Prenatal diagnosis of isolated (i.e. without intracardiac anomaly) right aortic arch (IRAA) increases [1Evans W.N., Acherman R.J., Ciccolo M.L., Carrillo S.A., Mayman G.A., Luna C.F., et al. Vascular ring diagnosis and management: notable trends over 25 years World J Pediatr Congenit Heart Surg 2016 ;  7 (6) : 717-720 [cross-ref]

Cliquez ici pour aller à la section Références], mostly due to the integration of the tracheal-vessel image in fetal screening [2Bravo C., Gámez F., Pérez R., Álvarez T., De León-Luis J. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol J Ultrasound Med Off J Am Inst Ultrasound Med 2016 ;  35 (2) : 237-251 [cross-ref]

Cliquez ici pour aller à la section Références]. IRAA may constitute an encircling anomaly with functional digestive or respiratory repercussion [3Yoo S.-J., Min J.-Y., Lee Y.-H., Roman K., Jaeggi E., Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies Ultrasound Obstet Gynecol Off J Int Soc Ultrasound Obstet Gynecol 2003 ;  22 (5) : 535-546 [cross-ref]

Cliquez ici pour aller à la section Références, 4Mogra R., Kesby G., Sholler G., Hyett J. Identification and management of fetal isolated right-sided aortic arch in an unselected population Ultrasound Obstet Gynecol Off J Int Soc Ultrasound Obstet Gynecol 2016 ;  48 (6) : 739-743 [cross-ref]

Cliquez ici pour aller à la section Références], and is sometimes associated with genetic anomalies [5Miranda J.O., Callaghan N., Miller O., Simpson J., Sharland G. Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses Heart Br Card Soc 2014 ;  100 (1) : 54-59 [cross-ref]

Cliquez ici pour aller à la section Références], especially the microdeletion 22q1.1 [6McElhinney D.B., Clark B.J., Weinberg P.M., Kenton M.L., McDonald-McGinn D., Driscoll D.A., et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching J Am Coll Cardiol 2001 ;  37 (8) : 2114-2119 [cross-ref]

Cliquez ici pour aller à la section Références, 7Maya I., Singer A., Baris H.N., Goldberg Y., Shalata A., Khayat M., et al. Prenatal microarray analysis in right aortic arch-a retrospective cohort study and review of the literature J Perinatol Off J Calif Perinat Assoc 2018 ;  38 (5) : 468-473 [cross-ref]

Cliquez ici pour aller à la section Références, 8Peng R., Xie H.-N., Zheng J., Zhou Y., Lin M.-F. Fetal right aortic arch: associated anomalies, genetic anomalies with chromosomal microarray analysis, and postnatal outcome Prenat Diagn 2017 ;  37 (4) : 329-335 [cross-ref]

Cliquez ici pour aller à la section Références]. However, antenatal and postnatal cares are not consensual and based only on partial data. The aim of this study was to describe the management of this new kind of population in two French M3C centers.

We conducted a retrospective observational study in two M3C competence centers (Montpellier and Tours) from 2010 to 2018 including all patients with a prenatal diagnosis of IRAA.

We included 36 patients and observed an increased prenatal diagnosis among time in both centers (Fig. 1). Amniocentesis were performed in 27 cases (75%) and conducted to the diagnosis of del22q1.1 in one case (3%). After birth, 16 children were diagnosed for encircling anomaly, but only 4 (11%) needed a surgical repair, all for a double aortic arch. Comparing the practice in the two centers, we found an increased number of tomodensitometry (67% versus 27%; P=0.04) and proportionally an increased number of diagnosis of encircling anomalies (62% versus 14%; P=0.02) in center 1 (Table 1). Indeed the scanner, when performed, completed the echocardiographic prenatal diagnosis in 100% of cases.

The lack of harmonization of practices concerning the prenatal diagnosis of IRAA may have consequences for patients care. Therefore, we begin a national multicentric retrospective study between 2010 to 2019 to collect data about the management of prenatal diagnosis of IRAA: ARCADE (2019_IRB-MTP_03-31) that aims to evaluate the reliability of antenatal diagnosis of IRAA in terms of anatomy and functional prognosis to harmonize the medical care for this population.