 (95% CI 0.27-1.12). Overall, the GRADE strength of evidence for the primary outcomes was low. Evidence on the duration of fever and inflammatory biomarkers was sparse, heterogeneous and inconclusive. Moderate-certainty evidence indicates that infliximab may reduce the incidence of treatment resistance in children with refractory KD. However, the limited strength of evidence warrants further research.

Spontaneous defervescence and its prediction in the acute phase of Kawasaki disease.

BackgroundIn Kawasaki disease (KD), fever occasionally resolves spontaneously before 10 days from the onset, right after diagnosing. However, there is not enough evidence of intravenous immunoglobulin (IVIG) treatment in this case. The aim of this study was to investigate the relationship between spontaneous defervescence and coronary artery aneurysm and to develop a scoring model for its prediction in acute KD.MethodsAll patients admitted for acute KD in Asan Medical Center were considered for inclusion. Acute management involved the administration of 2 g/kg of IVIG and 5 mg/kg/day of aspirin. The patient whose temperature was <37.5°C for more than 48 h from the diagnosis was discharged under the judgment of spontaneous defervescence, without IVIG administration.ResultsThe incidence of coronary artery aneurysm was 5.7% in 94 defervesced patients and 4.6% in the 1,277 patients treated with IVIG in the subacute phase (P = 0.593), and 2.5 and 2.2% in respective patient groups in the convalescent phase (P = 0.924). A scoring model which predicted spontaneous defervescence under the combination of C-reactive protein ≤10mg/dL and ≥2 conditions of no rash, neutrophil ≤65%, and/or alanine aminotransferase ≤80 IU/L, was developed and showed 80.7% sensitivity, 68.8% specificity, 15.8% positive predictive value, and a 97.8% negative predictive value.ConclusionThe incidence of coronary artery aneurysm in patients with the defervesced KD was not different from the IVIG treated patients. In the cases suitable for the predictive model, patients can wait for the spontaneous defervescence under intensive observation by medical professionals.

A Preliminary, Single-Center Retrospective Chart Review of Infusion Times of Intravenous Immune Globulin in Kawasaki Disease and Clinical Outcomes.

Patients diagnosed with Kawasaki disease (KD) are at a high risk of developing coronary artery aneurysms. Intravenous immune globulin (IVIG) given in combination with aspirin is the standard of treatment for the prevention of coronary aneurysm. IVIG is recommended to be administered as a dose of 2 g/kg infused during 10 to 12 hours for the prevention of coronary aneurysms in KD; however, this does not always occur in practice. We aimed to investigate if an infusion time of <10 hours is associated with more coronary artery aneurysms than the recommended infusion time of 10 to 12 hours. Patients with a diagnosis of and treated for KD with IVIG at the University of Chicago Medicine Comer Children's Hospital were identified by drug use reports that included patients who received IVIG between September 2008 and August 2018. Data were collected though chart review and patients were divided into 2 groups based on duration of infusion (<10 hours and 10-12 hours). The primary outcome was the incidence of coronary artery aneurysm. The secondary outcome was the time to defervescence. The safety outcome was the development of renal dysfunction. A total of 70 patients were screened and 44 were included in the analysis. Coronary aneurysm occurred in 2 of 33 patients (6.0%) in the <10-hour group and no patients in the 10- to 12-hour group (p = 0.558). The median time to defervescence was 0.5 hours in the <10-hour group and 0.95 hours in the 10- to 12-hour group (p = 0.166). The incidence of acute kidney injury was 6% (2 of 33 patients) in the 10-hour group and 9.1% (1 of 11 patients) in the 10- to 12-hour group (p = 0.588). All incidences of coronary artery aneurysm occurred in the patients who received IVIG with an infusion time of <10 hours. The incidence of acute kidney injury was numerically higher in the 10- to 12-hour group. Based on the recommendations in the American Heart Association KD guideline, our internal hospital policy, and our results, we recommend the infusion of IVIG be administered at a rate of 10 to 12 hours.

Mycotic and non-mycotic coronary artery aneurysms-A review of the rarity.

