ABSTRACT. Background. By decreasing preoperative morbidity, prenatal diagnosis could improve neurodevelopmental outcomes in infants with critical congenital heart disease. We explored the impact of prenatal diagnosis on perinatal and perioperative variables and on outcomes at 1 year of age.Methods. We analyzed a database of children enrolled in prospective studies on surgical support techniques from 1988 to 2000. Selection criteria included a diagnosis of D-transposition of the great arteries with intact ventricular septum or ventricular septal defect, no extracardiac congenital anomalies, birth weight >2.3 kg, and repair by arterial switch procedure.Results. Of 346 patients at enrollment, 25 had a prenatal diagnosis, and 321 did not. Children with prenatal diagnosis, compared with those without, had a lower likelihood of birth by spontaneous labor, lower birth weights, lower Apgar 5 scores, a higher rate of preoperative endotracheal intubation, and surgery at a younger age. They tended to have a lower incidence of fetal distress during labor. At 1 year of age, 272 patients were tested with the Psychomotor Development Index and Mental Development Index of the Bayley Scales. Mean z scores were similar in those with and without prenatal diagnosis for both Psychomotor Development Index (؊0.92 ؎ 0.93 vs ؊0.88 ؎ 1.05) and Mental Development Index (؊0.29 ؎ 1.13 vs ؊0.41 ؎ 0.93).Conclusions. Infants with D-transposition of the great arteries with and without prenatal diagnosis differed with respect to perinatal and perioperative variables, but their development at 1 year of age was similar. Future studies should include a greater number of children with prenatal diagnosis and a variety of congenital heart lesions. Pediatrics 2004;113:e335-e340. URL: http: //www.pediatrics.org/cgi/content/full/113/4/e335; congenital heart disease, transposition of the great arteries, cardiac surgery, child development, prenatal diagnosis.ABBREVIATIONS. CHD, congenital heart disease; D-TGA, Dtransposition of the great arteries; IVS, intact ventricular septum; VSD, ventricular septal defect; PDI, Psychomotor Development Index; MDI, Mental Development Index; SD, standard deviation. P renatal echocardiography is being performed with increasing frequency to identify complex congenital heart disease (CHD) in the fetus. It has been hypothesized that prenatal diagnosis of CHD might allow for optimal medical management in the newborn period, thereby improving surgical and neurologic outcomes. 1 Previous studies have examined the impact of a prenatal diagnosis on neonatal mortality and morbidity. 2,3 However, advances in surgical technology and perioperative management have improved survival of children born with CHD, thereby heightening interest in their subsequent developmental outcomes. 4 Presently, data are limited regarding the impact of prenatal diagnosis on longer-term cognitive development in children born with CHD.Prenatal diagnosis of D-transposition of the great arteries (D-TGA) allows a controlled environment for delivery, timely tran...