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Morphology of vascular ring arch anomalies influences prognosis and management
  1. Elena Ctori1,
  2. Adrian Crucean1,2,
  3. Benjamin Pinkey3,
  4. Simon P McGuirk3,
  5. Robert H Anderson2,4,
  6. John Stickley2,
  7. Timothy J Jones2,
  8. Anna N Seale1,2
  1. 1 College of Medical and Dental Science, University of Birmingham, Birmingham, UK
  2. 2 Heart Unit, Birmingham Children's Hospital NHS Foundation Trust, Birmingham, UK
  3. 3 Department of Radiology, Birmingham Children's Hospital NHS Foundation Trust, Birmingham, UK
  4. 4 Institute of Genetics, Newcastle, UK
  1. Correspondence to Elena Ctori, College of Medical and Dental Science, University of Birmingham, Birmingham B15 2TT, UK; elena.ctori{at}nhs.net

Abstract

Objective This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention.

Methods This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data.

Results Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery.

Conclusion Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

  • cardiology
  • ent
  • respiratory

Data availability statement

Data are available upon reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information. The corresponding author (EC, elena.ctori@nhs.net) has stored the deidentified data in a password-locked computer and can provide the data on reasonable request.

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Data availability statement

Data are available upon reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information. The corresponding author (EC, elena.ctori@nhs.net) has stored the deidentified data in a password-locked computer and can provide the data on reasonable request.

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Footnotes

  • Collaborators Esther Poole.

  • Contributors EC (corresponding author) contributed towards the methodological design, data collection, interpretation of the data and drafting of the manuscript. She agrees to be accountable for all aspects of the work. AC, ANS, JS and TJ collaborated on the methodological design of the study and supervised the data collection. BP and SM contributed towards the acquisition and interpretation of the data in this study. JS performed the data analysis. EC, AC, AS, JS, TJ, BP, SMG and RHA contributed towards data interpretation and revised the subsequent manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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