Aortic atresia with a ventricular septal defect: modified single-stage neonatal biventricular repair

The spectrum of hypoplastic left heart disease is diverse but the surgical repair is strictly dichotomous, culminating in either a univentricular or a biventricular surgical repair. Although aortic atresia with a ventricular septal defect historically has been managed by conversion to a univentricular physiology, a biventricular repair occasionally has been attempted in stages or in conjunction with the implantation of multiple allografts or prosthetic conduits. Our repair strategy recently has evolved to the use of a modified single-stage biventricular repair using only autologous tissues without conduits.

Retrospective analysis (1989 to 1997) of neonates with aortic atresia with a ventricular septal defect.

Five neonates underwent repair of aortic atresia with a ventricular septal defect. One died in the hospital. The mean age and weight of the neonates who underwent repair were 7.8 days (range, 2 to 17 days) and 3.2 kg (range, 3 to 3.6 kg), respectively. Three neonates had a univentricular repair and 2 had a modified biventricular repair. The latter two procedures were successful and the patients were discharged from the hospital.

Long-term results are lacking to attest to this surgical modification's superiority over either the standard multistage univentricular operation or the single-stage biventricular repair using multiple conduits. However, we are optimistic that routine use of this modification will enable a greater percentage of neonates to undergo a biventricular repair without the need for serial conduit revisions or future aortoplasty.