Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply

Right aortic arch (RAA) anomalies are rare congenital vascular malformations with a prevalence of 0.01% to 0.1% in the general population. These anomalies are often associated with congenital cardiac abnormalities, though many individuals remain asymptomatic and are diagnosed incidentally. However,...

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Main Authors: Ranjit N. Joseph, MD, Andrew J. Martin, MD, Nigel Wolfe, MD, Gi Tae Kwon, MD
Format: Article
Language:English
Published: Elsevier 2025-07-01
Series:Radiology Case Reports
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Online Access:http://www.sciencedirect.com/science/article/pii/S1930043325002614
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author Ranjit N. Joseph, MD
Andrew J. Martin, MD
Nigel Wolfe, MD
Gi Tae Kwon, MD
author_facet Ranjit N. Joseph, MD
Andrew J. Martin, MD
Nigel Wolfe, MD
Gi Tae Kwon, MD
author_sort Ranjit N. Joseph, MD
collection DOAJ
description Right aortic arch (RAA) anomalies are rare congenital vascular malformations with a prevalence of 0.01% to 0.1% in the general population. These anomalies are often associated with congenital cardiac abnormalities, though many individuals remain asymptomatic and are diagnosed incidentally. However, cerebrovascular anomalies associated with RAA have not been previously reported. We present the case of a 23-year-old male with an incidental finding of a Type II RAA variant characterized by an aberrant left subclavian artery and an unusual, complex configuration of the Circle of Willis (CoW). This CoW anomaly includes an absent right internal carotid artery, an absent left vertebral artery, and unique collateralization pathways. Despite the variant anatomy, the patient has remained neurologically asymptomatic. No underlying genetic abnormalities were detected during further investigation with a chromosomal analysis. This case highlights the novel association between RAA and anomalies of the CoW, emphasizing the importance of detailed cerebrovascular imaging in patients with RAA. Since an anomalous CoW is inherently associated with an increased risk of cerebrovascular accidents and poorer outcomes, information on cerebrovascular anatomy can facilitate proactive risk factor monitoring, and is valuable for surgical/neuro-interventional planning when indicated.
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spelling doaj-art-72e454e747d74d99a71b51a3af38270c2025-08-20T03:49:42ZengElsevierRadiology Case Reports1930-04332025-07-012073179318510.1016/j.radcr.2025.03.049Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supplyRanjit N. Joseph, MD0Andrew J. Martin, MD1Nigel Wolfe, MD2Gi Tae Kwon, MD3Corresponding author.; Department of Neurology, Blacktown Hospital, Blacktown, New South Wales, AustraliaDepartment of Neurology, Blacktown Hospital, Blacktown, New South Wales, AustraliaDepartment of Neurology, Blacktown Hospital, Blacktown, New South Wales, AustraliaDepartment of Neurology, Blacktown Hospital, Blacktown, New South Wales, AustraliaRight aortic arch (RAA) anomalies are rare congenital vascular malformations with a prevalence of 0.01% to 0.1% in the general population. These anomalies are often associated with congenital cardiac abnormalities, though many individuals remain asymptomatic and are diagnosed incidentally. However, cerebrovascular anomalies associated with RAA have not been previously reported. We present the case of a 23-year-old male with an incidental finding of a Type II RAA variant characterized by an aberrant left subclavian artery and an unusual, complex configuration of the Circle of Willis (CoW). This CoW anomaly includes an absent right internal carotid artery, an absent left vertebral artery, and unique collateralization pathways. Despite the variant anatomy, the patient has remained neurologically asymptomatic. No underlying genetic abnormalities were detected during further investigation with a chromosomal analysis. This case highlights the novel association between RAA and anomalies of the CoW, emphasizing the importance of detailed cerebrovascular imaging in patients with RAA. Since an anomalous CoW is inherently associated with an increased risk of cerebrovascular accidents and poorer outcomes, information on cerebrovascular anatomy can facilitate proactive risk factor monitoring, and is valuable for surgical/neuro-interventional planning when indicated.http://www.sciencedirect.com/science/article/pii/S1930043325002614Aberrant left subclavian arteryCerebrovascular disordersCircle of Willis variantsKommerell diverticulumRight aortic arch
spellingShingle Ranjit N. Joseph, MD
Andrew J. Martin, MD
Nigel Wolfe, MD
Gi Tae Kwon, MD
Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply
Radiology Case Reports
Aberrant left subclavian artery
Cerebrovascular disorders
Circle of Willis variants
Kommerell diverticulum
Right aortic arch
title Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply
title_full Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply
title_fullStr Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply
title_full_unstemmed Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply
title_short Right aortic arch with aberrant left subclavian artery and anomalous Circle of Willis supply
title_sort right aortic arch with aberrant left subclavian artery and anomalous circle of willis supply
topic Aberrant left subclavian artery
Cerebrovascular disorders
Circle of Willis variants
Kommerell diverticulum
Right aortic arch
url http://www.sciencedirect.com/science/article/pii/S1930043325002614
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