Double-inlet left ventricle (DILV) includes a diverse range of anatomic variables that affect the surgical strategy. The aim of this study was to determine the impact of anatomic subtype, associated anomalies, and fetal diagnosis on the management and outcomes of fetuses and infants with DILV. The outcomes of fetuses and infants with DILV diagnosed from 1990 to 2004 at 3 major referral centers were reviewed. Sixty-five cases of DILV were detected prenatally. Twenty-one of these pregnancies were terminated, including 17 of 37 (46%) in which the diagnoses were made at < or =24 weeks of gestation. An additional 106 patients were diagnosed with DILV within the first 3 months of life. The percentage of patients diagnosed prenatally increased significantly over the study period. Transplantation-free survival was 88%, 82%, 79%, and 76% at 1 month, 1 year, 5 years, and 10 years, respectively. Factors associated with improved survival in univariate analysis included year of birth after 1994, no neonatal Norwood or Damus procedure, and no neonatal surgery at all. In multivariate analysis, any neonatal surgery was the only factor associated with worse survival. Associated anomalies and prenatal diagnosis were not associated with postnatal outcome. In conclusion, although the frequency of prenatal diagnosis of DILV has increased significantly over the past 15 years, prenatal diagnosis is not associated with better postnatal survival. During this same period, postnatal survival has improved substantially. Neonatal surgery of any type was the only independent risk factor for worse survival.