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Impact on the trachea in children with prenatally diagnosed vascular ring formed by a right aortic arch
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  • Christopher Oakley,
  • Alice Hurn,
  • Claire Suckling,
  • Grace Moriarty,
  • john simpson,
  • Simona Turcu,
  • Jane Heraghty,
  • Vita Zidere,
  • Andrew Nyman,
  • Trisha Vigneswaran V
Christopher Oakley
Evelina London Children's Hospital
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Alice Hurn
Evelina London Children's Hospital
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Claire Suckling
Evelina London Children's Hospital
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Grace Moriarty
Evelina London Children's Hospital
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john simpson
Evelina London Children's Hospital
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Simona Turcu
Evelina London Children's Hospital
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Jane Heraghty
Evelina London Children's Hospital
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Vita Zidere
Evelina London Children's Hospital
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Andrew Nyman
Evelina London Children's Hospital
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Trisha Vigneswaran V
Evelina London Children's Hospital

Corresponding Author:trisha.vigneswaran@gstt.nhs.uk

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Abstract

Objective: There has been a rise in the prenatal detection of right sided aortic arch (RAA). When associated with a left-sided arterial duct (LD), this forms a vascular ring encircling the trachea. Infants may have symptoms or signs suggestive of trache-oesophageal compression but many are asymptomatic. The objective of this study was to investigate the relationship between symptoms and severity of tracheo-bronchial compression assessed by bronchoscopy. Methods: Retrospective review of all cases of prenatally diagnosed RAA-LD in the absence of associated congenital heart disease at Evelina London Children’s Hospital and Kings College Hospital over a 4-year period between April 2015-2019. Clinical records, fetal echocardiograms and free-breathing flexible bronchoscopy (FB) data were reviewed. Results: 112 cases of isolated RAA-LD were identified of whom 82 cases (73%) underwent FB. FB was performed median age of 11 months (range 1-36 months), no complications occurred. Aberrant left subclavian artery (ALSA) was present in 86% (96/112) and mirror image branching (MIB) in 13% (15/112). 34/112 (30%) reported symptoms during follow up. 36/77 (47%) with ALSA who underwent FB showed moderate-severe compression mostly at distal tracheal and carinal level of whom 38% had parent-reported symptoms. Moderate-severe compression was seen in 3/5 (60%) with MIB mostly at mid tracheal level of whom 3 were symptomatic but only 2 with compression. In total 36% (18/50) of investigated asymptomatic patients showed moderate to severe compression. Respiratory symptoms were poorly predictive of moderate-severe compression (positive predictive value 66%). Conclusion: The absence of symptoms did not exclude significant tracheal compression. The anatomical effect of the vascular ring is underappreciated when symptoms alone are used as a marker of tracheal compression.
17 Nov 2022Submitted to Pediatric Pulmonology
17 Nov 2022Submission Checks Completed
17 Nov 2022Assigned to Editor
17 Nov 2022Review(s) Completed, Editorial Evaluation Pending
25 Nov 2022Reviewer(s) Assigned
19 Dec 2022Editorial Decision: Revise Major
19 Feb 20231st Revision Received
22 Feb 2023Reviewer(s) Assigned
22 Feb 2023Submission Checks Completed
22 Feb 2023Assigned to Editor
22 Feb 2023Review(s) Completed, Editorial Evaluation Pending
12 Mar 2023Editorial Decision: Revise Minor
31 Mar 20232nd Revision Received
11 Apr 2023Review(s) Completed, Editorial Evaluation Pending
11 Apr 2023Submission Checks Completed
11 Apr 2023Assigned to Editor
11 Apr 2023Reviewer(s) Assigned
29 Apr 2023Editorial Decision: Accept