Double Aortic Arch

During fetal development, the aorta is formed through a series of arches that later regress leaving just the left-sided aorta. When the arches regress abnormally, they form congenital abnormalities of aortic arch. One such anomaly is double aortic arch, also considered a form of vascular ring where the trachea and esophagus are surrounded in the form of a circle by the right and left aortic arches (1). In the majority of cases (83.3%) it is an isolated anomaly and in about 16.6% cases it can be associated with other cardiac malformations such as  ventricular septal defects and double outlet right ventricles. 

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Aortic arch anomalies occur in 1% to 2% of the general population.² 
A double aortic arch (DAA) is a rare type of congenital aortic arch anomaly, affecting approximately 0.005% ~ 0.007% of fetuses.³

The exact etiology of DAA is as yet unknown. However, it can be rarely associated with 22q11.2 deletion even in the absence of intra-cardiac disease.

Development of double aortic arch is easily understood by Edward’s theoretical model in which various anomalies in aortic arch explained by the pattern of breaks at different levels.  Abnormal persistence or regression of various segments leads to several types of malformations. Double aortic arch forms when both the right and the left fourth branchial arches persist, each one to be seen on either side of trachea.⁴ The common carotid and subclavian artery arises from the ipsilateral arches on both sides. This arrangement encircles the trachea and esophagus completely and is the most common cause of complete vascular ring (5). Most of cases (approximately 80%) have a right dominant system, with a left aortic arch that is more frequently patent, but it can also be hypoplastic or atretic (6). Given that the descending aorta is usually opposite the dominant arch, a large right aortic arch, left-sided descending aorta and left sided ligamentum arteriosus is the most common presentation.(7)

Cardiac anomalies are less common in a double aortic arch than in other aortic arch anomalies and it is isolated anomaly in about (83.3%) cases.  Approximately 16.6% of double aortic arch cases are associated with other congenital heart anomalies, mainly ventricular septal defects and double outlet right ventricles.8 Extracardiac malformations have rarely been mentioned in association with double aortic arch.
There seems to be a low association with 22q11 micro deletion in the absence of other structural heart anomalies, given that most cases affected by this chromosomal abnormality have congenital cardiac or extracardiac defects. Among patients with a double aortic arch, those with an atretic minor arch had a significantly higher rate of deletion than those with a patent minor arch, but there was no difference between patients with left and right dominance of the double arch.⁹

The risk of recurrence is mostly related to the recurrence of associated chromosomal and deletion abnormalities.

Recent ISOUG guidelines (2022) recommend addition of the 3-vessel and trachea view in the routine mid trimester ultrasound scan. The diagnosis of double aortic arch is made in a transverse view of the fetal thorax at the level of the 3-vessel and trachea view when there are two aortic arches that form a complete vascular ring encircling the trachea.  In double aortic arch the ascending aorta bifurcates at the level of trachea to have one arch to the right and one to the left (in the Greek letter “lambda” configuration) which is better demonstrated on color Doppler.¹⁰ The carotid and subclavian arteries arise separately from each ipsilateral arch and the descending aorta inconsistently tends to appear deviated to one side or another, usually to the side of the obvious ductus arteriosus. For this very reason, the branching pattern of the arches helps in the differential diagnosis, as this kind of branching has not been described for a right aortic arch, either with an aberrant left subclavian artery or mirror branching. Hence adding an axial view of the upper mediastinum is highly recommend (subclavian artery view) to the 3-vessel and trachea view, which would allow us to recognize part of the branching pattern of the aortic arch. When a subclavian artery with a retrotracheal course (aberrant) is observed, we are able to rule out the diagnosis of a double aortic arch with certainty.¹¹
There is a high percentage of double aortic arch cases in which one of the arches is atretic, commonly the left one, and it is located in a more caudal position than the other. This can hamper visualization of all the vessels at the same time, causing difficulties for the differential diagnosis between a double aortic arch and a right aortic arch. Once double arch anomaly is diagnosed, a comprehensive ultrasound scan, including fetal echocardiography, detailed fetal structural scan and evaluation of the thymic size is suggested to assess associated other cardiac and extra cardiac abnormalities and provide clues to the presence or absence of a 22q11.2 microdeletion.¹²
STIC technology helps to assess the fetal heart in a four-dimensional (4D) cineloop sequence with slow-motion imaging, without fetal movements and allow other observers to review the data offline. Collectively it helps for the better assessment of aortic arch anomalies.

The differential diagnoses of vascular ring include the following abnormal variants:
•    Right aortic arch with mirror-image branching  and a left ductus arteriosus arising from the retroesophageal dimple, resulting from an atretic left arch of a double aortic arch anomaly; 
•    Right aortic arch with aberrant left subclavian artery associating a left-sided ductus; 
•    Right aortic arch with left descending aorta and left ductus arteriosus; 
•    Right aortic arch with a retroesophageal left brachiocephalic trunk and a left ductus arteriosus.
•    Left aortic arch associated with right descending aorta and also right ductus arteriosus; 
•    Left aortic arch associating an aberrant right subclavian artery and also a right ductus arteriosus¹³

Prenatal diagnosis of isolated RAA and DAA provides the option of undertaking further genetic investigations and counseling the parents of the need for postnatal follow-up. Some patients with RAA or DAA with left arterial duct become symptomatic with respiratory or swallowing difficulties in infancy and these require surgery.¹⁴

ISUOG guidelines (2022) recommend that the three-vessel view or three-vessels-and-trachea view should be included in routine mid-trimester fetal ultrasound screening in order to improve the prenatal detection of DAA and its related findings. Prenatal detection may identify a unique cohort of infants with a double aortic arch, in whom postnatal imaging is recommended before referral for surgical correction. Early surgical repair of a double aortic arch has a favorable long-term prognosis; therefore, prompt detection and intervention are required to prevent long-term morbidity.¹⁵

