LA8B.22

ICD-11 MMS 2026-01

Interrupted aortic arch

Description

A congenital cardiovascular malformation in which there is an absence of luminal continuity between the ascending and descending aorta. Additional information: this includes luminal atresia with discontinuity between the aortic segments and also luminal atresia with fibrous continuity between the aortic segments. Interrupted aortic arch is defined as the loss of luminal continuity between the ascending and descending aorta. In most cases, blood flow to the descending thoracic aorta is through a patent arterial duct, and there is a large ventricular septal defect. Arch interruption is further defined by site of interruption. In type A, interruption is distal to the left subclavian artery; in type B, interruption is between the left carotid and left subclavian arteries; and in type C, interruption occurs between the innominate and left carotid arteries.

Index terms

  • Interrupted aortic arch
  • IAA - [interrupted aortic arch]
  • aorta arch atresia
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  • aorta atresia
  • aorta ring atresia
  • aortic arch atresia
  • aortic arch interruption
  • aortic ring atresia
  • Interrupted aortic arch distal to subclavian artery, type A
  • IAA type A - [interrupted aortic arch type A]
  • aortic arch interruption, type A
  • Interrupted aortic arch between subclavian and common carotid arteries, type B
  • Aortic interruption, type B
  • IAA type B - [interrupted aortic arch type B]
  • Interruption of the aortic arch, type B
  • aortic arch interruption, type B
  • Interrupted aortic arch between carotid arteries, type C
  • Aortic interruption, type C
  • IAA type C - [interrupted aortic arch type C]
  • Interruption of the aortic arch, type C
  • aortic arch interruption, type C

Exclusions from above levels

  • LA85.4 – Common arterial trunk
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA8B.21 – Coarctation of aorta
  • LA8B.23 – Congenital anomaly of descending thoracic or abdominal aorta
  • LA8B.24 – Congenital anomaly of aortic arch branch
  • LA8B.2Y – Other specified congenital anomaly of aorta or its branches
  • LA8B.2Z – Congenital anomaly of aorta or its branches, unspecified

Foundation entity

Interrupted aortic arch – http://id.who.int/icd/entity/1769930414

Part of: LA8B.2: Congenital anomaly of aorta or its branches › LA8B: Congenital anomaly of great arteries including arterial duct › 20: Developmental anomalies