RESUMO
OBJECTIVE: To report an extremely rare case of atrioventricular discordance and ventriculoarterial concordance associated with a ventricular septal defect which was diagnosed prenatally. CASE REPORT: By fetal echocardioraphy at 20 weeks' gestation, we diagnosed a rare case of atrioventricular discordance and ventriculoarterial concordance associated with a ventricular septal defect. This is the first case reported from Vietnam prenatally. We present our management of this pregnancy and the baby's neonatal course. This rare anomaly remains a challenge for the baby's early neonatal course before initial neonatal discharge. CONCLUSION: A combined multidisciplinary and individualized approach for the optimal management of this complicated pregnancy and further neonatal surgical treatment plans for the baby are recommended.