After birth and during the first 2 years of life, a double aortic arch is generally symptomatic (72.4%). The most common symptoms, which can range from mild to severe depending on the degree of compression of trachea or esophagus caused by the vascular ring, are stridor, cough, asthma, respiratory distress, apnea, recurrent episodes of pneumonia, dysphagia and choking episodes.¹⁶ This prognosis should be provided to parents before birth to avoid a delay in the diagnosis of airway compression. The definitive diagnosis is made after birth by either contrast-enhanced computed tomography or magnetic resonance imaging, which should only be performed in symptomatic patients and in those cases in which surgery is needed.¹⁷

Respiratory distress of the newborn is initially investigated by chest radiography, followed by computed tomography angiography or magnetic resonance angiography, which offer the three-dimensional imaging of the vascular ring and provide anatomic details needed for corrective surgery. Surgical options include either minimally invasive techniques such as thoracoscopic or robotic-assisted surgery, or open procedures involving thoracotomy. 

Early surgical division of the arches, freeing the trachea and esophagus from surrounding tissues, is imperative to prevent the long-term sequelae of tracheobronchial compression of patients with DAA. An atretic or stenotic segment of one of the arches is the selected site for surgical division, with section of the the ductus or ligamentum insuring the release of trachea from the decompressive ring. The outcomes of surgical treatment are excellent, with most patients having complete resolution of symptoms after some time.  Complications occur in 11% of cases and 4% of infants die because of residual stenosis. Chronic respiratory symptoms can be present and 42% of patients undergo surgery after three years of age. The reoperation rate is generally low, under 10%.¹⁸

1.    Seo HK, Je HG, Kang IS, Lim KA. Prenatal double aortic arch presenting with a right aortic arch and an anomalous artery arising from the ascending aorta. Int J Cardiovasc Imaging. 2010;26 (Suppl 1): 165-8)
2.    Yoo SJ, Min JY, Lee YH, Roman K, Jaeggi E, Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol 2003; 22:535–546.
3.    Achiron R, Rotstein Z, Heggesh J, Bronshtein M, Zimand S, Lipitz S, Yagel S. Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. Ultrasound Obstetrics Gynecol. 2002;20 (6):553–7.
4.    Hanneman K, Newman B, Chan F. Congenital Variants and Anomalies of the Aortic Arch. RadioGraphics 2017;37:32-51.
5.    Thankavel PP, Brown PS, Lemler MS. Left-Dominant Double Aortic Arch in Critical Pulmonary Stenosis and Ventricular Septal Defect. Pediatr Cardiol 2012;33:1469-71.
6.    Ramos-Duran L, Nance JW Jr, Schoepf UJ, et al. Developmental aortic arch anomalies in infants and children assessed with CT angiography. American Journal of Roentgenology 2012;198:W466-W474. 
7.    Hanneman Kate, Newman Beverly, Chan F. Congenital variants and anomalies of the aortic arch. Radiographics 2017; 37:32-51.
8.    Trobo D, Bravo C, Álvarez T, Pérez R, Gámez F, De León-Luis J. Prenatal sonographic features of the double aortic arch: literature review and perinatal management. J Ultrasound Med 2015; 34:1921–1927.
9.    McElhinney DB, Clark BJ III, Weinberg PM, et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 2001; 37:2114–2119.
10.    R. Chaoui, J. Hoffmann and K. S. Heling, Three-dimensional (3D) and 4D color Doppler fetalechocardiography using spatio-temporal image correlation (STIC), Ultrasound Obstet Gynecol2004;23: 535 – 545.
11.    Duna Trobo, MD, Coral Bravo, MD, PhD, Teresa Alvarez et al Prenatal Sonographic Features of a Double Aortic Arch J Ultrasound Med 2015; 34:1921–1927 | 0278-4297.
12.    Qiao Guo1,2, Yifan Kong1, Shi Zeng1 et al Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes, Guo et al. BMC Pregnancy and Childbirth (2020) 20:614.
13.    Roxana Elena Bohiltea, Paul Costin Pariza, Irina Stavarache, et al, Prenatal Ultrasound Diagnosis of Double Aortic Arch versus Right Aortic Arch Variant in Vascular Ring Formation – Case Report and Review of the Literature,MAEDICA – a Journal of Clinical Medicine 2021; 16(4): 717-722
14.     Anand R, Dooley KJ, Williams WH, et al. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatric cardiology. 1994 Mar-Apr;15(2):58-61. 
15.    L. Hunter, N. Callaghan, K. Patel, L. Rinaldi, H. Bellsham-Revell and G. Sharland, Prenatal echocardiographic diagnosis of double aortic arch, Ultrasound Obstet Gynecol 2015; 45: 483–485
16.     Yagel S, Silverman NH, Gembruch U (eds). Fetal Cardiology: Embryology, Genetics, Physiology, Echocardiographic Evaluation, Diagnosis and Perinatal Management of Cardiac Disease.2nd ed. New York, NY: Informa Healthcare USA; 2009.
17.     Koontz CS, Bhatia A, Forbess J, Wulkan ML. Video-assisted thoracoscopic division of vascular rings in pediatric patients. Am Surg 2005; 71:289–291
18.    Roxana Elena Bohiltea, Paul Costin Pariza, Irina Stavarache, et al, Prenatal Ultrasound Diagnosis of Double Aortic Arch versus Right Aortic Arch Variant in Vascular Ring Formation – Case Report and Review of the Literature,MAEDICA – a Journal of Clinical Medicine 2021; 16(4): 717-722

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