Background--Pulmonary venous Doppler (PVD) flow patterns in the fetus with hypoplastic left heart syndrome (HLHS) have been correlated with restrictive interatrial communication or intact atrial septum (RAS) postnatally; however, the ability of PVD to identify the neonate requiring emergent atrial septoplasty (EAS) for severe left atrial hypertension and hypoxemia has not been critically evaluated. It was the purpose of this study to determine the predictive power of fetal PVD in identifying the need for EAS in newborns with HLHS and RAS.

Methods and Results--Forty-one patients with fetal PVD flow analysis and postnatally confirmed HLHS were studied. Pulsed-wave assessment of PVD flow included S-, D-, and A-wave velocity, time-velocity integral (VTI) of forward and reverse flow, and S/D velocity and forward/reverse VTI ratio. Neonatal EAS was used as the primary clinical outcome variable. Receiver operating characteristic curves were used to determine cutpoints at which PVD indices best predicted EAS. Cutpoints were evaluated for clinical accuracy and usefulness by use of Bayesian analysis. Eight of 41 subjects underwent EAS. Need for EAS was most accurately predicted by forward/reverse VTI ratio <5 (sensitivity, 0.88, 95% CI, 0.49 to 0.99; specificity, 0.97, 95% CI, 0.82 to 0.99), which, when present, increases the posttest likelihood of EAS to 74%, assuming a pretest prevalence of 10%. Accuracy and usefulness of other PVD indices were affected by false-positive results.

Conclusions--In the fetus with HLHS, a PVD forward/reverse VTI ratio of <5 is the strongest predictor of the need for EAS in the newborn period. These observations should improve our ability to identify and expectantly manage the fetus with HLHS and RAS.