Abstract 28: African American Race is Not Protective Against Coronary Artery Involvement in Kawasaki Disease

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Emily P Williams ◽  
Michael S Kelleman ◽  
William T Mahle

It has been previously reported that African American race may be protective against coronary artery aneurysm development in Kawasaki Disease (KD). We aimed to test this with our own cohort of KD patients from a large pediatric cardiology practice. Data from 250 subjects diagnosed with KD and followed as outpatients with surveillance echocardiography over a two-year period were analyzed. Twelve patients were excluded due to incomplete records or an unconfirmed diagnosis. Race designated by parent was recorded. Charts were reviewed for any coronary involvement (ectasia or aneurysm) and coronary Z-score greater than 2.5 at the time of diagnosis and at subsequent follow-up visits. Odds rations were calculated comparing each racial group to others for any coronary involvement and for coronary Z-score > 2.5. Of 238 included patients, 44.5% were African American, 37.4% were non-Hispanic white, 10.5% were Hispanic, and 7.6% identified with other racial designations. Approximately 21.9% of African American patients had any coronary involvement and 9.5% had a coronary Z-score > 2.5. Approximately 21.4% of non-Hispanic whites had any coronary involvement and 13.5% of non-Hispanic whites had a coronary Z-score > 2.5. Twenty-eight percent of Hispanic patients had any coronary involvement and 12% had a coronary Z-score > 2.5%. Of patients that identified with other racial designations, 38.9% had coronary involvement and 22.2% had a coronary Z-score > 2.5. No statistically significant odds ratios were identified. Relative to reference group (non-Hispanic whites) African American patients had nearly identical rates of 1) any coronary involvement, or 2) coronary Z-score > 2.5. KD occurs commonly in African-American children. Given equal risk for late coronary sequelae vigilance and strict adherence to consensus guidelines is essential.

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Christina Ronai ◽  
Akiko Hamaoka-Okamoto ◽  
Christian Stopp ◽  
Jane W Newburger ◽  
Kevin Friedman

Background: Coronary artery (CA) z-scores are commonly used for clinical decisions in Kawasaki disease (KD). We evaluated reliability in CA measurement, reproducibility of z-score calculation, and frequency with which different z-score formulas lead to divergent management strategies. Methods: We randomly selected 21 KD patients (pts) with ≥1 CA z-score 1.5-3 and all KD pts with ≥ 1 CA z-score 7-14 (n=20). Two echocardiographers measured LMCA, LAD and RCA. Inter- and intraobserver reliability were calculated. T-tests were used to compare CA z-score using 3 commonly used formulas (Boston, DC and Montreal). Results: Median age at KD echo was 1.2 y (0.2-11.5 y). Interobserver reliability was high for LAD (intraclass correlation [ICC] 0.970) and RCA (ICC 0.943) and lower for LMCA (ICC 0.725). Intraobserver reliability was also high for LAD and RCA (ICC 0.991 and 0.999) and lower for LMCA (ICC 0.946). Z-scores for the 3 formulas were similar at smaller CA size, i.e., z < 3, but varied markedly at larger CA dimensions (Figure). Z-scores for the same CA dimension calculated by each of the 3 formulas resulted in disparate classification of normal vs. mild dilation in 7/21 (22%) pts, and different guidance for anticoagulation based on CA z ≥10 in 10/20 (50%) pts. Conclusion: Although CA measurements have high inter- and intraobserver agreement, CA z-scores vary dramatically based on the z-score formula, particularly at larger CA dimensions. Discrepancies in CA z-score between calculators impacts not only the distinction between normal and mild dilatation, but most importantly, the recommendation of anticoagulation for pts with larger CA dimensions.


2016 ◽  
Vol 29 (2) ◽  
pp. 150-157 ◽  
Author(s):  
Christina Ronai ◽  
Akiko Hamaoka-Okamoto ◽  
Annette L. Baker ◽  
Sarah D. de Ferranti ◽  
Steven D. Colan ◽  
...  

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Naoya Fukushima ◽  
Masaru Miura ◽  
Toru Kobayashi ◽  
Shigeto Fuse ◽  
Tsutomu Saji ◽  
...  

Background: The standard values of normal coronary artery internal diameters in Japanese children have been recently established, making it possible to calculate Z-scores based on body surface area. The aim of this study was to clarify the appropriate cut-off points of coronary artery aneurysm (CAA) Z-scores to predict coronary events such as stenosis, obstruction, and thrombosis in patients with Kawasaki disease (KD). Methods: In this multicenter retrospective study, we investigated height, weight, CAA diameters measured by echocardiography in acute phase KD, and coronary events in CAA patients with KD (age 18 years or younger) who had coronary angiography from 1992 to 2011. Results: Interim analysis was performed on data of the 928 patients recruited from 45 institutions. Body surface area (calculated from height and weight) and CAA diameters were available in 702, 680, and 539 cases of right coronary artery (RCA), left main trunk (LMT), left anterior descending artery (LAD), respectively. Coronary events occurred in 62 RCA cases (8.8%), 8 LMT cases (1.2%), and 45 LAD cases (8.3%) . Areas under the ROC curves to predict coronary events were similar for actual diameter, Z-score, and the ratio of actual diameter to that showing a Z-score of zero in each segment. The cut-off points for the actual diameter, Z-score, and ratio which yielding the highest sensitivity plus specificity were 6.3 mm, 9.6, and 3.9 times for RCA; 7.4 mm, 11.1, and 2.8 times for LMT; and 5.3 mm, 8.9, and 3.5 times for LAD. Conclusions: We identified cut-off Z-scores for CAA diameters useful for coronary events prediction. Attention should be paid to coronary events when the Z-score for CAA diameter is over 10.


