Coronary Artery Aneurysms In Patients Research Articles

Sex-Specific Susceptibility Loci Associated With Coronary Artery Aneurysms in Patients With Kawasaki Disease.

Kawasaki disease (KD) is an acute vasculitis that primarily affects children under age 5 years. Approximately 20-25% of untreated children with KD and 3-5% of those treated with intravenous immunoglobulin therapy develop coronary artery aneurysms (CAAs). The prevalence of CAAs is much higher in male than in female patients with KD, but the underlying factors contributing to susceptibility to CAAs in patients with KD remain unclear. This study aimed to identify sex-specific susceptibility loci associated with CAAs in KD patients. A sex-stratified genome-wide association study (GWAS) was performed using previously obtained GWAS data from 296 KD patients and a new replication study in an independent set of 976 KD patients by comparing KD patients without CAA (controls) and KD patients with aneurysms (internal diameter ≥5 mm) (cases). Six male-specific susceptibility loci, PDE1C, NOS3, DLG2, CPNE8, FUNDC1, and GABRQ (odds ratios [ORs], 2.25-9.98; p=0.00204-1.96×10-6), and 2 female-specific susceptibility loci, SMAD3 (OR, 4.59; p=0.00016) and IL1RAPL1 (OR, 4.35; p=0.00026), were significantly associated with CAAs in patients with KD. In addition, the numbers of CAA risk alleles additively contributed to the development of CAAs in patients with KD. A sex-stratified GWAS identified 6 male-specific (PDE1C, NOS3, DLG2, CPNE8, FUNDC1, and GABRQ) and 2 female-specific (SMAD3 and IL1RAPL1) CAA susceptibility loci in patients with KD.

Treatment of immunoglobulin-resistant kawasaki disease: a Bayesian network meta-analysis of different regimens.

This study aimed to gather evidence from clinical trials on the efficacy and safety of the available treatments for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) in children. This work adopted the Newcastle-Ottawa scale to analyse the quality of the enrolled articles. A network meta-analysis was performed using clinical trials that compared drugs used to treat IVIG-resistant KD. Aggregate Data Drug Information System software v.1.16.5 was employed to analyse whether infliximab, second IVIG infusions, and intravenous pulse methylprednisolone (IVMP) were safe and effective. Ten studies, involving 704 patients with IVIG-resistant KD, were identified and analysed. Overall, infliximab exhibited remarkable antipyretic activity compared with the second IVIG infusions (2.46, 1.00-6.94). According to the drug rank, infliximab was the best option against IVIG-resistant KD. Regarding adverse effects, the infliximab group was more prone to hepatomegaly. A second IVIG infusion was more likely to result in haemolytic anaemia. IVMP treatment was more susceptible to bradycardia, hyperglycaemia, hypertension, and hypothermia. In addition, infliximab, IVMP, and the second IVIG infusions showed no significant differences in the risk of developing a coronary artery aneurysm (CAA). Infliximab was the best option against IVIG-resistant KD, and IVMP, infliximab, and second IVIG infusions have not significant differences of prevent CAA in patients with IVIG-resistant KD. Identifier: https://osf.io/3894y.

Coronary changes and cardiac events in children diagnosed with kawasaki disease without initial coronary aneurysm: A multicenter retrospective cohort study.

