Background: Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established.

Objective: We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings.

Materials And Methods: We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course.

Results: CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively.

Conclusion: CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.

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http://dx.doi.org/10.1007/s00247-021-05011-0DOI Listing

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