Clinical Investigation
Echocardiography in Children
Diagnosis of Coronary Artery Abnormalities in Patients with Kawasaki Disease According to Established Guidelines and Z Score Formulas

https://doi.org/10.1016/j.echo.2021.01.002Get rights and content

Highlights

  • Diagnostic differences of CAA and aneurysm subtypes were shown between guidelines.

  • More CAA patients were identified using the AHA criteria and a Z score cutoff of 2.0.

  • The prevalence of CAA varied among the various Z score formulas.

  • Misclassification was observed among Z score formulas as well as between guidelines.

  • Discrepancies in Z scores increased with increasing coronary artery diameters.

Background

The diagnosis of coronary artery abnormalities (CAA), including dilation and aneurysm, in patients with Kawasaki disease is paramount to treatment planning. CAA are defined using various standards, which makes diagnosis difficult. The aims of this study were to determine the variability of CAA prevalence according to existing guidelines and Z score formulas and to examine the discrepancies in widely used Z score formulas.

Methods

Using data from a Korean national survey on Kawasaki disease, 6,889 patients were included and analyzed. The overall prevalence of CAA and the prevalence for subgroups were compared on the basis of aneurysm severity, age, and body surface area. Finally, discrepancies among five Z score formulas were evaluated by comparing two of the formulas in pairs.

Results

According to the Japanese criteria, the prevalence of CAA was 18%. According to the American Heart Association criteria, the prevalence of dilation or aneurysm was about 21% to 42%, and that of aneurysm of the left anterior descending artery or right coronary artery was about 8% to 27%. The prevalence of CAA and that of left anterior descending or right coronary artery aneurysm was significantly different, with discrepancies between the Japanese and AHA Z score criteria, as well as among the five Z score formulas. Additionally, misclassification of aneurysm severity was observed for each criterion or Z score formula. There was significant variation among calculated Z scores. The more extreme the Z score values, the more discrepancy was observed.

Conclusions

Different guidelines and Z score formulas yield significantly different prevalence rates and classifications of CAA. In addition, more discrepancies were observed with higher Z score values. As CAA or aneurysm severity could be changed by guidelines or Z score formulas, they should be chosen carefully, and when a particular formula is chosen, consistency is needed.

Section snippets

Methods

We reviewed 12,926 patients diagnosed with KD who received intravenous immunoglobulin or other regimens from the data of the KD nationwide survey obtained from 98 hospitals in Korea from January 2012 to December 2014.

This survey assesses the epidemiologic features of KD by reviewing medical records, and it has been performed by the Korean Society of Kawasaki Disease every 3 years since 1991.17 The present survey collected demographic data, such as age, sex, province of residence, and date of

Results

Table 3 shows demographic data of the inclusion and exclusion groups. Men constituted 59% of the included patients. Besides sex, no statistical differences were seen between the two groups.

Among included patients, the diameters of the LMCA, LAD, RCA, and LCx were obtained in 6,754, 5,673, 6,709, and 246 cases, respectively, and their maximum diameters were 18.0, 13.3, 18.0, and 6.2 mm, respectively.

The prevalence rates and statistical differences in CAA according to the Japanese criteria and

Discussion

In this study, we found that the distinct guidelines and Z score formulas produced inconsistent CAA prevalence. In addition, when compared with one another, the five Z score formulas produced different CAA prevalence rates. We confirmed this difference by plotting all the calculated Z scores; the variation increased with the magnitude of the coronary Z scores.

Several studies have examined the diagnostic discrepancies between the JCS and AHA guidelines.7,8 In a study conducted by de Zorzi et al.,

Conclusion

The two guidelines showed a clear diagnostic difference. Specifically, more patients were identified as having coronary dilation or aneurysm using the AHA guidelines compared with those using the Japanese criteria. Additionally, the prevalence of CAA was significantly different, and we found misclassification of CAA or aneurysm subtype between each criterion or across the various Z score formulas. The prevalence of CAA was more significantly associated with BSA than with age. By plotting Z

Acknowledgment

We thank the members of the Korean Society of Kawasaki Disease for their assistance with data acquisition.

References (20)

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Conflicts of interest: None.

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