OBJECTIVES To determine the accuracy of prenatal diagnosis of pulmonary atresia and intact ventricular septum (PAIVS), and pulmonary stenosis, including prenatal detection of ventriculocoronary connections, to evaluate heart size during the prenatal period, and to evaluate the outcome.

DESIGN AND PATIENTS Medical records of 20 cases with prenatally diagnosed PAIVS and pulmonary stenosis were reviewed retrospectively. Prenatal and postnatal echocardiography were also reviewed and dimensions of the ventricles and vessels were measured retrespectively.

RESULTS Of 20 prenatal diagnoses (15 PAIVS and five pulmonary stenosis), 16 were confirmed as correct. One critical pulmonary stenosis case had been diagnosed as PAIVS prenatally; three had no confirmation. Eight pregnancies were terminated, three had no active treatment, and nine were treated; all survived. Of 13 assessed with ventriculocoronary connections prenatally, seven were diagnosed correctly (four with, three without ventriculocoronary connections), but one was falsely positive; five had no confirmation. The more prominent hypoplasia of the main pulmonary artery and the tricuspid valve annulus, and the sigmoid shape of the ductus arteriosus, seemed to be associated with the presence of ventriculocoronary connections.

CONCLUSIONS Current prenatal echocardiography can accurately diagnose right ventricular outflow tract obstruction and ventriculocoronary connections. Prenatal detection of this constellation of abnormalities aids in family counselling and decisions on postnatal management.