Kawasaki disease (KD) is a systemic vasculitis affecting young children, which may lead to coronary artery aneurysm (CAA). The optimal timing of serial echocardiography in patients with uncomplicated KD is debated. To assess changes in coronary artery Z-scores from the initial diagnosis, two weeks, eight weeks, and one year following diagnosis and adverse cardiac events in children diagnosed with KD without initial CAA. Retrospective chart reviews of four referral centers in Thailand were conducted of all children who were diagnosed with KD without initial CAA (coronary artery Z-score < 2.5) between 2017 and 2020. Eligibility criteria included the absence of congenital heart disease and patients with available echocardiographic evaluations at baseline and at eight weeks of illness. The two-week and one-year echocardiographies were reported. Adverse cardiac events at one year from diagnosis were explored. The primary outcome was a maximal coronary Z-score on the follow-up echocardiography at eight weeks and one year. Of 200 patients diagnosed with KD, 144 patients (72%) did not have CAA. A total of 110 patients were included in the study. The median age was 23 months (IQR, 2-39 months) and 60% were male. Fifty patients (45.5%) had incomplete KD, and four (3.6%) received a second intravenous immunoglobulin treatment. Of 110 patients, 26 patients (23.6%) had coronary ectasia (Z-score of 2-2.49) on their initial echocardiographic examination. Sixty-four patients were evaluated in two-week echocardiographic studies, which showed four new small CAAs and five coronary ectasia. At 8 weeks, 110 patients had undergone complete echocardiographic studies. No patient had residual CAAs. Only one patient had persistent coronary ectasia that regressed to normal within one year. At one-year follow-up (n = 90), no cardiac events were reported. New CAA in-patients with KD who had no previous CAA in their initial echocardiography are rare. In addition, patients who had normal echocardiographic follow-up at two weeks or eight weeks mostly continued to be normal at one year. The optimal timing of the echocardiographic follow-up should be at two to eight weeks in patients without initial CAA, who still have a coronary artery Z-score < 2 at the second echocardiography.Trial registration: TCTR20210603001.

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.

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.

Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant

BackgroundThere are few data on the prevalence and clinical consequences of coronary artery aneurysms (CAAs) in adult patients with Marfan syndrome (MFS). MethodsWe performed a retrospective cohort study of 109 patients with pathogenic variants in the FBN1 gene. Diameters of the left main coronary artery (LMCA) and right- coronary artery (RCA) were measured by computed tomography angiography. ResultsThe overall prevalence of CAA was 46%. The prevalence rates of CAA were 18% and 68% in patients with a native aortic root (group 1) and patients with previous aortic-root replacement (group 2), respectively. Previous aortic dissection or aortic intervention, longer time from aortic-root replacement, higher systemic score, significant mitral valve involvement, and diffuse aortic disease were correlated with CAA. During a mean follow-up of 8.5 ± 7.6 years, 4 patients developed pseudoaneurysms of the coronary anastomoses, requiring surgery. ConclusionsCAAs are common in adult patients with MFS and are associated with a more severe aortic phenotype and a longer follow-up after aortic-root replacement. Our study demonstrates that coronary artery size should be regularly followed, mostly after aortic-root replacement and in patients with severe aortic phenotypes. Large multicentre studies are warranted to elucidate the most appropriate surveillance plan.

Delayed Coronary Artery Aneurysm in Patients With Kawasaki Disease

Delayed Coronary Artery Aneurysm in Patients With Kawasaki Disease

Delayed Development of Coronary Artery Aneurysm in Patients with Kawasaki Disease Who Were Clinically Responsive to Immunoglobulin

To clarify the frequency and characteristics of discrepant outcomes of intravenous immunoglobulin (IVIG) between fever and coronary artery aneurysms (CAAs) in patients with Kawasaki disease. This study included 325 patients who responded to oral aspirin and IVIG alone. The main outcome was CAA 4weeks after disease onset. CAA was defined as ≥2.5 of maximum z score (Zmax) representing the highest value of 4 coronary artery branches. Immunoglobulin dosage and sequential changes in Zmax were reviewed to investigate the effects on fever and timing of CAA development. Logistic regression analyses with receiver operating characteristic curves using clinical and laboratory variables including the initial Zmax were performed to identify predictors of CAA at 4weeks. CAAs were either persistent or appeared de novo 4weeks after diagnosis in 13 of 325 patients who responded to a single or repeated IVIG. Four single-dose IVIG-responders developed CAA although they had pretreatment Zmax of <2.0. The 2 single-dose IVIG responders with the greatest pretreatment Zmax (>4.5) developed persistent CAA. Receiver operating characteristic analysis demonstrated Zmax of 2.57 as the cut-off for predicting CAA. Multivariable analyses identified >2.5 Zmax (OR 9.08, 95% CI 1.26-65.3, P=.028, 50% sensitivity, 91% specificity) as the sole risk factor for CAA at 4weeks in single-dose IVIG responders. Delayed development and persistence of CAA in single-dose IVIG responders indicate that some factors other than those responsible for systemic inflammation may contribute to vasculitis in CAA. Baseline Zmax 2.5 aids in predicting CAAs.

