An IAA anomaly is extremely rare and represents approximately 1% of CHD. In IAA, there is complete aorta atresia somewhere along its arch, which is usually associated with a VSD and other complex cardiovascular anomalies. This defect is categorized into three types depending on where the atresia occurs. Left untreated, it is uniformly lethal within 1 week of life as a result of closure of the ductus arteriosus. In type A (30%), the aorta is interrupted between the left subclavian artery and the aortic isthmus. In type B (the most common, 65%-70%), the interruption lies between the left common carotid and the left subclavian arteries. With type C (least common type, <1%), the arch is interrupted between the innominate and the left common carotid arteries. About 50% of patients with IAA have the 22q11.2 deletion, which is usually associated with a more proximal location of the arch interruption (type B).

References

• Friedman, K. Preoperative physiology, imaging, and management of interrupted aortic arch. Semin Cardiothorac Vasc Anesth. 2018;22:265-269.
• Goldmuntz E. 22q11.2 deletion syndrome and congenital heart disease. Am J Med Genet C Semin Med Genet. 2020;184:64-72.
• Ron HA, Crowley TB, Liu Y, et al. Improved outcomes in patients with 22q11.2 deletion syndrome and diagnosis of interrupted aortic arch prior to birth hospital discharge, a retrospective study. Genes. 2023;14:1-11.
• Sanchez Mejia AA, Cambronero N, Dongarwar D, et al. Hospital outcomes among infants with interrupted aortic arch with simple and complex associated heart defects. Am J Cardiol. 2022;166:97-106.