When does an aberrant right subclavian artery create a vascular ring?

Area of Science:

• Cardiovascular Medicine
• Medical Genetics
• Fetal Cardiology

Background:

• A left aortic arch with an aberrant right subclavian artery is a rare vascular anomaly.
• Vascular rings can cause significant symptoms, necessitating accurate prenatal diagnosis.
• The association of this anomaly with genetic abnormalities requires further investigation.

Purpose of the Study:

• To describe the rare combination of a left aortic arch, aberrant right subclavian artery, and vascular ring.
• To investigate the association with a right-sided arterial duct.
• To improve understanding for accurate prenatal counseling.

Main Methods:

• Retrospective analysis of prenatally diagnosed cases (2016-2025) from three fetal cardiac centers.
• Documentation of aortic arch branching patterns.
• Review of genetic testing and postnatal imaging.

Main Results:

• Aberrant right subclavian artery identified in 5 out of 6 cases.
• Genetic abnormalities, including 22q11 microdeletion, found in 50% of cases.
• Associated cardiac and vascular abnormalities noted, with no extracardiac findings.

Conclusions:

• Left aortic arch with aberrant right subclavian artery can be a marker for chromosomal abnormalities.
• Accurate diagnosis is crucial for appropriate prenatal counseling regarding potential symptoms.
• Further understanding of vascular ring formation is needed to guide clinical practice.