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.

Therapeutic dilemma: Giant left main coronary artery aneurysm in patient with untreated Kawasaki disease

Therapeutic dilemma: Giant left main coronary artery aneurysm in patient with untreated Kawasaki disease

Identification of the TIFAB Gene as a Susceptibility Locus for Coronary Artery Aneurysm in Patients with Kawasaki Disease.

Kawasaki disease (KD) is a self-limiting systemic vasculitis of unknown etiology. KD is often complicated by coronary artery aneurysms (CAAs), which develop in about 20-25% of untreated children and 3-5% of children treated with intravenous immunoglobulin therapy. To identify the risk loci for CAA susceptibility in patients with KD, we performed a genome-wide association study (GWAS) using our previous Illumina HumanOmni1-Quad BeadChip data (296 KD patients) and a new replication study in an independent sample set (713 KD patients) by grouping KD patients without CAA (control) versus KD patients with extremely large aneurysms (diameter ≥ 5mm) (case). Among 44 candidate single -nucleotide polymorphisms (SNPs) selected from the initial GWAS data (33 cases vs. 215 controls), a SNP (rs899162) located 7kb upstream of the TIFAB gene on chromosome five was replicated in an independent sample (12 cases vs. 532 controls). In the combined analysis (45 cases vs. 747 controls), the SNP (rs899162) showed a highly significant association with CAA formation (diameter ≥ 5mm) in patients with KD (odds ratio = 3.20, 95% confidence interval = 2.02-5.05, Pcombined = 1.95 × 10-7). These results indicate that the TIFAB gene may act as a CAA susceptibility locus in patients with KD.

Effective infliximab therapy for the early regression of coronary artery aneurysm in Kawasaki disease

BackgroundThere is limited information available regarding the role of infliximab (IFX) following the acute phase of Kawasaki disease (KD). We aimed to evaluate whether IFX is associated with coronary artery aneurysm (CAA) regression. MethodsBetween 2005 and 2016, we identified 971 consecutive patients with KD from 3 tertiary institutions, and 49 (5%) with CAAs were enrolled in our study. Patients were divided into 2 groups: 27 who received IFX and 22 who did not. The persistence rate of CAAs was compared between the groups. ResultsAge, sex, and duration of the febrile period did not significantly differ between the groups. The maximum value of C-reactive protein was higher in the IFX- than in the non-IFX group. The maximum z-score of CAAs did not differ between the groups. The 2-, 4- and 6-year cumulative persistence rate of CAA was 24%, 24% and 24% in IFX-group, whereas 67%, 52% and 33% in non-IFX group, respectively (P = 0.03). The median duration of CAA regression was 1.1 vs. 4.6 years. Among those who developed medium- or large-sized CAAs, the 2-, 4- and 6-year cumulative persistence rate of CAA was 33%, 33% and 33% in IFX group, whereas 77%, 51% and 48% in non-IFX group, respectively (P = 0.047). Multivariate logistic regression analysis indicated that the maximum z-score (hazard ratio 0.72, p < 0.001) and response to IFX (hazard ratio 4.56, p = 0.017) were independently related to regression. ConclusionIFX therapy was observed to be effective for the early improvement of CAAs in patients with intravenous immunoglobulin-resistant KD.