Sir William Osler coined the term “mycotic” to identify aneurysms secondary to an infectious cause, which may not be necessarily fungal and are caused mainly by bacteria. The literature’s reported incidence of coronary artery aneurysms (CAA) is from 1.5-5%. The right coronary artery (RCA) is mainly involved, followed by the left side coronary circulation. Mycotic aneurysms are more commonly associated with infective endocarditis. More recently, coronary artery stents, particularly drug-eluting stents, are typically causing mycotic coronary aneurysms. CT angiography (CTA) has been the forefront diagnostic modality, showing both the lumen and wall of the coronary arteries. It also aids in preoperative planning. MRI is useful in diagnosing and following children with Kawasaki’s disease. Smaller mycotic coronary aneurysms may resolve with antibiotic therapy; however, aneurysms more significant than 1-2 cm diameter needs corrective surgery. Early diagnosis and appropriate management are the critical factors in the successful treatment of infective coronary artery aneurysms.

Identifying children with Kawasaki disease at high risk for coronary aneurysms

Kawasaki disease, a childhood vasculitis manifested by symptoms of high fever, mucocutaneous inflammation, and unilateral cervical adenopathy, can cause lasting damage to the coronary arteries, and more rarely, other medium-sized muscular arteries. Most children respond well to prompt treatment with high-dose intravenous immunoglobulin (IVIG). However, coronary artery aneurysms, defined by internal lumen diameter at least 2.5 SDs above the population mean for body surface area (Z≥2.5), occur in ∼25% of affected children and are the principal cause of long-term morbidity and mortality in Kawasaki disease. Several risk scores have attempted to predict which children are likely to develop coronary aneurysms. In previous studies in North America, baseline risk factors for coronary artery aneurysms on echocardiography performed 2-8 weeks after Kawasaki disease onset have included higher coronary artery dimensions on baseline echocardiography, IVIG resistance (the persistence or recrudescence of fever after a single dose of IVIG), young age (<12 months or <6 months depending upon the risk score), Asian race, and late diagnosis and treatment with IVIG. Japanese risk scores based upon clinical and laboratory variables have been highly accurate in predicting IVIG resistance, one of the strongest risk factors for coronary artery aneurysms. In Japanese children predicted to have high risk of IVIG resistance, primary adjunctive therapy with corticosteroids or cyclosporine has been shown in randomized clinical trials to reduce the incidence of coronary artery aneurysms. However, Japanese children predicted to be at low risk for aneurysms on the basis of laboratory measures may still develop coronary abnormalities. Understanding the risk factors for aneurysm development thus requires further refinement. In this volume of The Journal, Iio et al analyze independent risk factors for development of coronary artery aneurysms in 1632 Japanese children with Kawasaki disease who were predicted to be at low risk for resistance to primary IVIG treatment. In this low-risk group, 5.5% of patients developed any coronary artery aneurysms and 1%, medium or large coronary aneurysms. In multivariable analysis, baseline coronary artery Z score >2.5, age <12 months at fever onset, and IVIG resistance were independent risk factors for diagnosis of coronary artery aneurysms on echocardiography one month after disease onset. Among these risk factors, coronary artery Z score ≥2.5 was most strongly associated with aneurysm development. Taken together with previous literature, these data suggest that enlarged coronary Z scores measured on baseline echocardiography can be used to select children at high risk for subsequent coronary artery aneurysms and thus who may benefit from adjunctive therapy. The study of Iio et al adds weight to the concept that a high-risk cohort for randomized clinical trials of primary adjunctive therapy in Kawasaki disease may be most simply selected through findings on baseline echocardiography. The ability to select such a high-risk cohort is critical to the design of trials to answer remaining questions in Kawasaki disease therapy: In children of all races and ethnicities, who should receive treatment with adjunctive therapies? How do antiinflammatory therapies compare with each other, and what is the cost/benefit ratio for these therapies? Article page 158 Risk Factors of Coronary Artery Aneurysms in Kawasaki Disease with a Low Risk of Intravenous Immunoglobulin Resistance: An Analysis of Post RAISEThe Journal of PediatricsVol. 240PreviewTo detect risk factors of coronary artery aneurysm (CAA) development in patients with Kawasaki disease determined to have a low risk for resistance to primary intravenous immunoglobulin (IVIG) treatment based on the Kobayashi score. Full-Text PDF

FNDC1 Polymorphism (rs3003174 C > T) Increased the Incidence of Coronary Artery Aneurysm in Patients with Kawasaki Disease in a Southern Chinese Population.

