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Management of prenatally detected vascular rings: a United Kingdom national survey

Published online by Cambridge University Press:  15 August 2022

Trisha V. Vigneswaran Open the ORCID record for Trisha V. Vigneswaran [Opens in a new window] ,
Lindsey E. Hunter ,
Julene S. Carvalho Open the ORCID record for Julene S. Carvalho [Opens in a new window]  and
Anna N. Seale
Trisha V. Vigneswaran*
Affiliation:
Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s & St. Thomas’ NHS Trust, London SE1 7EH, UK
Lindsey E. Hunter
Affiliation:
Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, UK
Julene S. Carvalho
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK Fetal Medicine Unit, St. George’s University Hospitals NHS Foundation Trust, London, UK Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Research Institute, St. George’s University of London, London, UK
Anna N. Seale
Affiliation:
Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK Heart Unit, Birmingham Children’s Hospital NHS Foundation Trust, Birmingham, UK
*
Author for correspondence: Dr Trisha V. Vigneswaran MBBS BSc(Hons.) MD(res) MRCPCH, Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s & St Thomas’ NHS Trust, London SE1 7EH, UK. E-mail: Trisha.vigneswaran@gstt.nhs.uk
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Abstract

Objective:

To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA).

Methods:

Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies.

Results:

There were 28 prenatal and 90 postnatal surveys completed. Prenatally, there was consensus for potential associated chromosomal/genetic anomalies, but there was variation in the risk quoted. Confidence in defining aortic arch morphology was reported by 43% (12/28) of fetal cardiologists. There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention.

Conclusion:

This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.

Keywords

Congenital heart diseaseVascular ringPrenatal diagnosis

Information

Type
Original Article
Information
Cardiology in the Young , Volume 33 , Issue 8 , August 2023 , pp. 1332 - 1335
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Copyright
© The Author(s), 2022. Published by Cambridge University Press

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References

Department of Health. Fetal anomaly screening programme 2015 [Available from:. Available at: https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/421650/FASP_Standards_April_2015_final_2_.pdf.Google Scholar
Ctori, E, Crucean, A, Pinkey, B, et al. Morphology of vascular ring arch anomalies influences prognosis and management. In Archives of disease in childhood 2020, [published Online First: 2020/10/28] DOI 10.1136/archdischild-2020-319388.Google Scholar
Vigneswaran, TV, Allan, L, Charakida, M, et al. Prenatal diagnosis and clinical implications of an apparently isolated right aortic arch. Prenat Diagn 2018; 38: 1055–1061. DOI 10.1002/pd.5388.CrossRefGoogle ScholarPubMed
Vigneswaran, TV, Van Poppel, MP, Griffiths, B, et al. Postnatal impact of a prenatally diagnosed double aortic arch. Archives of disease in childhood 2021 2021; 106: 564–569.CrossRefGoogle ScholarPubMed
Edwards, FR. Vascular compression of the trachea and oesophgus. Thorax 1959; 14: 187–200. DOI 10.1136/thx.14.3.187.CrossRefGoogle ScholarPubMed
Worhunsky, DJ, Levy, BE, Stephens, EH, et al. Vascular rings. Semin Pediatr Surg 2021; 30: 151128. DOI 10.1016/j.sempedsurg.2021.151128.CrossRefGoogle ScholarPubMed
Stephens, EH, Eltayeb, O, Kennedy, C, et al. Influence of fetal diagnosis on management of vascular rings. Ann Thorac Surg 2022; 113: 630–636. DOI 10.1016/j.athoracsur.2021.01.025.CrossRefGoogle ScholarPubMed
Evans, WN, Acherman, RJ, Ciccolo, ML, et al. Vascular rings and slings: contemporary observations. Prog Pediatr Cardiol 2019; 53: 42–46.CrossRefGoogle Scholar
Backer, CL. The vascular ring decision tree. Eur J Cardio-Thorac Surg 2022; 62: ezab525. DOI: 10.1093/ejcts/ezab525.CrossRefGoogle Scholar
Young, AA, Hornberger, LK, Haberer, K, et al. Prenatal detection, comorbidities, and management of vascular rings. Am J Cardiol 2019; 123: 1703–1708.CrossRefGoogle ScholarPubMed
Han, M-T, Hall, DG, Manché, A, et al. Double aortic arch causing tracheoesophageal compression. Am J Surg 1993; 165: 628–631.CrossRefGoogle ScholarPubMed
Swarnkar, P, Speggiorin, S, Austin, BC, et al. Contemporary surgical outcome and symptomatic relief following vascular ring surgery in children: effect of prenatal diagnosis. Eur J Cardio-thorac Surg.Google Scholar
Alsenaidi, K, Gurofsky, R, Karamlou, T, et al. Management and outcomes of double aortic arch in 81 patients. Pediatrics 2006; 118: e1336–e1341.CrossRefGoogle ScholarPubMed
Anand, R, Dooley, KJ, Williams, WH, et al. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatr Cardiol 1994; 15: 58–61.CrossRefGoogle ScholarPubMed
Depypere, A, Proesmans, M, Cools, B, et al. The long-term outcome of an isolated vascular ring - a single-center experience. Pediatr Pulmonol 2019; 54: 2028–2034.CrossRefGoogle ScholarPubMed
Callahan, CP, Merritt, TC, Canter, MW, et al. Symptom persistence after vascular ring repair in children. J Pediatr Surg 2020; 55: 2317–2321.CrossRefGoogle ScholarPubMed
Vigneswaran, TV, Kapravelou, E, Bell, AJ, 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 2018; 39: 665–673.CrossRefGoogle ScholarPubMed
Luciano, D, Mitchell, J, Fraisse, A, et al. Kommerell diverticulum should be removed in children with vascular ring and aberrant left subclavian artery. Ann Thorac Surg 2015; 100: 2293–2297.CrossRefGoogle ScholarPubMed
Francois, K, Panzer, J, De Groote, K, et al. Early and late outcomes after surgical management of congenital vascular rings. Eur J Pediatr 2017; 176: 371–377.CrossRefGoogle ScholarPubMed
Biermann, D, Holst, T, Huners, I, et al. Right aortic arch forming a true vascular ring: a clinical review. Eur J Cardio-thorac Surg, 2021; 60: 1014–1021.CrossRefGoogle ScholarPubMed
D’Antonio, F, Khalil, A, Zidere, V, et al. Fetuses with right aortic arch: a multicenter cohort study and meta-analysis. Ultrasound Obstet Gynecol 2016; 47: 423–432.CrossRefGoogle ScholarPubMed
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