Outcomes using predominantly single-stage approach to interrupted aortic arch and associated defects

Neonatal repair of interrupted aortic arch (IAA) involves an early choice between a single-stage or two-stage strategy. Risk factors for each are not yet fully investigated, especially as they relate to major associated cardiac malformations. We aimed to assess the outcome of neonates undergoing biventricular repair of IAA and associated congenital heart defects.

Preoperative assessment, operative management, and outcome were retrospectively reviewed for 18 consecutive patients undergoing biventricular IAA repair at Starship Children's Hospital from 2000 to 2005.

Seventeen patients underwent a single-stage procedure and one patient weighing 970 g underwent a two-stage procedure. All but one had a ventricular septal defect. Major associated cardiac defects were present in 7 and included aortopulmonary window (1), truncus arteriosus (3), transposition of the great arteries (1), and aortic valve atresia (2). Those with major associated cardiac defects had longer procedural times but similar early mortality and intensive care unit and hospital stay. One patient required a pacemaker for complete heart block. Mean follow-up was 4.5 years with one late death and all survivors reporting normal functional status. Developmental delay was present in 5 (27%), 4 of whom had 22q deletion. Late reoperation was required in 4, including two Konno procedures and two pulmonary conduit changes.

A good functional outcome and low reoperation rate can be achieved with a single-stage repair regardless of the presence of major additional cardiac abnormalities. Neonates with risk factors such as low birth weight and prematurity require an individualized approach.