BackgroundA large number of studies demonstrated that the key to the occurrence and development of Kawasaki disease (KD) is the over-activation of immune cells and the generation of various inflammatory factors, leading to the imbalance of the immune system. Recently, mutations in the FNDC1 gene have been shown to be associated with inflammatory responses. However, there have been no reports on the relationship between FNDC1 gene and KD so far.MethodsWe enrolled 1611 controls and 1459 patients with KD, including 372 patients with coronary artery aneurysm (CAA) and 179 patients with coronary artery lesion (CAL). The relationship between FNDC1 rs3003174 polymorphism and KD with CAA or without CAA was investigated.ResultsThis study showed no evidence that the association between FNDC1 rs3003174 C>T polymorphism and KD susceptibility was statistically significant (CT versus CC: adjusted odds ratio (OR) =0.897, 95% confidence interval (CI) =0.769–1.045, P=0.162; TT versus CC: adjusted OR=0.995, 95% CI=0.786–1.260, P=0.968; dominant model: adjusted OR=0.916, 95% CI=0.792–1.059, P=0.235; and recessive model: adjusted OR=1.055, 95% CI=0.845–1.316, P=0.638). However, our further stratified analysis in the control and KD group bore out that the incidence of TT genotype of FNDC1 rs3003174 C > T polymorphism was higher than that of CC/CT genotype in KD patients stratified by CAA (adjusted OR=1.437, 95% CI=1.034–1.996, P=0.031). Moreover, a stratified analysis of age and gender in KD patients indicated that the rs3003174 TT genotype increased the risk of CAA formation in aged ≦60 months (CC/CT vs TT: adjusted OR=1.580, 95% CI=1.106–2.259, P=0.012) and male (CC/CT vs TT: adjusted OR=1.653, 95% CI=1.101–2.481, P=0.015) KD patients.ConclusionThe results of this study demonstrated that the FNDC1 rs3003174 C>T polymorphism may be a hazard factor in the formation of CAA in KD patients that was not disclosed before.

Evaluation of high-dose aspirin elimination in the treatment of Kawasaki disease in the incidence of coronary artery aneurysm.

Background:Standard first-step therapy for Kawasaki disease consists of Intravenous immunoglobulin and high dose Aspirin (80-100 mg/kg/day). The standard dose of Intravenous immunoglobulin (2gr/kg) is strongly effective in reducing the risk of coronary arteries abnormalities. So, the proper dose and efficacy of Aspirin to decrease the risk of coronary arteries abnormalities is a controversial issue. In this study, it is tried to assess the result of eliminating high-dose Aspirin in the treatment of the acute phase of Kawasaki and observe the incidence rate of coronary arteries abnormalities when only Intravenous immunoglobulin was administered.Methods:This study is a prospective randomized, open-label, blinded end-points clinical trial performed in Afzalipour hospital in Kerman University of Medical Sciences from September 2017 to September 2018 in 62 patients with typical and atypical Kawasaki disease. The study group received Intravenous immunoglobulin (2 g/kg) and the control group get the same dose of Intravenous immunoglobulin plus Aspirin with the dose of 80-100 mg/Kg/day until they were afebrile for 48 hours. Afterward, both groups received a daily single dose (3-5 mg/kg) of Aspirin for six weeks. Echocardiography was done after two weeks, six weeks, and six months. Internal diameter of the left and right main coronary arteries was measured and then the corresponding Z-score was calculated.Results:In the study group, coronary arteries abnormalities decreased from 38.7% in the 2nd week to 16.1% in the 6th month. In the control group, it declined from 54.8% to 22.6%. There was no statistically significant difference between the groups in term of frequency of abnormal coronary arteries at the study period (P=0.151).Conclusions:We concluded that high dose Aspirin does not have a significant role in preventing coronary arteries abnormalities in Kawasaki disease and giving standard 2 gr/kg/day Intravenous immunoglobulin without high-dose Aspirin in acute-phases therapy does not increase the risk of coronary arteries abnormality.

Characteristics and trends in diagnosis of Kawasaki disease outside the usual age range.

