Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum

In this prospective study, 27 consecutive neonates suspected to be suffering from pulmonary atresia and intact ventricular septum underwent detailed two-dimensional echocardiographic examination before cardiac catheterization. Of the 27 neonates 25 had pulmonary atresia and intact ventricular septum and the remaining 2 had "functional pulmonary atresia" secondary to severe Ebstein's anomaly of the tricuspid valve. In all 25 neonates with pulmonary atresia and intact ventricular septum, the diagnosis and right ventricular morphology based on the tripartite approach were correctly established by echocardiography. The associated Ebstein's anomaly in two babies with pulmonary atresia and intact ventricular septum was also correctly identified by echocardiography. Among the five babies who had a sinusoidal-coronary artery communication, echocardiography demonstrated the fistula in one and provided clues for its diagnosis in two others. In the 25 neonates with pulmonary atresia and intact ventricular septum, the echocardiographic dimensions of their tricuspid anulus, right ventricular infundibulum and main pulmonary artery correlated well with the angiocardiographic measurements (r greater than 0.8). The results of this study suggest that, in the management of neonates with pulmonary atresia and intact ventricular septum, preoperative evaluation by echocardiography is usually sufficient and cardiac catheterization should be reserved for selected cases.