Abstract
Purpose
To give a report on the experience of our tertiary perinatology clinic on the pre- and postnatal management of the right aortic arch (RAA) by evaluating the patients as isolated and non-isolated RAA.
Materials and methods
Patients referred to our perinatology clinic for fetal echocardiography were evaluated retrospectively. They were assessed in two groups: isolated RAA and non-isolated RAA. The isolated RAA group consisted of patients without any additional cardiac or extracardiac anomalies. According to our routine practice, all patients received detailed prenatal ultrasonography following fetal echocardiography and genetic counseling.
Results
A total of 60 patients were evaluated. 38 patients (63.3%) presented with additional cardiac anomalies. 21.7% had extracardiac anomalies, including 16.7% who also had cardiac anomalies. In 2 patients (3.7%) 22q11.2 microdeletion, in 2 patients (3.7%) trisomy 21, in 1 patient (1.9%) trisomy 13 and in 1 patient (1.9%) 20p12.1p11.23 (a deletion of 2880 kbp) were reported. The most common cardiac anomaly associated with RAA was Tetralogy of Fallot (25%). Fetal growth restriction was reported in 8.3% of the cases. 18 patients had isolated RAA. 16 out of the 18 patients had normal genetic analysis. 2 of them (11.11%) presented with a 22q11.2 microdeletion.
Conclusion
A single-center experience on the diagnosis and management of RAA has been reported in this study. The results indicate that a prenatal cardiac evaluation in 3VV is of utmost importance in all pregnancies to detect RAA and refer these patients to the appropriate perinatology clinics for further evaluation and care.
Similar content being viewed by others
Data availability statement
Research data are available upon request.
References
Achiron R, Rotstein Z, Heggesh J, Bronshtein M, Zimand S, Lipitz S et al (2002) Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. Ultrasound Obstet Gynecol 20(6):553–557
Mogra R, Kesby G, Sholler G, Hyett J (2016) Identification and management of fetal isolated right-sided aortic arch in an unselected population. Ultrasound Obstet Gynecol 48(6):739–743
Vigneswaran TV, Jabak S, Syngelaki A, Charakida M, Simpson JM, Nicolaides KH et al (2021) Prenatal incidence of isolated right aortic arch and double aortic arch. J Matern Fetal Neonatal Med 34(18):2985–2990
Miranda JO, Callaghan N, Miller O, Simpson J, Sharland G (2014) Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses. Heart 100(1):54–59
Carvalho JS, Allan LD, Chaoui R, Copel JA, DeVore GR, International Society of Ultrasound in Obstetrics and Gynecology null et al (2013) ISUOG Practice Guidelines (updated): sonographic screening examination of the fetal heart. Ultrasound Obstet Gynecol 41(3):348–359
Edwards JE (1948) Anomalies of the derivatives of the aortic arch system. Med Clin N Am 32:925–949
Edwards J (1953) Malformations of the aortic arch system manifested as vascular rings. Lab Invest 2(1):56–75
Yoo S-J, Min J-Y, Lee Y-H, Roman K, Jaeggi E, Smallhorn J (2003) Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol 22(5):535–546
Zachary CH, Myers JL, Eggli KD (2001) Vascular ring due to right aortic arch with mirror-image branching and left ligamentum arteriosus: complete preoperative diagnosis by magnetic resonance imaging. Pediatr Cardiol 22(1):71–73
Weinberg PM (2006) Aortic arch anomalies. J Cardiovasc Magn Reson 8(4):633–643
Cinà CS, Althani H, Pasenau J, Abouzahr L (2004) Kommerell’s diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg 39(1):131–139
De León-Luis J, Gámez F, Bravo C, Tenías JM, Arias Á, Pérez R et al (2014) Second-trimester fetal aberrant right subclavian artery: original study, systematic review and meta-analysis of performance in detection of Down syndrome. Ultrasound Obstet Gynecol 44(2):147–153
Berg C, Bender F, Soukup M, Geipel A, Axt-Fliedner R, Breuer J et al (2006) Right aortic arch detected in fetal life. Ultrasound Obstet Gynecol 28(7):882–889