To explore the trends in diagnosis of Kawasaki disease (KD) and determine the characteristics for patients outside the usual age range of 6months to 4years. A retrospective review of patients with KD identified in four epidemiological surveys spanning 20years (1998-2017) in Shanghai was performed. A total of 8416 patients were included. All in all, 223 (2.6%) were aged 0 to 2months, 639 (7.6%) were 3 to 5months, 6556 (77.9%) were 6months to 4years, 915 (10.9%) were 5 to 9years, and 83 (1.0%) were ≥ 10years. The use of intravenous immunoglobulin increased in all extreme age groups, and delayed treatment rates decreased in all patients except in those aged 0 to 2months and ≥ 10years. The number of patients outside the usual age increased over time, but the proportion of these patients did not change significantly. They had more incomplete KD and coronary artery aneurysms (CAA), while those aged 0 to 2months and ≥ 10years had more delayed diagnoses in their respective age groups. The incidence of CAA was similar in younger and older children, but the former group had more cases of incomplete KD and abnormal laboratory parameters, while the latter group had longer duration of fever and was more difficult to diagnose promptly. The proportion of KD outside the usual age range did not increase over time. Older and younger children have different clinical and laboratory characteristics. Key Points • The number of patients outside the usual age increased over time, but the proportion of these patients did not increase. • Patients aged 0 to 2months and ≥ 10years had more delayed diagnoses in their respective age groups. • Younger children had more cases of incomplete KD and abnormal laboratory parameters, while older children had longer duration of fever and were more difficult to diagnose promptly.

Predictors of Immunoglobulin therapy resistance in Kawasaki disease: Section on Cardiology and Cardiac Surgery Program: Day 2

Purpose: Kawasaki disease (KD) is the most common cause of acquired heart disease in children in developed countries. Timely initiation of treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery aneurysms from 25% to 4%. Approximately 10% to 20% of patients with KD are IVIG resistant. Because IVIG-resistant patients are at higher risk for coronary artery aneurysms, it is important to identify these patients who might benefit from more aggressive initial therapy. Currently available IVIG resistance prediction models, originally developed with data from Asian populations, are …

Effect of different doses of aspirin on the prognosis of Kawasaki disease

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.

Infliximab Treatment for Intravenous Immunoglobulin-resistant Kawasaki Disease: a Multicenter Study in Korea.

Background and ObjectivesWe investigated the status of infliximab use in intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients and the incidence of coronary artery aneurysms (CAAs) according to treatment regimens.MethodsBetween March 2010 and February 2017, 16 hospitals participated in this study. A total of 102 (32.3±19.9 months, 72 males) who received infliximab at any time after first IVIG treatment failure were enrolled. Data were retrospectively collected using a questionnaire.ResultsSubjects were divided into two groups according to the timing of infliximab administration. Early treatment (group 1) had shorter fever duration (10.5±4.4 days) until infliximab infusion than that in late treatment (group 2) (16.4±4.5 days; p<0.001). We investigated the response rate to infliximab and the incidence of significant CAA (z-score >5). Overall response rate to infliximab was 89/102 (87.3%) and the incidence of significant CAA was lower in group 1 than in group 2 (1/42 [2.4%] vs. 17/60 [28.3%], p<0.001).ConclusionsThis study suggests that the early administration of infliximab may reduce the incidence of significant CAA in patients with IVIG-resistant KD. However, further prospective randomized studies with larger sample sizes are required.

Aspirin Dose in Kawasaki Disease: The Ongoing Battle.

