Asymptomatic right circumflex aortic arch associated with ventricular septal defect and biscuspid aortic valve

Anomalies of the aortic arch are frequent congenital malformations, which rarely form partial or complete vascular rings. A rare form of vascular ring is the encircling, or circumflex, aortic arch.

A 19-month-old boy, with no respiratory symptoms, was referred for ventricular septal defect (VSD) repair. Cardiac magnetic resonance imaging and echocardiography confirmed the perimembranous VSD, a bicuspid aortic valve with normal function, and showed a right-sided ascending aorta, bifurcating to the left behind the esophagus and trachea above the tracheal bifurcation, with a left-sided descending aorta, a left ligamentum arteriosum and aberrant left subclavian artery, realizing a circumflex aortic arch. The child underwent successful VSD repair and ligamentum arteriosum division, with an uneventful postoperative course.

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.