Abstract
Neonates with critical left heart obstruction and intact atrial septum (IAS) or restrictive atrial septum (RAS) are at risk for hypoxia within hours of birth and remain a group at high risk for mortality. Prenatally diagnosed fetuses with critical left heart obstruction and IAS or RAS with follow-up from January 1, 2005, to February 14, 2017, were included. Primary outcome was a composite measure of severe neonatal illness (pH<7.15, venous pH<7.10, bicarbonate<16mmol/L, lactic acid>5mmol/L, or median oxygen saturation<60% within 2hours of birth). Of 68 live born fetuses, 52 (76.5%) had hypoplastic left heart syndrome, 14 (20.5%) had critical aortic stenosis, and two (3%) had complex anatomy with mitral stenosis/atresia. There were 27 (39.7%) fetuses with IAS and 41 (60.3%) with RAS. Severe neonatal illness was present in 36 (52.9%). The strongest discriminators for severe neonatal illness were a pulmonary vein A:R VTI≤2.7 (P<0.001, AUC 0.93) and larger pulmonary vein diameter (P=0.025, AUC 0.77). A:R VTI≤2.7 predicted death or transplant (log-rank P=0.03). In neonates with hypoplastic left heart syndrome and IAS or RAS, A:R VTI≤2.7 is predictive of severe neonatal instability. This threshold can help guide resource planning, delivery management, and improve fetal intervention criteria.
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