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Characterisation of computed tomography angiography findings in paediatric patients with heterotaxy

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Abstract

Background

Heterotaxy refers to the abnormal arrangement of organs across the left–right axis and is typically associated with complex cardiovascular malformations.

Objective

To characterise the range of cardiac and extracardiac CT angiography findings in children with heterotaxy using the latest nomenclature consensus and to compare the different types of isomerism.

Materials and methods

We retrospectively analysed the data of 64 consecutive paediatric patients referred to our tertiary paediatric cardiovascular centre who underwent CT angiography for the evaluation of known or suspected heterotaxy within a 52-month period.

Results

Right atrial isomerism was identified in 44 (69%) children, while left atrial isomerism was identified in 18 (28%) children. Atrial appendage anatomy and situs could not be determined in 2 children (3%). Associated heart defects included complete atrioventricular canal (CAVC) in 51 (80%) children, total anomalous pulmonary venous return in 43 (67%) and pulmonary atresia in 23 (36%). The bronchial branching pattern corresponded to atrial appendage morphology in all children except in the two in whom atrial appendage morphology could not be defined. In children with right atrial isomerism, the most common associated abnormalities were CAVC (n=41, 93%) and asplenia (n=34, 77%), while in those with left atrial isomerism, the most common associated abnormalities were polysplenia (n=17, 94%) and an interrupted inferior vena cava with azygos continuation (n=15, 83%).

Conclusion

CT angiography provides useful cardiovascular and extracardiac data on heterotaxy, which frequently involves a pattern of side-related findings but has great anatomical variability.

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Acknowledgements

Thank you to the team of the Department of Diagnostic Imaging at the Instituto Nacional de Salud del Niño San Borja, especially to technologists Victor Guerra, Veronica Megue and Eddin Saavedra, who generated the 3-D reconstructions used in this manuscript.

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Authors and Affiliations

  1. Department of Diagnostic Imaging, Instituto Nacional de Salud del Niño San Borja, Av. Javier Prado Este 3101, 15037, Lima, Peru

    Carlos F. Ugas Charcape, Larry R. Alpaca Rodriguez, Irma A. Matos Rojas, Claudia I. Lazarte Rantes, Melissa Valdez Quintana & Doris A. Katekaru Tokeshi

  2. Department of Medical Imaging/Radiology Nemours Children’s Health System/Nemours Children’s Hospital, University of Central Florida, College of Medicine, Orlando, FL, USA

    Monica Epelman

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  1. Carlos F. Ugas Charcape
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Correspondence to Carlos F. Ugas Charcape.

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Ugas Charcape, C.F., Alpaca Rodriguez, L.R., Matos Rojas, I.A. et al. Characterisation of computed tomography angiography findings in paediatric patients with heterotaxy. Pediatr Radiol 49, 1142–1151 (2019). https://doi.org/10.1007/s00247-019-04434-0

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