Surgical Management of Interrupted Aortic Arch with Associated Anomalies

Interrupted aortic arch (IAA) is a rare congenital malformation characterised by loss of continuity between the ascending and descending aorta. It typically presents in the neonatal period and is frequently accompanied by ventricular septal defect (VSD), bicuspid aortic valve and left ventricular outflow tract obstruction (LVOTO). The primary goal of surgical management is to re-establish unobstructed systemic flow while addressing associated lesions in a single stage whenever feasible. Standard techniques involve direct end-to-end anastomosis of the aortic segments, closure of VSD and enlargement of the obstructed left ventricular outflow tract, often under hypothermic circulatory arrest or selective antegrade cerebral perfusion for organ protection. In complex anatomies such as aortic atresia or hypoplastic left heart variants, biventricular approaches including the Yasui procedure or hybrid strategies combining surgical debranching with endovascular stenting are gaining traction. Risk factors for residual or recurrent obstruction include small pre-operative aortic dimensions, aberrant subclavian arteries and use of synthetic interposition grafts. Long-term outcomes hinge on meticulous follow-up to detect valve dysfunction, arch re-narrowing and conduit degeneration, emphasising the need for multidisciplinary lifelong care.

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Surgical Management of Interrupted Aortic Arch with Associated Anomalies publication trend

The graph below shows the total number of articles in surgical management of interrupted aortic arch with associated anomalies across all publications each year (not limited to Nature Index journals).

Technical terms

Interrupted aortic arch: congenital absence of anatomical continuity between the ascending and descending aorta.

Ventricular septal defect (VSD): an opening in the interventricular septum allowing shunting between left and right ventricles.

Left ventricular outflow tract obstruction (LVOTO): narrowing of the pathway from the left ventricle to the aorta, impeding systemic flow.

Yasui procedure: a biventricular repair combining a VSD closure and a right ventricle–to–aortic continuity conduit, often used in aortic atresia with arch interruption.

Bicuspid aortic valve: a two-leaflet aortic valve variant associated with increased risk of stenosis or regurgitation.

References

1. Biventricular repair in low-weight patient with interrupted aortic arch and aortic atresia. The Egyptian Heart Journal (2024).
2. Predictors of Left Ventricular Outflow Tract Obstruction After Conventional Repair for Patients with Interrupted Aortic Arch or Coarctation of the Aorta, Combined with Ventricular Septal Defect: A Single-Center Experience. Pediatric Cardiology (2021).
3. The association of bicuspid aortic valve on long-term outcome following one-stage repair of aortic arch obstruction associated with ventricular septal defect. Cardiology in the Young (2022).