RESUMEN
Congenitally corrected transposition of the great arteries (CCTGA) in the absence of major cardiac anomalies is thought to have a good outcome, although this has not been well documented. The objective of the present study was to compare the characteristics and outcomes of patients with a prenatal diagnosis of CCTGA to the characteristics and outcomes of those diagnosed postnatally. The optimal outcome was defined as intervention-free survival. All patients with CCTGA diagnosed prenatally and postnatally from 1999 to 2006 at 2 tertiary care institutions were reviewed. Patients with a single ventricle, heterotaxy, or valvar atresia were excluded. The differences between groups were assessed using the t test and chi-square test. A total of 54 patients (16 prenatal with 14 live born and 39 postnatal) were included. The patients diagnosed prenatally were diagnosed at a median gestational age of 20 weeks (range 16 to 37). Two deaths in each group were due to heart failure. The intervention-free survival rate for the prenatal and postnatal groups at 1, 6, and 36 months was 79%, 45%, and 30% and 85%, 61%, and 23%, respectively (p = NS). Of 37 patients, 14 (38%) underwent an arterial switch plus atrial baffling so that the morphologic left ventricle supported the systemic circulation, and 6 (16%) underwent repair of associated lesions so the morphologic right ventricle supported the systemic circulation; 4 (11%) of the 37 patients had pacemaker only. Prenatal patients with >1 fetal echocardiogram (12 of 14) did not have progression before birth. In conclusion, CCTGA has a >70% risk of intervention in the first 3 years after birth. The outlook is guarded and has an important effect on prenatal counseling.