Fetal double aortic arch

Doble arco aórtico fetal

Introduction: double aortic arch represents a congenital anomaly in which the right embryonic arch does not involute, resulting in the continuity of the left and right aortic arch, encircling the trachea and esophagus. Case presentation: we present the case of a 21-year-old nulliparous gravida that was referred to prenatal care due to the suspicion of fetal cardiac alterations. Prenatal ultrasound scanning revealed four brachiocephalic vessels emerging from the right superior vena cava suggesting a symmetrical double aortic arch. Both arches encircled the trachea and the esophagus to form a vascular ring around these structures, joining to form a left-sided normal caliber descending aorta. Postnatal echocardiography demonstrated a patent right dominant aortic arch and an atretic left aortic arch. Discussion: the aortic arch courses right to the trachea and bifurcates to form a right and left arch. Posterior to the trachea, the arches merge into the descending aorta, that runs in the midline and lies in front of the vertebral column. Conclusion: double aortic arch is a rare anomaly with potentially negative consequences. Prenatal diagnosis allows early surgical repair and adequate follow-up to prevent the development of airway damage.

1. Guo Q, Kong Y, Zeng S, Zhou J, Wang X, Shang Q, Zhou J, Yuan H, Wang L, Tong L, Yi A, Zhou Q. Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes. BMC Pregnancy Childbirth. 2020;20(1):614. http://dx.doi.org/10.1186/s12884-020-03300-4.
2. Trobo Marina D, Bravo C, Lancharro Á, Gámez Alderete F, Marín C, de León-Luis J. Neonatal magnetic resonance imaging in double aortic arch diagnosed prenatally by ultrasound. J Obstet Gynaecol. 2016;36(4):526-8. doi: 10.3109/01443615.2015.1110125.
3. Noronha N, Hobbs A, Caldas P. Antenatal Diagnosis of Double Aortic Arch. Arq Bras Cardiol. 2021;116(2 suppl 1):4-7. http://dx.doi.org/10.36660/abc.20190310.
4. Hazan G, Simpson-Lavy Y, Golan-Tripto I, Taragin B, Aviram M. Neonatal presentation of double aortic arch: Case series and literature review. Pediatr Int. 2021;63(3):344-346. http://dx.doi.org/10.1111/ped.14422.
5. Petrescu AM, Ruican D, Pătru CL, Zorilă GL, Tudorache Ş, Comănescu AC, Istrate-Ofiţeru AM, Badiu AM, Ioana M, Stoica GA, Iliescu DG. Prenatal findings and pregnancy outcome in fetuses with right and double aortic arch. A 10-year experience at a tertiary center. Rom J Morphol Embryol. 2020;61(4):1173-1184. http://dx.doi.org/10.47162/RJME.61.4.19.
6. Bakhru S, Koneti NR, Patil S, Dhulipudi B, Dash T, Kolar G, Vavilala S. Prenatal diagnosis of vascular rings and outcome. Ann Pediatr Cardiol. 2021;14(3):359-365. http://dx.doi.org/10.4103/apc.APC_108_20.
7. Bhatt J, Prager JD. Neonatal Stridor: Diagnosis and Management. Clin Perinatol. 2018;45(4):817-831. http://dx.doi.org/10.1016/j.clp.2018.07.015.
8. Depypere A, Proesmans M, Cools B, Vermeulen F, Daenen W, Meyns B, Rega F, Boon M. The long-term outcome of an isolated vascular ring - A single-center experience. Pediatr Pulmonol. 2019 Dec;54(12):2028-2034. http://dx.doi.org/10.1002/ppul.24490.
9. Vigneswaran TV, Kapravelou E, Bell AJ, Nyman A, Pushparajah K, Simpson JM, 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(4):665-673. http://dx.doi.org/10.1007/s00246-017-1804-5.
10. Ullmann N, Menchini L, Salerno T, Tomà P, Cutrera R. Late diagnosis of double aortic arch: consequences on long-term follow-up. Pediatr Pulmonol. 2014;49(3):E75-7. http://dx.doi.org/10.1002/ppul.22852.