Understanding Right Aortic Arch Abnormality with Aberrant Left Subclavian Artery: Causes, Symptoms and Associated Congenital Heart Defects

*What is the most common aortic arch abnormality? What syndrome should you look for with this abnormality?

Aberrant Right Subclavian ArteryThe most common aortic arch abnormality is an aberrant right subclavian artery from the descending aorta, but it rarely causes symptoms. Look for complete or partial DiGeorge syndrome in infants with aortic arch abnormalities.

The most common aortic arch abnormality is the right aortic arch with aberrant left subclavian artery. This anomaly occurs when the aortic arch forms in a way that the fourth arch (which would typically give rise to the left subclavian artery) regresses on the left side while the right fourth arch persists, forming the right subclavian artery. As a result, the left subclavian artery arises from the aorta beyond the right subclavian artery, making a U-shaped vascular ring around the trachea and esophagus.

Clinically, this anomaly is associated with a syndrome called the vascular ring, which can cause dysphagia, stridor, and respiratory distress. Additionally, this anomaly can be seen in patients with other congenital heart defects such as Tetralogy of Fallot and truncus arteriosus. Thus, when such an anomaly is seen, it is important to evaluate for other accompanying congenital malformations.

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