Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate

To determine which morphologic features are associated with early death, the complete echocardiograms and medical records of 16 consecutive patients with Ebstein's anomaly and concordant atrioventricular connections who presented in the fetal (n = 5) or neonatal (n = 11) period were reviewed. The cohort was classified into two groups on the basis of survival at 3 months. Group 1 consisted of seven patients who died at less than or equal to 3 months of age, and Group 2 consisted of the nine surviving patients. Comparing Groups 1 and 2, the respective incidence rates of morphologic features that correlated with early death (p less than 0.05) included tethered distal attachments of the anterosuperior tricuspid leaflet (86% versus 11%), right ventricular dysplasia (86% versus 0%), left ventricular compression by right heart dilation (71% versus 11%) and the area of the combined right atrium and atrialized right ventricle being greater than the combined area of the functional right ventricle, left atrium and left ventricle (57% versus 0%) measured in the apical four chamber view. Right ventricular dysplasia was present in all patients with marked right atrial and atrialized right ventricular enlargement, in 86% of patients with tethered anterior leaflets and in 83% of those with left ventricular compression; 86% of patients with right ventricular dysplasia had tethered distal attachments. In conclusion, echocardiography defines morphologic features in the fetus and neonate that are highly predictive of death by 3 months of age.