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Original research
Implications of right aortic arch prenatal diagnosis: the multicentric nationwide ARCADE cohort
  1. Sophie Guillaumont1,2,
  2. Marie Vincenti2,3,
  3. Fanny Thomas2,4,
  4. Helena Huguet5,
  5. Marie-Christine Picot5,
  6. Hamouda Abassi2,3,
  7. Anne-Cecile Huby6,7,
  8. Daniela Laux8,
  9. Julie Thomas-Chabaneix6,7,
  10. Laurence Cohen9,
  11. Arhur Gavotto2,3,10,
  12. Pascal Amedro6,11
  13. the ARCADE study group
    1. 1 Foetal and Paediatric Cardiology Explorations Unit, Saint-Pierre Institute, Palavas-les-Flots, France
    2. 2 Department of Paediatric and Congenital Cardiology, Montpellier University Hospital, Montpellier, France
    3. 3 PhyMedExp, INSERM U1046, University of Montpellier, Montpellier, France
    4. 4 Department of Paediatric and Congenital Cardiology, Tours University Hospital, Tours, France
    5. 5 Department of Epidemiology and Biostatistics, Montpellier University Hospital, Montpellier, France
    6. 6 Department of Paediatric and Congenital Cardiology, National Reference Centre for Complex Congenital Heart Disease, CRMR M3C, Bordeaux University Hospital, Bordeaux, France
    7. 7 Aquitaine Congenital Anomalies Registry, ATENA, Bordeaux University Hospital, Bordeaux, France
    8. 8 Cardiology and Congenital Heart Disease Explorations Unit, UE3C Lowendal, Paris, France
    9. 9 Foetal, Paediatric and Adult Congenital Cardiology Explorations Unit, ETCC, Massy, France
    10. 10 Pediatric Intensive Care Unit, Montpellier University Hospital, Montpellier, France
    11. 11 IHU Liryc, INSERM U1045, University of Bordeaux, Bordeaux, France
    1. Correspondence to Professor Pascal Amedro, Department of Pediatric and Congenital Cardiology, CRMR M3C, Haut-Lévêque Cardiology Hospital, Bordeaux University Hospital, Pessac 33604, France; pascal.amedro{at}gmail.com

    Abstract

    Objectives This study aims to describe the various presentations of the prenatally diagnosed isolated right aortic arch (RAA), that is, without associated congenital heart defect and to evaluate the impact of prenatal diagnosis of isolated RAA in terms of postnatal outcome.

    Method In this multicentric retrospective study, from 2010 to 2019, all live births with a prenatal ultrasound diagnosis of isolated RAA were included, with a 1-year postnatal follow-up. The concordance between the different diagnostic steps (prenatal ultrasound, postnatal ultrasound and postnatal CT scan) was evaluated using Gwet’s AC1 coefficient.

    Results A total of 309 cases of prenatally diagnosed RAA were analysed, most of which had a left ductus arteriosus (83%). The concordance between prenatal and postnatal ultrasound diagnosis was excellent regarding the RAA type (AC1=0.97, 95% CI=(0.94 to 0.99)). The rare discrepancies mainly involved non-diagnosed or misdiagnosed double aortic arch (2%). CT scan was performed in 108 neonates (35%) and the concordance between prenatal ultrasound and postnatal CT scan was good regarding the RAA diagnosis (AC1=0.80, 95% CI=(0.69 to 0.90)) but poor regarding the distribution of brachiocephalic vessels (AC1=0.21, 95% CI=(0.06 to 0.36)). An associated genetic anomaly was sought for in half of the cases and identified in 4% of the cohort. During the first year of life, 50 (18%) infants presented with vascular ring symptoms and 24 (8%) underwent aortic arch surgery.

    Conclusion This multicentric nationwide cohort of 309 prenatally diagnosed isolated RAA demonstrated the reliability of prenatal screening, highlighted the rare cases of discrepancies between prenatal and postnatal diagnosis and underlined the value of CT scan to improve the postnatal follow-up.

    Trial registration number NCT04029064.

    • Cardiology
    • Neonatology

    Data availability statement

    Data are available on reasonable request.

    https://doi.org/10.1136/archdischild-2024-327242

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      Data availability statement

      Data are available on reasonable request.

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      Footnotes

      • X @Pascal_Amedro

      • SG and MV contributed equally.

      • AG and PA contributed equally.

      • Collaborators The ARCADE study group: Marianne Peyre, MD (Foetal, Paediatric and Adult Congenital Cardiology Clinic, Marseille, France); Isabelle Durand, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Rouen, University Hospital, Rouen, France); Tristan Hazelzet, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Rouen, University Hospital, Rouen, France); Pauline Gras, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Lille University Hospital, Lille, France); Matthias Lachaud, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Grenoble University Hospital, Grenoble, France); Herve Joly, MD (Paediatric and Congenital Cardiology Department, Val d'Ouest Clinic, Lyon, France); Guy Vaksmann, MD (Paediatric and Congenital Cardiology Department, La Louviere Clinic, Lille, France); Florent Paoli, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, La Timone Marseille, University Hospital, Marseille, France); Claire Bertail, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Louis Pradel Lyon University Hospital, Lyon, France); Yvan Bouzguenda, MD (Paediatric and Congenital Cardiology Department, La Louviere Clinic, Lille, France); Sylvie Falcon Eicher, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Dijon University Hospital, Dijon, France).

      • Contributors SG, MV, GA and PA directed, conceptualised and designed the study, enrolled patients, drafted the initial manuscript, reviewed and revised the manuscript. FT, DL, JT and LC enrolled patients and revised the manuscript. HH, M-CP, MD, HA and A-CH supervised the data management, performed the analyses and revised the manuscript. PA is the guarantor responsible for the overall content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

      • 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.