Predictors of intravenous immunoglobulin resistance and coronary artery aneurysm in patients with Kawasaki disease

Background: Patients with Kawasaki disease (KD) who have intravenous immunoglobulin (IVIG) resistance are at increased risk for development of coronary artery abnormalities. Although in Japan several risk scoring systems are able to predict patients with IVIG-resistant (KD), they do not accurately predict non-responders in other regions.Aim: The objectives of this study were to determine the predictors of IVIG resistance and coronary artery aneurysm (CAA) and to develop risk scoring systems for predicting IVIG-resistant KD in the Thai population.Methods: A total of 217 patients with KD between 2004 and 2014 were retrospectively reviewed. All patients including 116 with complete KD and 101 with incomplete KD were diagnosed and treated with 2 g/kg IVIG.Results: Twenty-six patients (85% male) with IVIG-resistant KD had a reduced platelet count and increased neutrophil-to-lymphocyte ratio compared with those with an IVIG response. Fifty-five patients with CAA eight weeks after diagnosis had a longer duration of fever (≥8 days) and increased platelet count (≥550 × 109/L) than those with non-CAA. Based on analysis by multivariate logistic regression, haematocrit ≤30%, platelet count ≤300 × 109/L, aspartate aminotransferase ≥40 U/L and neutrophil-to-lymphocyte ratio ≥3.2 were predictors of IVIG resistance. The new scoring system using these significant factors had a sensitivity of 80.8% and a specificity of 66.8% in identifying patients with IVIG resistance. Japanese scoring systems had low sensitivity and specificity.Conclusions: KD patients with reduced mean haemoglobin, increased AST level, increased neutrophil-to-lymphocyte ratio and reduced platelet count should be considered for conjunctive therapy such as a corticosteroid in combination with standard treatment. Duration of fever ≥8 days and platelet count ≥550 × 109/L were predictors of CAA. To prevent cardiovascular complications, patients should be treated promptly after KD has been diagnosed.

Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events

Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA). To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA. This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011. The time-dependent occurrence of CE, including thrombosis, stenosis, obstruction, acute ischemic events, and coronary interventions, was analyzed for small (z score, <5), medium (z score, ≥5 to <10; actual internal diameter, <8 mm), and large (z score, ≥10 or ≥8 mm) CAA by the Kaplan-Meier method. The Cox proportional hazard regression model was used to identify risk factors for CE after adjusting for age, sex, size, morphology, number of CAA, resistance to initial intravenous immunoglobulin (IVIG) therapy, and antithrombotic medications. Of 1006 patients, 714 (71%) were male, 341 (34%) received a diagnosis before age 1 year, 501 (50%) received a diagnosis between age 1 and 5 years, and 157 (16%) received a diagnosis at age 5 years or older. The 10-year event-free survival rate for CE was 100%, 94%, and 52% in men (P < .001) and 100%, 100%, and 75% in women (P < .001) for small, medium, and large CAA, respectively. The CE-free rate was 100%, 96%, and 79% in patients who were not resistant to IVIG therapy (P < .001) and 100%, 96%, and 51% in patients who were resistant to IVIG therapy (P < .001), respectively. Cox regression analysis revealed that large CAA (hazard ratio, 8.9; 95% CI, 5.1-15.4), male sex (hazard ratio, 2.8; 95% CI, 1.7-4.8), and resistance to IVIG therapy (hazard ratio, 2.2; 95% CI, 1.4-3.6) were significantly associated with CE. Classification using the internal diameter z score is useful for assessing the severity of CAA in relation to the time-dependent occurrence of CE and associated factors in patients with KD. Careful management of CE is necessary for all patients with KD with CAA, especially men and IVIG-resistant patients with a large CAA.

