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Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct

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Abstract

A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4–123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6–128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.

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Abbreviations

RAA:

Right aortic arch

ALSA:

Aberrant left subclavian artery

LAD:

Left arterial duct

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Acknowledgements

We wish to thank Professor Lindsey Allan and the Late Dr Ian Huggon who instigated training of the fetal medicine team at Kings College Hospital to screen for anomalies of the great arteries and for developing understanding of the laterality of the aortic arch in the prenatal period. The foetal and paediatric cardiology team at Evelina London Children’s Hospital who have managed the patients, the cardiac MRI team who performed the cross-sectional imaging, the multidisciplinary airway team who investigated these patients and the cardiothoracic surgeons who performed the surgery.

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Authors and Affiliations

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

    Trisha V. Vigneswaran, Eva Kapravelou, Aaron J. Bell, Kuberan Pushparajah, John M. Simpson & Vita Zidere

  2. Fetal Medicine Research Institute, King’s College Hospital, 16-20 Windsor Walk, London, SE5 8BB, UK

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

  3. Paediatric Intensive Care Unit, Evelina London Children’s Hospital, Guy’s & St Thomas’ NHS Trust, London, SE1 7EH, UK

    Andrew Nyman & Andrew Durward

Authors
  1. Trisha V. Vigneswaran
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  2. Eva Kapravelou
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  3. Aaron J. Bell
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  8. Vita Zidere
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Contributions

VZ and JS proposed the study. TV, VZ and JS designed the study. TV, EK, AD and AN collated the data. TV and AD analysed the data and wrote the draft manuscript. AB, JS, KP, AD, AN and VZ critically reviewed and approved the manuscript.

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Correspondence to Trisha V. Vigneswaran.

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Vigneswaran, T.V., Kapravelou, E., Bell, A.J. et al. Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct. Pediatr Cardiol 39, 665–673 (2018). https://doi.org/10.1007/s00246-017-1804-5

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  • DOI: https://doi.org/10.1007/s00246-017-1804-5

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