The frequency of congenital aortic arch abnormalities is less than 1% of congenital cardiovascular defects.

Cardiology

Vascular rings encircle the trachea and esophagus, which results in variable degrees of compression of both structures. Compression of the trachea causes upper airway obstruction that impairs airflow. The extent of airway compression is variable. Double aortic arch is more often associated airway compression and is also associated with more severe airway compression than other forms of vascular ring.

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  Hemorrhagic Necrosis; Massive, Postoperative: Gross; natural color, heart, in situ, a 2 day old infant, operative death, interrupted aortic arch and VSD (Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology)

Double aortic arch is associated with a chromosome band 22q11 deletion in approximately 20% of patients. Double aortic arch may be associated with band 22q11 deletion, which has various other possible manifestations. These include, but are not limited to, palatal abnormalities, laryngotracheal anomalies, speech and learning delay, characteristic facial features, hypocalcemia, abnormalities of T-cell–mediated immune function, and neurologic defects.

Cardiology

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Cardiology

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Aortic arch anomalies may be associated with the following:

1. Association with VACTERL: (vertebral, anal, cardiac, tracheal, esophageal, renal, and limb) or CHARGE (posterior coloboma, heart defect, choanal atresia, retardation, genital, and ear) associations.
2. Esophageal atresia
3. Congenital laryngeal web

Cardiology

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Cardiology

There are three common classifications to the branching pattern: ^[1]

1. Normal (as below) – seen in ~ 70% of patients.
2. Bovine arch – common origin of brachiocephalic and left common carotid artery – seen in approximately 15% of patients (more common in blacks)
3. Left common carotid has its origin from the brachiocephalic artery proper, rather than from a common trunk – – seen in approximately 10% of patients (also more common in blacks)

There may be additional branches that arise directly from the arch. These include:

• Thyroidea ima artery, usually between the brachiocephalic and left common carotid
• Left vertebral artery, usually between the left common carotid and the left subclavian arteries.
• Rarely the right subclavian and right common carotid arise independently.

Other major classifications:

Cardiology

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Cardiology