Our aims were to examine the diagnostic accuracy of prenatal diagnosis of concordant atrioventricular and discordant ventriculo-arterial connections, the accuracy of predictions made concerning the postnatal surgical approach, and a description of the different subtypes related to outcome. All fetuses were evaluated at a tertiary centre for fetal cardiology between January, 1994, and December, 2003. In this period, we identified congenitally malformed hearts in 1,835 fetuses, of whom 56 (3%) met the criterions of inclusion. Of the total, 30 (54%) had an intact ventricular septum, 9 (16%) had an associated ventricular septal defect, 7 (13%) had a ventricular septal defect and pulmonary stenosis, 1 (2%) had pulmonary stenosis and an intact ventricular septum, 8 (14%) had a ventricular septal defect and aortic coarctation, and 1 (2%) had coarctation of the aorta with an intact ventricular septum. All the discordant ventriculo-arterial connections were correctly identified. For associated ventricular septal defects, the diagnostic sensitivity was 96%, with specificity of 88%, positive predictive value of 85%, and negative predictive value of 97%. For aortic coarctation, the sensitivity was 100%, specificity 96%, positive predictive value 82%, and negative predictive value 100%. The prediction of the surgical approach was accurate in 41 of 48 cases (85%). For those fetuses with pulmonary stenosis and ventricular septal defect, the ratio of the diameters of the pulmonary trunk was shown to be helpful in predicting the possibility of an arterial switch as opposed to the Rastelli type of repair. Of the 49 liveborn infants, 46 were alive at 30 days (94%, with 95% confidence intervals from 83 to 99%), and 43 at one year (88%, 95% confidence intervals from 75 to 95%). Deaths were mainly related to the anatomy of the coronary arteries, and associated cardiac lesions.