RT Journal Article
SR Electronic
T1 Implications of right aortic arch prenatal diagnosis: the multicentric nationwide ARCADE cohort
JF Archives of Disease in Childhood - Fetal and Neonatal Edition
JO Arch Dis Child Fetal Neonatal Ed
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP fetalneonatal-2024-327242
DO 10.1136/archdischild-2024-327242
A1 Guillaumont, Sophie
A1 Vincenti, Marie
A1 Thomas, Fanny
A1 Huguet, Helena
A1 Picot, Marie-Christine
A1 Abassi, Hamouda
A1 Huby, Anne-Cecile
A1 Laux, Daniela
A1 Thomas-Chabaneix, Julie
A1 Cohen, Laurence
A1 Gavotto, Arhur
A1 Amedro, Pascal
A1 
YR 2024
UL http://fn.bmj.com/content/early/2024/07/04/archdischild-2024-327242.abstract
AB 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.Data are available on reasonable request.