Congenital Variants and Anomalies of the Aortic Arch

Abstract

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. RSNA, 2016.

Reduced maternal vitamin A status increases the incidence of normal aortic arch variants

“…The bovine arch variant in particular is associated with an increased risk for thoracic aortic aneurysm (Priya et al, 2018;Toya et al, 2018). Benign aortic arch abnormalities can also contribute to complications during surgical interventions (Hanneman, Newman, & Chan, 2017;Natsis et al, 2009). Therefore, identifying these variants and understanding their developmental origins is of clinical importance.…”

“…While anatomic variants of the aortic arch are often considered clinically insignificant, variants can be associated with clinically significant cardiac malformations or interfere with respiration and swallowing (Carucci & Turner, 2015;Poletto, Mallon, Stevens, & Avitabile, 2017;Priya, Thomas, Nagpal, Sharma, & Steigner, 2018;Stewart, 1964;Wells, Landing, & Shankle, 1993). Benign aortic arch abnormalities can also contribute to complications during surgical interventions (Hanneman, Newman, & Chan, 2017;Natsis et al, 2009). Benign aortic arch abnormalities can also contribute to complications during surgical interventions (Hanneman, Newman, & Chan, 2017;Natsis et al, 2009).…”

Unusual symptomatic late onset presentation of aberrant right subclavian artery: report of two cases and short literature review

“…Even if chest radiography [15] and manometry as well as findings in upper endoscopy may show abnormalities, first diagnostic procedure which should reveal this abnormality is barium esophagogram [6]. However, if it is unremarkable and dysphagia has a chronic course, a CT scan with contrast or CT angiography should be made [9]. Multidetector computed tomographic (MDCT) angiography is considered the golden standard if a surgical approach is to be followed [16,17].…”

“…The root of the ARSA is formed by the persisting right aortic arch and as a result a diverticulum at the proximal descending aorta can be found. This broad base of the ARSA is known as Kommerel's diverticulum [5,9]. Commonest symptoms include dyspnea during childhood and dysphagia mainly for solid food with aging [3,6] (Table 1).…”

Circumflex retroesophageal right aortic arch: rare differential of mediastinal widening

“…Embryologically, a circumflex right aorta with an aberrant LSCA results from regression of the left dorsal aorta between the left common carotid artery and the LSCA, with the right dorsal aorta forming the distal portion of the arch. It has also been postulated that the third aortic arch gives rise to the proximal aortic arch while the distal arch is formed by the fourth arch, which can explain the elongated transverse arch as well as the relatively higher position of the proximal aortic arch 1. Even in the absence of a ligamentum arteriosum, the circumflex aorta itself, especially if ectatic, can cause symptomatic tracheal compression, as in this case, which makes identification of this anomaly pertinent; as such, patients may require aortic uncrossing procedure, where the distal aortic arch is transected, the retroesophageal segment mobilised and brought anterior to trachea followed by its anastomosis with the ascending aorta 2…”