Interrupted Aortic Arch
Also written as: IAA — Interrupted Aortic Arch
A rare congenital heart defect characterized by a complete discontinuity of the aortic arch, requiring ductal-dependent systemic circulation.
Full Definition
Interrupted aortic arch (IAA) is a rare and severe congenital heart defect involving complete anatomical discontinuity of the aortic arch between the ascending and descending portions. The condition is classified into three types based on the location of interruption: Type A (distal to left subclavian artery), Type B (between left carotid and left subclavian arteries), and Type C (between brachiocephalic and left carotid arteries). IAA is frequently associated with ventricular septal defect, bicuspid aortic valve, and DiGeorge syndrome. Newborns are dependent on ductal flow for systemic perfusion distal to the interruption and present with severe congestive heart failure when the ductus arteriosus closes. Emergency surgical repair is required in the neonatal period.
Usage
Usage note: Always specify the anatomical type (A, B, or C) and associated genetic syndromes when present.
In Context
- "Type B interrupted aortic arch was diagnosed in association with a large ventricular septal defect." — Neonatal cardiology consultation
- "Emergency repair of interrupted aortic arch was performed using a direct anastomosis technique." — Congenital cardiac surgery operative report