scholarly journals Value of serial echocardiography in diagnosing Kawasaki’s disease

Author(s):  
Maria Hörl ◽  
Holger Michel ◽  
Stephan Döring ◽  
Markus-Johann Dechant ◽  
Florian Zeman ◽  
...  

Abstract 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 < 0.05). 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.

2014 ◽  
Vol 9 (4) ◽  
pp. 30-35
Author(s):  
S Datta ◽  
S Maiti ◽  
G Das ◽  
A Chatterjee ◽  
P Ghosh

Background The diagnosis of classical Kawasaki Disease was based on clinical criteria. The conventional criteria is particularly useful in preventing over diagnosis, but at the same time it may result in failure to recognize the incomplete form of Kawasaki Disease. Objective To suspect incomplete Kawasaki Disease, because early diagnosis and proper treatment may reduce substantial risk of developing coronary artery abnormality which is one of the leading causes of acquired heart disease in children. Method Nine cases of incomplete Kawasaki Disease were diagnosed over a period of one year. The diagnosis of incomplete Kawasaki Disease was based on fever for five days with less than four classical clinical features and cardiac abnormality detected by 2D- echocardiography. A repeat echocardiography was done after 6 weeks of onset of illness. The patients were treated with Intravenous Immunoglobulin and/or aspirin. Result The mean age of the patients was 3.83 years and the mean duration of symptoms before diagnosis was 12.1 days. Apart from other criteria all of our patients had edema and extreme irritability. All the patients had abnormal echocardiographic finding. Five patients received only aspirin due to nonaffordability of Intravenous Immunoglobulin and four patients received both aspirin and Intravenous Immunoglobulin, but the outcome was excellent in all the cases. Conclusion Incomplete Kawasaki Disease can be diagnosed with more awareness and aspirin alone may be used as a second line therapy in case of non affordability of Intravenous Immunoglobulin. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 30-35 DOI: http://dx.doi.org/10.3126/jcmsn.v9i4.10234


2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Nathan Jamieson ◽  
Davinder Singh-Grewal

Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices.Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles.Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individual’s exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “incomplete” presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever.


2009 ◽  
Vol 136 (1) ◽  
pp. 27-32 ◽  
Author(s):  
Matthew A. Crystal ◽  
Cedric Manlhiot ◽  
Rae S.M. Yeung ◽  
Jeffrey F. Smallhorn ◽  
Brian W. McCrindle

Heart ◽  
1998 ◽  
Vol 80 (5) ◽  
pp. 532-532
Author(s):  
F M FRUHWALD ◽  
O LUHA ◽  
M SCHUMACHER ◽  
N WATZINGER ◽  
W KLEIN

2020 ◽  
Author(s):  
Hyo Soon An ◽  
Gi-Beom Kim ◽  
Mi Kyoung Song ◽  
Sang Yun Lee ◽  
Hye Won Kwon ◽  
...  

Abstract BackgroundThis study aimed to assess the occurrence of coronary artery lesions (CAL) in patients with Kawasaki disease (KD) according to serum C-reactive protein (CRP) levels. MethodsThis retrospective analysis was based on the nationwide survey of KD conducted in the Republic of Korea between 2015 and 2017. We enrolled 9131 patients and defined low (<3 mg/dL) and high (≥3 mg/dL) CRP groups. Demographic data, clinical characteristics, z-scores, and scores based on the Japanese criteria for CAL were compared between the two groups. Logistic regression analysis was used to identify CAL risk factors.ResultsThe low CRP group accounted for 23% of patients. A significant difference was observed for the mean age at diagnosis (high vs. low CRP, 34.4 ± 24.9 vs. 31.7 ± 24.8 months, p<0.001) and fever duration (high vs. low CRP, 6.6 ± 2.2 vs. 6.3 ± 2.5 days, p<0.001). A non-response to intravenous immunoglobulin treatment was found in 1377 patients (20.1%) and 225 patients (11.7%) in the high and low CRP groups, respectively (p<0.001). CAL were found in 12.9% and 18.3% of the high and low CRP patients, respectively (p<0.001), based on z-scores; and in 9.9% and 12.5%, respectively (p = 0.001), based on the Japanese criteria in the acute phase. The giant coronary artery aneurysm occurrence ratio was similar between groups (p=1.0).ConclusionsCAL occurred in patients with both high and low CRP. Therefore, patients with KD should be carefully monitored regardless of their CRP levels.


2021 ◽  
Vol 9 ◽  
Author(s):  
Ching-Ying Huang ◽  
Nan-Chang Chiu ◽  
Fu-Yuan Huang ◽  
Yen-Chun Chao ◽  
Hsin Chi

Background: Precisely predicting coronary artery aneurysms (CAAs) remains a clinical challenge. We aimed to investigate whether coronary dimensions adjusted for body surface area (Z scores) on baseline echocardiography and clinical variables before primary treatment could predict the presence of late CAAs.Methods: We conducted a retrospective study including children hospitalized for Kawasaki disease and received intravenous immunoglobulin within 10 days of illness. We defined late CAAs as a maximum Z score (Zmax) ≥2.5 of the left main, right, or left anterior descending coronary artery at 11–60 days of illness. Associations between late CAAs and clinical parameters and baseline maximum Z scores were analyzed.Results: Among the 314 included children, 31 (9.9%) had late CAAs. Male, higher C-reactive protein, and higher baseline Zmax were risk factors of late CAAs. Late CAAs were significantly associated with baseline Zmax ≥2.0 vs. &lt;2.0 (25 [32.5%] vs. 6 [2.5%], P &lt; 0.001). The odds ratio for late CAAs among the patients with baseline Zmax ≥2.0 vs. &lt;2.0 was 18.5 (95% confidence interval, 7.23 to 47.41, P &lt; 0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of baseline Zmax ≥2.0 for the presence of later CAAs were 80.6, 81.6, 32.5, and 97.5%, respectively.Conclusions: Findings from this study suggest that Zmax ≥2.0 of coronary arteries on baseline echocardiography may be used to predict children at a high risk of late CAAs and allow for targeted early intensification therapy.


2021 ◽  
pp. 1-8
Author(s):  
Raymond P. Lorenzoni ◽  
Noah Elkins ◽  
Morgan Quezada ◽  
Ellen J. Silver ◽  
Joseph Mahgerefteh ◽  
...  

Abstract 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.


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.


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