Background Right aortic arch encompasses multiple anatomical variants, among which the subtype involving an aberrant left subclavian artery is most frequently encountered. By contrast, a right aortic arch with an anomalous origin of the left brachiocephalic artery arising from the main pulmonary artery constitutes an exceptionally rare congenital vascular anomaly and is most often identified incidentally. Case presentation A rare case of a right aortic arch accompanied by an isolated left innominate artery is described. The anomaly was initially identified during the fetal period, subsequently confirmed after birth, and monitored over time to inform decision-making regarding the most appropriate timing of surgical intervention. Conclusion Despite its extreme rarity, this congenital anomaly is being recognized with increasing frequency due to technological advances and heightened awareness of fetal echocardiography. In the postnatal setting, the integration of computed tomography angiography with three-dimensional reconstruction facilitates more accurate and timely diagnosis. This multimodal approach supports early identification and management, thereby potentially reducing the risk of irreversible injury.
Zheng et al. (2026) studied this question.