Yagel S, Silverman NH, Gembruch U (2008) Fetal cardiology: embryology, genetics, physiology, echocardiographic evaluation, diagnosis and perinatal management of cardiac diseases. Taylor & Francis, p 802
Vigneswaran TV, Allan L, Charakida M, Durward A, Simpson JM, Nicolaides KH et al (2018) Prenatal diagnosis and clinical implications of an apparently isolated right aortic arch. Prenat Diagn 38(13):1055–1061
Perolo A, De Robertis V, Cataneo I, Volpe N, Campobasso G, Frusca T et al (2016) Risk of 22q11.2 deletion in fetuses with right aortic arch and without intracardiac anomalies. Ultrasound Obstet Gynecol 48(2):200–203
Razon Y, Berant M, Fogelman R, Amir G, Birk E (2014) Prenatal diagnosis and outcome of right aortic arch without significant intracardiac anomaly. J Am Soc Echocardiogr 27(12):1352–1358
Bhide A, Acharya G, Bilardo CM, Brezinka C, Cafici D, Hernandez-Andrade E et al (2013) ISUOG practice guidelines: use of Doppler ultrasonography in obstetrics. Ultrasound Obstet Gynecol 41(2):233–239
Evans WN, Acherman RJ, Berthoty D, Mayman GA, Ciccolo ML, Carrillo SA et al (2018) Right aortic arch with situs solitus. Congenit Heart Dis 13(4):624–627
Yerlikaya G, Efetürk T, Springer S, Reischer T (2019) Prenatal detection of right aortic arch. Arch Gynecol Obstet 299(4):933–938
D’Antonio F, Khalil A, Zidere V, Carvalho JS (2016) Fetuses with right aortic arch: a multicenter cohort study and meta-analysis. Ultrasound Obstet Gynecol 47(4):423–432
McElhinney DB, Clark BJ, Weinberg PM, Kenton ML, McDonald-McGinn D, Driscoll DA et al (2001) Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 37(8):2114–2119
McElhinney DB, Driscoll DA, Emanuel BS, Goldmuntz E (2003) Chromosome 22q11 deletion in patients with truncus arteriosus. Pediatr Cardiol 24(6):569–573
Peng R, Xie H-N, Zheng J, Zhou Y, Lin M-F (2017) Fetal right aortic arch: associated anomalies, genetic anomalies with chromosomal microarray analysis, and postnatal outcome. Prenat Diagn 37(4):329–335
Zidere V, Tsapakis EG, Huggon IC, Allan LD (2006) Right aortic arch in the fetus. Ultrasound Obstet Gynecol 28(7):876–881
Velipasaoglu M, Sentürk M, Ayaz R, Atesli B, Tanir HM (2018) Characteristics of prenatally detected right aortic arch cases in a single institution. J Obstet Gynaecol 38(7):895–898
Schwarz K, Iolascon A, Verissimo F, Trede NS, Horsley W, Chen W et al (2009) Mutations affecting the secretory COPII coat component SEC23B cause congenital dyserythropoietic anemia type II. Nat Genet 41(8):936–940
Valletta EA, Pregarz M, Bergamo-Andreis IA, Boner AL (1997) Tracheoesophageal compression due to congenital vascular anomalies (vascular rings). Pediatr Pulmonol 24(2):93–105
Bonnard A, Auber F, Fourcade L, Marchac V, Emond S, Révillon Y (2003) Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg 38(4):539–543
Burch M, Balaji S, Deanfield JE, Sullivan ID (1993) Investigation of vascular compression of the trachea: the complementary roles of barium swallow and echocardiography. Arch Dis Child 68(2):171–176
Wójtowicz A, Respondek-Liberska M, Słodki M, Kordjalik P, Płużańska J, Knafel A et al (2017) The significance of a prenatal diagnosis of right aortic arch. Prenat Diagn 37(4):365–374
Bronshtein M, Zimmer EZ, Blazer S, Blumenfeld Z (2011) Right ductus arteriosus: facts and theory. Eur J Obstet Gynecol Reprod Biol 159(2):282–288
Funding
None received.
Author information
Authors and Affiliations
Contributions
NFTS: Data collection, Manuscript writing/editing; GS: Data Collection; DE: Data analysis; ZGO: Patients’ perinatological examination/ultrasound; SSC: Patients’ perinatological examination/ultrasound; HB: Postpartum evaluation/follow-up; BAC: Data analysis, Manuscript editing; MAY: Project development, manuscript editing.
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Topbas Selcuki, N.F., Senol, G., Esin, D. et al. Prenatal diagnosis and postnatal outcomes of right aortic arch anomalies. Arch Gynecol Obstet 306, 745–752 (2022). https://doi.org/10.1007/s00404-021-06346-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00404-021-06346-7