Kawasaki disease (KD) is an acute childhood vasculitis that may result in coronary aneurysms. Treatment of KD with a single infusion of 2 gm/kg intravenous immunoglobulin (IVIG) is well established, but acetylsalicylic acid (ASA) dose remains controversial. Our primary objective was to determine the difference in the incidence of IVIG resistance between 2 ASA doses. Our secondary objective was to compare the duration of hospital stay and the incidence of coronary artery aneurysm. We reviewed charts of patients with KD from 2 Canadian centers to assess the impact of ASA dose on IVIG resistance (operationally defined as administration of a second dose of IVIG). Both centers used standard IVIG dosing, but center 1 used low-dose ASA from diagnosis (3-5 mg/kg/day) while center 2 used initial high-dose ASA (80-100 mg/kg/day). There were no significant differences in baseline characteristics between the 2 centers. Retreatment with a second dose of IVIG was required in 28 of 122 patients (23%) treated with low-dose ASA, and in 11 of 127 patients (8.7%) treated with high-dose ASA in center 1 and center 2, respectively (P = 0.003). After adjusting for confounders, low-dose ASA was associated with higher odds of IVIG resistance (OR 3.2 [95% confidence interval 1.1, 9.1]). The mean duration of hospital stay was 4.1 and 4.7 days, respectively (P = 0.37). Coronary artery aneurysms were seen in 2 of 117 and 6 of 125 patients from centers 1 and 2, respectively (P = 0.28). Low-dose ASA was associated with 3-times higher odds of IVIG retreatment compared to high-dose ASA, with no significant difference in duration of hospital stay or incidence of coronary artery aneurysms.

Olbrzymi tętniak obejmujący kilka tętnic wieńcowych, w tym pień tętnicy wieńcowej lewej, powodujący objawy ostrego zespołu wieńcowego u 23-letniego chorego: nietypowe powikłanie choroby Kawasaki

The reported incidence of coronary artery aneurysms (CAA) on angiography varies between 0.3 to 5.3%. Right coronary artery is the most commonly affected followed by left circumflex or left anterior descending artery. Three-vessel or left main involvement is exceedingly rare. Atherosclerosis accounts for the vast majority of CAAs in adults, whereas Kawasaki disease is responsible for most cases in children. We report a rare case of Kawasaki disease associated with giant aneurysms ( > 8 mm) in coronary circulation, including left main coronary artery in 23-year-old male having maturity onset diabetes (MODY), who presented with acute coronary syndrome.

Urgent Coronary Artery Bypass Grafting Due to Multi- Vessel Coronary Aneurysm

Coronary Artery Aneurysm (CAA) is defined as a dilation of more than 1.5 times normal in a segment of the coronary artery. While the incidence of CAA is highest in the Right Coronary Artery (RCA), it is agreed that left main coronary artery or three-vessel involvement is extremely rare. Wide ranges of factors have been implicated in the cause of CAA with atherosclerosis being the most common at 50%. Congenital CAA occurs in 20-30% of cases followed by connective tissue disease at 10%. Blood stagnation and exposure of the underlying collagen make aneurysms prone to thrombosis, dissection, and vasospasm. Depending on the size, symptoms, and etiology of the aneurysms; a surgical, percutaneous, or medical approach may be used. In this paper, we are presenting a patient who presented to the emergency department (ED) with chest pain due to Acute Coronary Syndrome (ACS) that was found to be due to multi-vessels CAAs involving the left main as well as RCA, Left Anterior Descending (LAD) artery and Left Circumflex Artery (LCA) requiring urgent Coronary Artery Bypass Grafting (CABG).

Retrospective observational study indicates that the paediatric assessment triangle may suggest the severity of Kawasaki disease.

We examined whether the paediatric assessment triangle (PAT) could predict the severity of Kawasaki disease. We enroled patients diagnosed with Kawasaki disease between July 2012 and June 2016 at the emergency department of Tokyo Metropolitan Children's Medical Center in Tokyo, Japan. Triage nurses assigned participants to unstable or stable PAT groups. We compared the incidence of coronary artery aneurysms (CAA), the Kobayashi score, which measures resistance to intravenous immunoglobulin treatment, and the incidence of initial treatment resistance. Of the 420 participants, who were aged 0-145 months with a mean age of 31.2 ± 23.9 months, 66 (16%) were assigned to the unstable PAT group. The incidence of CAA was similar between the two groups. The percentage of unstable PAT group participants with a Kobayashi score of at least five points (39 versus 18%, p < 0.001) and initial treatment resistance (25 versus 15%, p = 0.047) were significantly higher than in the stable PAT group. Unstable PAT was an independent risk factor for initial treatment resistance (odds ratio 2.02, 95% confidence interval 1.05-3.90, p = 0.035). An unstable PAT was able to predict the severity of Kawasaki disease when measured by a higher rate of initial treatment resistance.