Stenotic Lesions and the Maximum Diameter of Coronary Artery Aneurysms in Kawasaki Disease

To determine the prevalence of subsequent stenotic lesions based on the maximum diameter of the largest coronary artery aneurysm in patients with Kawasaki disease and the threshold value of coronary artery diameter associated with risk of developing stenotic lesion. There were 214 patients (160 males) who had at least 1 aneurysm in a selective coronary angiogram (CAG) done <100 days after the onset of Kawasaki disease were studied. We measured the maximal coronary artery aneurysm diameter in 3 major branches in the initial CAGs. Branches were classified into 3 groups according to their maximal coronary artery aneurysm diameter: large, ≥8.0 mm; medium, ≥6.0 mm but <8.0 mm; and small, <6.0 mm. Subsequent CAGs were performed in the late follow-up period. We investigated the stenotic lesion in the follow-up CAGs, and evaluated the prevalence of stenotic lesion in each group based on body surface area (BSA) by the Kaplan-Meier method. Localized stenosis of ≥25% and complete occlusion were included as stenotic lesion in this study. We also determined the cutoff point for stenotic lesion. The median interval from the initial CAGs to the latest CAG was 8 years, with a maximum of 32 years. For a BSA of <0.50 m2, the 20-year prevalence of large and medium stenotic lesions was 78% (n = 62; 95% CI, 63-89) and 81% (n = 40; 95% CI, 63-89), respectively. For a BSA of ≥0.50 m2, large and medium stenotic lesions were 82% (n = 75; 95% CI, 67-91) and 40% (n = 56; 95% CI, 20-64), respectively (P < .0001). The cutoff points of the coronary artery diameter within the first 100 days after the onset of Kawasaki disease leading to a stenotic lesion in the late period, were a diameter of ≥6.1 mm with a BSA of <0.50 m2 and a diameter of ≥8.0 mm with a BSA of ≥0.50 m2. Those cutoff points would have corresponded with a Z score of at least 10 on 2-dimensional echocardiography. Careful follow-up and antithrombotic therapy should be provided to patients who meet these criteria.

Computed tomography predict regression of coronary artery aneurysm in patients with Kawasaki disease.

The study evaluated possible factors influencing the regression of coronary artery aneurysm (CAA) in patients with Kawasaki disease (KD) through electrocardiographically gated cardiac computed tomography (CT). 18 patients with KD exhibited CAAs in at least 2 CT examinations conducted from December 2004 to September 2015, and 37 aneurysms were observed. Every aneurysm was corrected through the descending aorta at the origin level of the left main coronary artery under a normal distribution and measured under a fixed window level. These aneurysms were divided into 2 groups according to regression. Clinical symptoms, laboratory data, and imaging characteristics of both groups were analyzed. All the aneurysms of 4 patients decreased in size, and totally, 14 aneurysms (37%) regressed. CAA regression tends to occur early after disease onset. No significant differences were observed in sex, aneurysm location, and the distance to the orifice between the 2 groups. The aneurysms with no calcification (p=0.012), smaller diameter (p=0.004), younger disease onset age (p=0.048), and ectatic shape (p<0.001) were more likely to regress according to univariate analysis. Receiver operating characteristic analysis revealed that the possible cut-off point of the maximal diameter to yield the highest sensitivity (91.3%) and specificity (92.9%) to predict CAA regression was 5.6mm. Calcified CAAs in patients with KD was less likely to regress. The aneurysm size and shape as well as disease onset age were possible factors influencing regression.

Surgery for an Isolated Huge Coronary Artery Aneurysm in a Patient with Behcet’s Disease: Case Report and Review of the Literature

Coronary artery aneurysms in patients with Behcet’s disease are rare. Giant aneurysms defined as greater than 2 cm are exceptional. We are reporting a rare case of a 33-year-old man with Behcet’s disease, admitted for chest pain, whose coronary angiogram revealed a huge aneurysm involving the proximal segment of proximal left anterior descending artery followed by a severe stenosis. The aneurysm was successfully resected and an arterial coronary bypass was performed. The patient was asymptomatic in the postoperative period.

