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DiGeorge syndrome and anomalous right aortic arch with arch-on-arch and figure-of-eight configurations: Aortic sac maldevelopment and left brachiocephalic artery abnormal remodeling


1 Children's Medical Center (Pediatric Center of Excellence), Department of Pediatric Cardiology, Tehran, Iran
2 Tehran University of Medical Sciences, Tehran, Iran

Correspondence Address:
Elaheh Malakan Rad
Department of Pediatric Cardiology, Children's Medical Center (Pediatric Center of Excellence), No. 62, Dr. Gharib's street, End of Keshavarz Boulevard, Tehran 1419733151
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_215_19

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Year : 2021  |  Volume : 14  |  Issue : 1  |  Page : 125-127

 

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We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a “five-embryonic aortic arches” concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the “five-embryonic-aortic-arches” concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.






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1 Children's Medical Center (Pediatric Center of Excellence), Department of Pediatric Cardiology, Tehran, Iran
2 Tehran University of Medical Sciences, Tehran, Iran

Correspondence Address:
Elaheh Malakan Rad
Department of Pediatric Cardiology, Children's Medical Center (Pediatric Center of Excellence), No. 62, Dr. Gharib's street, End of Keshavarz Boulevard, Tehran 1419733151
Iran
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_215_19

Rights and Permissions

We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a “five-embryonic aortic arches” concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the “five-embryonic-aortic-arches” concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.






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