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A right-sided aortic arch with an isolated left subclavian artery represents a rare anatomical variant, posing diagnostic challenges and clinical complexities. Here, we present a case of a 14-year-old male presenting with respiratory symptoms, unveiling a right-sided aortic arch with an isolated left subclavian artery. Through detailed clinical evaluation, radiographic imaging, and diagnostic modalities including chest radiography, computed tomography angiography, ultrasound, and time-of-flight magnetic resonance angiography, the anatomical features and associated complications were delineated. The discussion encompasses embryological underpinnings, clinical manifestations, and therapeutic considerations, shedding light on the rarity and clinical implications of this anomaly.

Variant arch anatomy may be seen associated with many congenital heart diseases. Its accurate preoperative identification is of paramount importance in optimal surgical planning of such cases. This case describes one such variant arch anatomy with two vessel right aortic arch, comprising of bicarotid trunk (giving rise to bilateral common carotid arteries) and right subclavian artery with isolation of the left subclavian artery, in a patient with tetralogy of Fallot. Right aortic arch with isolated left subclavian artery has already been described in association with tetralogy of Fallot. However, to the best of our knowledge, present arch pattern consisting of right aortic arch with bicarotid trunk and isolated left subclavian artery has not been reported in literature so far. Moreover, this case highlights the utility of multidetector computed tomography in accurate identification of variant arch anatomy in addition to delineation of cardiac and extracardiac details.

OBJECTIVE: This study aimed to characterize the pathological types, diagnosis, chromosomal abnormalities, and postnatal clinical manifestations of right and double aortic arch malformations in fetuses.
METHODS: In this retrospective study, all fetuses diagnosed with right or double aortic arch anomalies for whom conventional two-dimensional echocardiography combined with spatio-temporal image correlation was performed at our tertiary referral center between December 2012 and December 2021 were included.
RESULTS: In total, 234 fetuses with aortic arch abnormalities were identified. Forty-one cases lost to follow-up. One hundred ninety-three cases were included in this study. One hundred eighty-seven cases with right aortic arch. Six cases with double aortic arch. Most cases of right aortic arch with aberrant left subclavian artery (77/101, 76.2%) were isolated lesions, whereas most of those with mirror-image branching (45/75, 60%) were associated with intracardiac or extracardiac anomalies. Chromosomal abnormalities were screened prenatally in 113 fetuses with right aortic arch, among whom three with aberrant left subclavian artery (3/63, 4.8%) and eight with mirror-image branching (8/50, 16%) had chromosome anomalies (p < 0.05). Furthermore, three cases had microdeletion 22q11.2 and these were significantly associated with intracardiac malformations.
CONCLUSIONS: Most cases of isolated right aortic arch do not present with clinical symptoms except isolated left subclavian artery and isolated left brachiocephalic trunk. In addition, the risk of chromosomal abnormalities in patients with isolated right aortic arch is very low. We recommend that pregnant women should be informed of the risks and benefits of undergoing invasive prenatal chromosomal detection.

BACKGROUND: The occurrence of Isolated left subclavian artery (ILSA) is relatively rare, ILSA is caused by the persistence of the dorsal segment of the sixth left arch, with regression of the fourth arch artery and interruption of the left dorsal aorta at the distal end of the seventh intersegmental artery on the left side during embryonic development. The left subclavian artery is connected to the pulmonary artery through an arterial duct, which can be closed or unobstructed. This abnormality can lead to congenital subclavian steal syndrome and vertebrobasilar artery insufficiency.
METHODS: We reported three fetuses with ILSA and intracardiac malformation. Among them, one case was suspected to be diagnosed with ILSA by echocardiography, while the other two cases were not diagnosed, but were accidentally discovered during autopsy. We have also conducted a literature review of its prenatal screening, diagnosis, management, and outcomes. Our three cases were tested by WES-Trio (whole exome sequencing). Worldwide, the ILSA cases reported in English literature have not been detected by WES. And likely pathogenic results were found in our two cases. Although it could not explain the intracardiac malformation we found, it will help to explore the etiology in the future.
CONCLUSIONS: Prenatal echocardiography detection and diagnosis of ILSA is a new challenge, which has different effects on the prognosis of the fetus. When finding intracardiac malformation with right aortic arch, we need to perform an unconventional view of ultrasound scanning and combine with CDFI to find the origin of the left subclavian artery. Although we cannot find the cause of the disease temporarily, but our genetic results can help prenatal genetic counseling.

We present the unreported case of a rare association of truncus arteriosus with the ductal origin of the left subclavian artery. Understanding and preoperative identification of these aortic variations are essential to guide optimal surgical management. In this study, the role of advanced visualization 3D modeling techniques in imaging these complex anomalies is discussed.

Tetralogy of Fallot is a cyanotic heart disease wherein aortopulmonary collaterals serve as source of pulmonary blood flow to maintain oxygenation. We report an incidentally detected isolated left subclavian artery supplying a compensatory ductus in a child with Tetralogy of Fallot that effectively contributed as a de novo palliative systemic to pulmonary artery shunt. Clinically, the entity could not be suspected, as the child did not have symptoms suggestive of arterial insufficiency of the left arm or weak pulses or neurological symptoms. The child underwent successful intracardiac repair with a reimplantation of left subclavian artery to left common carotid artery.

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