Asymptomatic Right Circumflex Aortic Arch Associated with Ventricular Septal Defect and Biscuspid Aortic Valve

Abstract

Previous reports have described the association of circumflex aortic arch with VSD, but there is no previous report of its association with VSD and bicuspid aortic valve. Patients are usually symptomatic either preoperatively, or after VSD repair. For this reason, division of the ligamentum arteriosum, to open the vascular ring and free the trachea and esophagus from compression, should be performed in patients undergoing cardiac surgery for associated malformations.

Congenital arch malformation: a review of morphology and surgical management of circumflex aortic arch

“…Concomitant intra-cardiac lesions, commonly VSD, can also be present with circumflex arch [6,14,33] . Other reported cardiac lesions include atrial septal defect and bicuspid aortic valve [14,33] .…”

“…Concomitant intra-cardiac lesions, commonly VSD, can also be present with circumflex arch [6,14,33] . Other reported cardiac lesions include atrial septal defect and bicuspid aortic valve [14,33] . Less commonly, Tetralogy of Fallot or double outlet RV, a lesion with a higher frequency of right aortic arch than in the normal population, had also been reported in circumflex aorta [15] ; as well as left heart hypoplasia [5,34] .…”

“…A small proportion of patients with circumflex aortic arch may remain asymptomatic or do not present until much later in life [11,16,43] . Asymptomatic circumflex arch may also present as an incidental finding with intracardiac lesion such as VSD [33] . However, the presence of a retroesophageal arch segment may result in a more severe form of vascular ring and symptoms may be more common in those with an unobstructive arch than in those with hypoplastic arch.…”

“…Division of the arterial ligament alone, however, does not address compression of the mediastinal organs from the retroesophageal arch segment. In the absence of aortic arch hypoplasia, this approach may be justifiable in patients with mild symptoms or asymptomatic patients who underwent other cardiac or thoracic procedures [33] .…”