LA8B.22 Interrupted aortic arch

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01


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.

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