Examining the Utility of Coronary Artery Lack of Tapering and Perivascular Brightness in Incomplete Kawasaki Disease
In 2017, the AHA published revised guidelines for the diagnosis of Kawasaki disease (KD). In the absence of compelling data supporting or refuting the utility of lack of tapering (LT) and perivascular brightness (PB), expert panel consensus removed LT and PB from consideration. We hypothesize that LT and PB are unreliable, subjective findings, non-specific to KD, which can be seen in systemic febrile illnesses without KD and in normal controls.
We performed a single-center retrospective study from 1/2008 to 12/2016. De-identified coronary artery (CA) echocardiographic clips from patients 0–10 years old were interpreted blindly by six pediatric cardiologists. Subjects were grouped as follows: (1) healthy: afebrile with benign murmur, (2) KD: IVIG treatment, 4–5 clinical criteria at presentation, (3) incomplete KD (iKD): IVIG, 1–3 clinical criteria, (4) Febrile: ≥3 days of fever, no IVIG, KD not suspected. The presence or absence of LT and PB was recorded. Inter-rater and intra-rater reliabilities were analyzed using intra-class correlation coefficient, Fleiss’ Kappa and Cohen’s Kappa coefficients.
We interpreted 117 echocardiograms from healthy (27), KD (30), iKD (32), and febrile (28) subjects. Analysis showed moderate agreement in CA z score measurements. LT and PB were observed by most readers in control groups. LT exhibited fair inter-reader agreement (reliability coefficient 0.36) and PB slight inter-reader agreement (reliability coefficient 0.13). Intra-rater reliability was inconsistent for both parameters.
LT and PB are subjective, poorly reproducible features that can be seen in febrile patients without KD and in healthy children.
KeywordsIncomplete Kawasaki disease Kawasaki disease Lack of tapering Perivascular brightness Coronary vessel disease Acquired heart disease
Incomplete Kawasaki disease
Lack of tapering
Left main coronary artery
Left anterior descending artery
Right coronary artery
Body surface area
Compliance with Ethical Standards
Conflict of interest
All authors declare that they have no conflict of interest relevant to this article to disclose.
This article does not contain any studies with human participants or animals performed by any of the authors.
A waiver of informed consent was obtained from the Northwell Health institutional review board.
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