Outcomes for hypoplastic left heart syndrome have improved substantially in the era of prenatal diagnosis. Current stage 1 survival rates are in excess of 90% in the absence of risk factors. However, fetuses with an intact atrial septum continue to have poor postnatal survival. Accurate diagnosis of these fetuses with an intact atrial septum relies upon careful assessment of the patent foramen ovale, pulmonary venous Doppler flow patterns and branch pulmonary artery Doppler flow patterns. Prenatal and perinatal interventions, such as the placement of an atrial stent in utero or delivery at a center where a stent may be placed immediately after birth to relieve left atrial hypertension, may improve survival in this particularly high-risk group.