Objective This study was carried out to evaluate the additional predictive value of ductus venosus pulsatility index for veins (DV-PIV) in the identification of congenital heart defects (CHDs) in fetuses with an enlarged nuchal translucency (NT) and a normal karyotype. Methods All chromosomally normal fetuses referred to our Fetal Medicine Unit between September 1996 and December 2008 with known NT, DV-PIV and ductus venosus (DV) a-wave measurements were included. Intrafetus variation in DV-PIV was overcome by averaging three recordings. Follow-up included special focus on CHD. The odds of CHD at any NT and DV-PIV value were evaluated using logistic regression analysis. Results Of 792 fetuses included, the NT was enlarged (equal to or above the 95th percentile (P95)) in 318 (40.2%). The DV-PIV was abnormal (≥ P95) in 41.8% of the fetuses with an enlarged NT and the a-wave was abnormal (negative or reversed) in 29.9%. CHD was diagnosed in 35 fetuses, 33 of which had an enlarged NT. Amongst the fetuses with an enlarged NT, the sensitivities for CHD of abnormal DV-PIV and DV a-wave were 73% and 55%, with specificities of 62% and 73%, respectively. Logistic regression analysis showed that in this risk group the DV-PIV multiple of the median (MoM) (as a continuous variable) was significantly associated with the risk of CHD (odds ratio = 2.4), independent of the degree of NT enlargement, whereas the DV a-wave did not significantly add to the prediction of CHD. Conclusion Two-thirds of fetuses with an enlarged NT, a normal karyotype and CHD have an increased DV-PIV. DV-PIV can be used as continuous variable in combination with NT to increase specificity in the identification of CHD and to refine the individual risk assessment. Copyright © 2010 ISUOG. Published by John Wiley & Sons, Ltd.