Abstract 147: Coronary artery aneurysms on day 4 after onset of Kawasaki Disease: A Case report

Background: Coronary artery aneurysm (CAA) in patients with Kawasaki disease (KD) almost always occurs after day 8, and the most early detection report of CAA was in an autopsy case on day 6 after onset. The possibility of CAA formation prior to day 6 has not been documented. Case report: A 10-month-old Japanese boy presented with a 4-day fever, conjunctival injection, erythema of the lips, erythema of the palms and soles, erythematous rush, and induration at a Bacillus Calmette-Guerin inoculation site. On day 4, echocardiography revealed coronary artery dilatation (right coronary artery [RCA], 4.6 mm; left main trunk [LMT], 4.6 mm; left anterior descending [LAD] artery, 3.4 mm). Laboratory data were as follows: white blood cell count, 19,400/μL; C-reactive protein, 15.2 mg/dL; albumin, 3.2 g/dL; high-density lipoprotein cholesterol, 20 mg/dL, and erythrocyte sedimentation rate, 48 mm/h. He was diagnosed with KD and treated with oral aspirin 30 mg/kg/day and intravenous immunoglobulin (IVIG) 2 g/kg. His fever subsided the next day. On day 17, periungual peeling of the fingers and toes was noted. His fever recurred and he treated with additional IVIG 1 g/kg, after which his fever subsided again. On day 20, the aneurysms had not increased in size (RCA, 3.3 mm; LMT, 3.6 mm; LAD, 2.4 mm). On day 44, although echocardiography showed a mild coronary artery dilatation, a coronary angiogram showed no apparent abnormality. He had no obvious fever or symptoms of KD prior to this course of KD. This case may help clarify the pathophysiology of CAA in patients with KD.

Guideline-Concordant Treatment of Kawasaki Disease With Immunoglobulin and Aspirin and the Incidence of Coronary Artery Aneurysm.

Current guidelines for Kawasaki disease (KD) recommend intravenous immunoglobulin infusion and echocardiography. However, no previous studies have evaluated the relationship between the treating hospital's concordance with guidelines and the prevention of coronary artery aneurysm in patients with KD. KD patients between 2010 and 2013 were identified in a Japanese national inpatient database. Guideline concordance was defined as the proportions of patients who received echocardiography as well as treatment with both immunoglobulin and aspirin, which were divided into quartiles. Multivariable logistic regression analysis was conducted to examine the association between the guideline concordance and the occurrence of coronary artery aneurysm with adjustment for patient backgrounds. In sum, 20 156 patients with KD were identified. The very high (>83.3%) concordance group had a lower rate of coronary artery aneurysm than the very low (<59.0%) concordance group (4.9% vs 9.9%; odds ratio, 0.45; 95% CI, 0.27 to 0.74; P = .002). All patients with KD should be treated according to the guidelines.

Clinical Characteristics of Kawasaki Syndrome and the Risk Factors for Coronary Artery Lesions in China

Kawasaki syndrome (KS) is the leading cause of acquired heart disease in childhood in developed countries. This study was designed to identify the clinical characteristics of a large cohort of KS in China and explore the risk factors for coronary artery lesions. Clinical records of 1370 patients with acute KS were retrospectively reviewed. The clinical features of different age groups were analyzed, and a multivariate logistic regression analysis was performed to identify the risk factors for coronary artery lesions caused by KS. The prevalence of redness at a Bacille Calmette-Guèrin inoculation site was greatest in infants younger than 6 months (18.4%), whereas cervical lymphadenopathy was more frequent in patients older than 60 months (61.5%). Age, sex, therapeutic time, intravenous immunoglobulin dose, platelet count and erythrocyte sedimentation rate were risk factors for coronary artery lesions (P < 0.05). A total fever duration >10 days was a risk factor for coronary artery aneurysms in patients with coronary artery lesions (P < 0.05). KS occurs more frequently in children younger than 5 years, in boys and during the summer months. Redness at a Bacille Calmette-Guèrin inoculation site signals the diagnosis of incomplete KS in infants and young children. Male gender, younger age, intravenous immunoglobulin dose, delayed administration (>10 days), high platelet level and elevated erythrocyte sedimentation rate are predictive for coronary artery lesions, and total fever duration (>10 days) is predictive for coronary artery aneurysms in patients with coronary artery lesions.