State of the ArtManagement of Interrupted Aortic Arch

The introduction of prostaglandin E1 in the late 1970s revolutionized the management of interrupted aortic arch. Complete resuscitation should proceed over several days if necessary before surgery is undertaken. One-stage primary neonatal repair with direct arch anastomosis and ventricular septal defect closure is the preferred surgical approach. Selective cerebral perfusion with near-infrared monitoring is being used with increasing frequency. Although repair of interrupted arch is physiologically corrective, it should not be viewed as fully corrective because of the high incidence of important late left ventricular outflow tract obstruction. This may respond to a simple surgical reintervention such as subaortic resection, but in some cases, an extensive procedure to enlarge the left ventricular outflow tract is necessary. However, procedures directed against subaortic stenosis should rarely be used as part of the initial surgical management during the neonatal period. Careful developmental follow-up is needed for all patients because of the high incidence of DiGeorge syndrome, which frequently manifests as moderately severe developmental delay.