title: Aortic atresia or severe left ventricular outflow tract obstruction with ventricular septal defect: results of primary biventricular repair in neonates.
abstract: BACKGROUND
  Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction is a frequent component of complex congenital heart disease. Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction with two adequate ventricles is sometimes treated by Norwood palliation followed by late biventricular repair. We reviewed our experience with primary biventricular repair in this group of neonates.
  
  
  METHODS
  Retrospective review identified 17 neonates (10 males) with aortic atresia or severe left ventricular outflow tract obstruction with ventricular septal defect and an adequate left ventricle undergoing primary biventricular repair between 1986 and 2002. Mean age was 7.7 +/- 2.9 days, weight 3.3 +/- 0.7 kg, and body surface area 0.21 +/- 0.04 kg/m2. Associated anomalies included arch hypoplasia, 7 (41%); aortic atresia, 7 (41%); and coarctation, 5 (29%). Results are reported as mean +/- standard deviation.
  
  
  RESULTS
  Median follow-up was 6 years (range, 1 to 17.7 years). Three of the 17 (18%) died within 30 days. There were no deaths in this series since 1992. Nine patients (38.9%) required one reoperation, 7 of which were for conduit stenosis, 1 for left ventricular outflow tract obstruction, and 1 for residual ventricular septal defect with left ventricle-to-right atrium shunt. Freedom from death at 10 years was 82% by Kaplan-Meier estimate.
  
  
  CONCLUSIONS
  Excellent long-term survival can be achieved by primary biventricular repair as corroborated by our survival rate of 82%. Primary biventricular repair is an effective operation for aortic atresia and severe left ventricular outflow tract obstruction with adequate sized left ventricle that avoids interstage attrition associated with Norwood palliation and is our procedure of choice.
authors.affiliation: Department of Cardiac Surgery, Harvard Medical School, Children's Hospital Boston, Boston, Massachusetts 02115, USA.
authors.forename: Meena
authors.initials: M
authors.lastname: Nathan
authors.forename: David
authors.initials: D
authors.lastname: Rimmer
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.forename: Andrew
authors.initials: A
authors.lastname: Shin
authors.forename: William
authors.initials: W
authors.lastname: Regan
authors.forename: Rodrigo
authors.initials: R
authors.lastname: Gonzalez
authors.forename: Frank
authors.initials: F
authors.lastname: Pigula
mesh_terms: D001021:Aortic Valve / Q000002:abnormalities; D001024:Aortic Valve Stenosis / Q000150:complications / Q000601:surgery*; D006348:Cardiac Surgical Procedures / Q000401:mortality*; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000601:surgery*; D006349:Heart Valve Diseases / Q000151:congenital / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014694:Ventricular Outflow Obstruction / Q000150:complications / Q000601:surgery*