91
4.6. Vascular Rings and Pulmonary Artery Slings
COR LOE
Recommendations
Diagnostic
1 B-NR
1. For adults with unexplained symptoms of airway or
esophageal compression that raise concern for vascular ring or
PA sling, anatomic assessment with cross-sectional imaging of
the aortic arch and PAs is recommended.
1 B-NR
2. For adults with persistent symptoms of airway or esophageal
compression after repair of vascular ring or PA sling, cross-
sectional advanced imaging is recommended to evaluate
postoperative anatomy and indications for additional
intervention.
erapeutic
2a B-NR
3. For adults with symptoms of airway or esophageal
compression and a vascular ring or PA sling, surgical repair* is
reasonable to improve symptoms.
3: No
Benefit
B-NR
4. For asymptomatic adults with a vascular ring or PA sling,
surgical repair is not indicated.
* A cardiothoracic surgeon with expertise in ACHD should perform the repair.
4.5.3. Coronary Artery Fistula
Table 46. Anatomy of Vascular Rings
Complete ring
• Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum
• Right aortic arch with mirror image branching and left ligamentum arteriosum
extending from descending aorta to main pulmonary artery
• Left aortic arch with aberrant right subclavian artery and right ligamentum arteriosum
• Double aortic arch
• Circumflex aorta
Incomplete ring
• Left aortic arch with aberrant right subclavian artery
• Right aortic arch with aberrant left subclavian artery and right ligamentum arteriosum
Other
• Innominate artery compression syndrome
• Left pulmonary artery sling
Modified with permission from Porcaro et al via a Creative Commons Attribution 4.0
International License (http://creativecommons.org/licenses/by/4.0/). Copyright © 2023
Springer Nature.
