Pediatric Right Aortic Arch With Aberrant Left Subclavian Artery

Etiology: Congenital
Imaging:
— Right arch
— Aberrant left subclavian artery
— May cause airway compression
— Has diverticulum of Kommerell which is bulbous configuration of portion of residual dorsal aorta at origin of an aberrant left subclavian artery
— Descending aorta usually right sided
— Left sided ligamentum arteriosum completes the ring extending between left pulmonary artery and diverticulum
— Ductus is opposite side of aortic arch and arises from junction between diverticulum and normal sized subclavian artery
— If there is no diverticulum, ductus is on same side as aortic arch and there is no ring
Imaging UpperGI: Posterior indentation on the esophagus
DDX:
Complications:
Treatment:
Clinical:
— Second most common vascular ring
— Most common right arch
— Rarely associated with congenital heart disease

Radiology Cases of Right Aortic Arch With Aberrant Left Subclavian Artery

CXR of right aortic arch with aberrant left subclavian artery — CXR shows a right side aortic arch. Echocardiogram showed an aberrant left subclavian artery.

CXR of right aortic arch with aberrant left subclavian artery in a patient with ventricular septal defect — CXR AP shows a right-sided aortic arch, cardiomegaly, and marked pulmonary vascular congestion.

Upper GI of right aortic arch with aberrant left subclavian artery — AP image (left) from an UGI exam shows a right sided aortic arch and an indentation running across the upper esophagus which on the lateral image (right) shows the indentation to be posterior to the esophagus.

CT angiogram of right aortic arch with aberrant left subclavian artery — Lateral image from an esophagram (above left) shows a posterior indentation on the esophagus which was persistent. Coronal CT with contrast of the chest (above right) with 3D reconstructions (below) show a right-sided aortic arch with an aberrant left subclavian artery that originated from a Kommerell diverticulum and that courses behind the esophagus. The ductus arteriosus was noted to extend from the Kommerell diverticulum and completed the vascular ring.

CT of right aortic arch with aberrant left subclavian artery — AP image from an upper GI exam (above left) shows a right sided aortic arch and an indentation running at an angle from right to left across the upper esophagus. Lateral image from an upper GI exam (above right) shows a posterior indentation across the upper esophagus. 3D CT with contrast of the chest viewed from the front (below left) shows a right sided aortic arch and a right sided descending aorta and viewed from the back (below right) shows the aberrant left subclavian artery arising from a large diverticulum of Kommerell. The airway (in blue) was not compressed.

Surgery Cases of Right Aortic Arch With Aberrant Left Subclavian Artery

Surgical image through a thoracotomy incision (left) shows blue vessel loop around an aberrant left subclavian artery arising from a Kommerell diverticulum. Black suture loop is around a patent ductus arteriosus that was noted to extend from the Kommerell diverticulum and completed a vascular ring which was compressing the esophagus posterior to it. Surgical image (right) with the patent ductus arteriosus mobilized off the esophagus which is between the tips of the hemostat show the esophagus now resorting to its normal caliber.

Surgical image of right aortic arch with aberrant left subclavian artery — Surgical image taken through a left thoracotomy shows a blue vessel loop around the ligamentum arteriosum which was compressing the esophagus posterior to it. The red vessel loops are around the right aortic arch (on the right) and the left subclavian artery (on the left). The vascular ring was subsequently divided by division of the ligamentum arteriosum. The vascular ring then sprang open dramatically, relieving its compression upon the esophagus.