Repair of Rare Vascular Ring: Circumflex Aortic Arch with Associated Hypoplasia and Coarctation

Abstract

We describe a neonate with an unusual vascular ring formed by a right-sided aortic arch with associated coarctation and distal hypoplasia in the presence of an aberrant left subclavian artery. The descending aorta traveled behind the esophagus to descend on the left side of the spine. A left ductus arteriosus connected to the descending aorta completing the vascular ring, with notable esophageal compression. Surgical correction was accomplished through median sternotomy, resection of the hypoplastic circumflex arch, aortic arch advancement, and end-to-side anastomosis.

Circumflex aorta: An uncharted territory

“…Mauchley et al reported a case of right circumflex aortic arch with associated hypoplasia and coarctation, aberrant LSCA with a tiny muscular VSD, parachute mitral valve, VACTERL anomaly in a neonate (day 13 of life) who underwent excision of the hypoplastic circumflex arch, arch advancement with end-side anastomosis via median sternotomy and deep hypothermic circulatory arrest. 29 This technique with direct anastomosis between native tissues allows for growth, decreasing the probability of future reoperation secondary to the child outgrowing the exogenous graft. Additionally, a median sternotomy allows for division of the vascular ring and access to any intracardiac defects that may need to be repaired concomitantly.…”

“…At third operation median sternotomy was done and the retro-esophageal segment of the circumflex arch was resected, the aortic continuity was established using a 22-mm Hemashield graft. 33 Ahluwalia et al reported a 12-year-old boy with a hypoplastic retro-esophageal circumflex right-sided cervical aortic arch and coarctation with aberrant Native tissue anastomosis Song et al 16 1 mo VSD 22q11 deletion Right Alive Bader et al 30 2 wks VSD Right Alive Bleakney et al 13 4 yrs Right Alive Mauchley et al 29 13 dys VACTERL Right Alive Extra-anatomic grafts Hellebrand et al 31 2.5 yrs VSD 22q11 deletion Right Alive Konstantinov and Puga 33 32 yrs Right Alive Gil-Jaurena et al 32 9 yrs Right Alive Ahluwalia et al 12 12 yrs Right Alive Garg et al 15 40 yrs Right Alive Sett et al 14 47 LSCA. The patient underwent extra-anatomic bypass between the ascending and descending aorta using a Gelatin-impregnated knitted vascular prosthesis of size 18 in an end-to-side fashion.…”