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    To describe the fetal echocardiographic features of a double aortic arch (DAA) and secondly, to assess the performance of these features to differentiate between a right aortic arch with left duct (RAA-LD) in a blinded cohort of vascular rings. Review of records to identify surgically confirmed cases of DAA diagnosed prenatally from 2014 to 2018 (cohort-A). Prenatal echocardiograms were reviewed and the segments of the aortic arches anterior and posterior to the trachea, aortic isthmuses and the presence/absence of the Z-sign were described. The utility of these markers were assessed in a separate cohort (B) of fetuses with surgically confirmed cases of DAA or RAA-LD. Cohort-A comprised 34 cases with DAA; there was a dominant RAA in 32/34 (94%) and balanced left aortic arch (LAA) and RAA in two cases. The proximal LAA was seen in 29/34 (85%), distal LAA in 15/34 (44%) and the LAA aortic isthmus in 4/34 (12%). The "Z" configuration was present in 29/34 (85%) cases. The most predictive marker for DAA in cohort-B was the Z-sign (sensitivity: 100%, specificity: 81%). The "Z" sign is a useful differentiator between RAA-LD and DAA. The absence of visualization of the left aortic isthmus does not preclude the presence of a DAA. © 2022 John Wiley & Sons Ltd.


    Milou P M Van Poppel, Vita Zidere, John M Simpson, Trisha V Vigneswaran. Fetal echocardiographic markers to differentiate between a right and double aortic arch. Prenatal diagnosis. 2022 Apr;42(4):419-427

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    PMID: 35060138

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