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Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta

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Abstract

To report the prevalence of coarctation of the aorta (CoA) in fetuses with single left superior vena cava (SL-SVC) and to evaluate changes in echocardiographic measurements. Additionally, to report the prevalence of associated malformations. Retrospective observational study of fetuses diagnosed with SL-SVC between 2012 and 2021 at a tertiary fetal cardiology unit. In fetuses without intracardiac abnormalities, Z-scores of the ventricles, great arteries, and Doppler flow patterns are reported. We identified 47 fetuses with SL-SVC of which 8/47 (17%) had abnormal intracardiac anatomy. One fetus was lost to follow-up. Of those with normal intracardiac anatomy and postnatal follow-up (38), karyotype abnormalities were confirmed in 2/38 (5%) and ECA in 8/38 (21%). 33/38 were live-born. None developed CoA postnatally. Paired analysis of Z-scores between early and late scans of 24 fetuses showed that diameters of the right heart structures and Doppler flows of tricuspid valve increased significantly during pregnancy, while the left heart structures and flow patterns did not change. The median risk of CoA did not change between the early and the late scan. We did not observe CoA in this cohort. A degree of ventricular asymmetry was present, but this was due to right heart dominance rather than hypoplasia of left heart structures. This likely reflects redistribution of blood and does not appear to confer increased risk of CoA. Predictive models of the postnatal development of CoA which set the dimensions of right and left heart structures in relation might not be applicable in this situation.

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Data Availability

Raw data were generated at the Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s & St Thomas’ NHS Trust and the Harris Birthright Centre, Fetal Medicine Research Institute, King’s College Hospital, London UK. Derived data supporting the findings of this study are available from the senior author (JMS) on reasonable request.

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Acknowledgements

We are grateful for the assistance of Nicky Callaghan, Louise Rinaldi, Claire Suckling, and Dr Ioana Dumitrascu-Biris for their assistance in ascertainment of data.

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This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s & St Thomas’ NHS Trust, Westminster Bridge Road, London, SE1 7EH, UK

    Beate Rücker, Trisha V. Vigneswaran, Vita Zidere & John M. Simpson

  2. Harris Birthright Centre, Fetal Medicine Research Institute, King’s College Hospital, London, UK

    Beate Rücker, Trisha V. Vigneswaran, Vita Zidere & John M. Simpson

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  1. Beate Rücker
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  2. Trisha V. Vigneswaran
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  3. Vita Zidere
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  4. John M. Simpson
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Contributions

All authors contributed to the study conception and design. Data were collected by Beate Rücker and Vita Zidere. Statistical analysis and writing the first draft of the manuscript were performed by Beate Rücker. Review and editing were performed by Trisha V. Vigneswaran and John M. Simpson. All authors read and approved the final manuscript.

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Correspondence to Beate Rücker.

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Rücker, B., Vigneswaran, T.V., Zidere, V. et al. Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta. Pediatr Cardiol 45, 749–758 (2024). https://doi.org/10.1007/s00246-024-03407-x

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