Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect

Objective: Aortic valve atresia or hypoplasia can present with a ventricula r septal defect and a normal mitral valve and left ventricle, These patient s may be suitable for biventricular repair, although the optimal initial ma nagement strategy remains unknown. Methods: From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair , Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-sco res of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomali es included interrupted aortic arch (n = 12 patients), coarctation (n = 6 p atients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 pat ient). Nine patients mere staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular re pair after a Norwood procedure. The conditions of II patients were correcte d,vith a single procedure. Results: Among the 9 patients who underwent stag ed repair, there were no deaths after the Norwood procedure and 1 death aft er biventricular repair. For the 11 patients who underwent a primary bivent ricular repair, there was 1 early death and 2 late deaths from noncardiac c auses. Follow-up ranged from 1 to 85 months (mean, 28 months), Actuarial su rvival for the entire group was 78% +/- 10% at 5 years and was nat signific antly different between staged repair (89%) and primary biventricular repai r (73%), Conclusions: Both primary and staged biventricular repair for pati ents with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit ins ertion and arch reconstruction have resulted in primary biventricular repai r becoming our preferred approach.