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IPCCC ICD-11 Congenital Heart Atlas

You are here: Persistent fifth aortic arch Congenital anomaly of aortic arch Congenital anomaly of aorta or its branches Congenital anomaly of great arteries including arterial duct Structural developmental anomaly of heart or great vessels

IPCCC Term Persistent fifth aortic arch
IPCCC Code 09.28.08
ICD-11 Code 82536098


A congenital cardiovascular malformation in which there is an accessory artery originating from the ascending aorta proximal to the ostium of the innominate (brachiocephalic) artery which connects to the descending aorta or near the confluence of the right and left pulmonary arteries.



STS-EACTS-derived IPCCC term

Aortic pathology, Abnormality involving aortic arch, Persisting fifth aortic arch

EPCC-derived IPCCC term

Persisting fifth aortic arch

ICD-11 MMS code or crossmap

LA8B.2Y*: This Foundation term is not in ICD-11 MMS. If using MMS to code, crossmap to "Other specified congenital anomaly of aorta or its branches" with code LA8B.2Y

ICD-10 MMS code or crossmap

Q25.4**: This Foundation term is not in ICD-10. Crossmap to "Other congenital malformations of aorta" with code Q25.4


Congenital anomaly of aortic arch


Coding Notes

When the arterial connection is between the ascending and descending aorta, it is often associated with coarctation of the aorta. This malformation may be distinguished from a double aortic arch by the lack of arch vessels arising from it and by establishing that the accessory vessel and the aortic arch lie on the same side of the tracheo-oesophageal axis. Although this malformation has been termed a “persistent fifth aortic arch”, the use of this term is in dispute.

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