title: Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect.
abstract: OBJECTIVE
  Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management 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-scores of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure.
  
  
  RESULTS
  Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% +/- 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%).
  
  
  CONCLUSIONS
  Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach.
authors.affiliation: Division of Pediatric Cardiovascular Surgery, Section of Cardiac Surgery, C.S. Mott Children's Hospital, University of Michigan School of Medicine, Ann Arbor, USA.
authors.forename: R G
authors.initials: RG
authors.lastname: Ohye
authors.forename: K
authors.initials: K
authors.lastname: Kagisaki
authors.forename: L A
authors.initials: LA
authors.lastname: Lee
authors.forename: R S
authors.initials: RS
authors.lastname: Mosca
authors.forename: C S
authors.initials: CS
authors.lastname: Goldberg
authors.forename: E L
authors.initials: EL
authors.lastname: Bove
mesh_terms: D000206:Actuarial Analysis; D000714:Anastomosis, Surgical / Q000009:adverse effects; D001011:Aorta / Q000002:abnormalities; D001017:Aortic Coarctation / Q000150:complications; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D002423:Cause of Death; D003914:Dextrocardia / Q000150:complications; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities; D013530:Surgical Wound Infection / Q000209:etiology; D015996:Survival Rate; D014188:Transposition of Great Vessels / Q000150:complications; D016896:Treatment Outcome