2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Yuji Moritoh ◽  
Masahiro Kamada ◽  
Shinsaku Matsumoto ◽  
Koji Kido

Abstract Background Ruptured coronary artery aneurysm is rare, but the most serious complications of an acute phase of Kawasaki disease (KD) with giant coronary artery aneurysm (GCAA). Progressive or super GCAA, which rapidly dilates and continue to increase over a diameter of 10 mm, are more susceptible to rupture. Case summary We report the case of a 6-year-old boy with KD who had multiple super GCAAs with a high risk of GCAA rupture. On admission to our hospital, he presented with fever, chest pain, and Stage II hypertension. Echocardiographic Z-scores adjusted for body surface area were used for measurements. The coronary artery diameter of segment 1 was 24.3 mm with a Z-score of 20.8; the diameter of segment 3 was 24.4 mm; the diameter of the left anterior descending branch was 32.6 mm with a Z-score of 20.1. The super GCAAs showed a tendency to expand compared to the latest echocardiography, and thrombus formation was observed in the super GCAA of segment 3. The patient was treated with anti-inflammatory therapy, antithrombotic therapy, and antihypertensive therapy with continuous arterial pressure monitoring with the goal of not exceeding the 5th percentile of the normal standard during the period when there was a risk of progressive coronary aneurysm expansion. He was discharged without any neurological complications. Discussion We speculated that the patient's hypertension was the cause of an expanding coronary artery aneurysm. In conclusion, KD patients with super GCAA may benefit from aggressive blood pressure control with continuous arterial pressure monitoring.


Author(s):  
Takayuki Suzuki ◽  
Nobuyuki Kakimoto ◽  
Tomoya Tsuchihashi ◽  
Tomohiro Suenaga ◽  
Takashi Takeuchi ◽  
...  

2019 ◽  
Vol 29 (06) ◽  
pp. 828-832 ◽  
Author(s):  
Laxmi V. Ghimire ◽  
Fu-Sheng Chou ◽  
Narayan B. Mahotra ◽  
Sharan P. Sharma

AbstractBackground:Kawasaki disease is an acute vasculitis of childhood and is the leading cause of acquired heart disease in the developed countries.Methods:Data from hospital discharge records were obtained from the National Kids Inpatient Database for years 2009 and 2012. Hospitalisations by months, hospital regions, timing of admission, insurance types, and ethnicity were analysed. Length of stay and total charges were also analysed.Results:There were 10,486 cases of Kawasaki disease from 12,678,005 children hospitalisation. Kawasaki disease was more common between 0 and 5 years old, in male, and in Asian. The January–March quarter had the highest rate compared to the lowest in the July–September quarter (OR=1.62, p &lt; 0.001). Admissions on the weekend had longer length of stay [4.1 days (95 % CI: 3.97–4.31)] as compared to admissions on a weekday [3.72 days (95 % CI: 3.64–3.80), p &lt; 0.001]. Blacks had the longest length of stay and whites had the shortest [4.33 days (95 % CI: 4.12–4.54 days) versus 3.60 days (95 % CI: 3.48–3.72 days), p &lt; 0.001]. Coronary artery aneurysm was identified in 2.7 % of all patients with Kawasaki disease. Children with coronary artery aneurysm were hospitalised longer and had higher hospital charge. Age, admission during weekend, and the presence of coronary artery aneurysm had significant effect on the length of stay.Conclusions:This report provides the most updated epidemiological information on Kawasaki disease hospitalisation. Age, admissions during weekend, and the presence of coronary artery aneurysm are significant contributors to the length of stay.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Kyu Yeun Kim ◽  
Mo Kyung Jung ◽  
Yoon-Sun Bae ◽  
Woohyuk Ji ◽  
Dongjik Shin ◽  
...  

Kawasaki disease (KD) is an acute systemic vasculitis syndrome that predominantly affects children younger than 5 years of age, and may causes serious, sometimes life-threatening, cardiac sequela associated with coronary artery aneurysm (CAA). To identify genetic variants that confers a highly increased risk of coronary artery aneurysm-related in Kawasaki disease. In this study, we carried out genome-wide association study (GWAS) in a Korean children population including 102 CAA-related KD cases and 126 controls. Fifteen genetic loci were found to be significantly correlated with KD risk (P<1.0X10(-7)). Our case-control study revealed that rs4236089 C allele in chloride intracellular channel 5 (CLIC5) gene at 6p21.1 was significantly associated with KD patients with CAA (odds ratio (OR)=4.6, P=7.53X10(-7)). These findings suggest that the CLIC5 gene may play a crucial role in CAA development pathway of KD.


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