Fetal Ebstein Anomaly: From Integrated Echocardiographic Assessment to Evolving Prognostic Scoring Models

Ebstein anomaly is a rare congenital cardiac malformation with a highly variable prenatal course, ranging from stable physiology to progressive fetal heart failure and perinatal death. Predicting outcome remains difficult because anatomical severity alone does not reliably reflect physiological burden, ventricular performance, or disease progression. Therefore, fetal echocardiography plays a central role in both diagnosis and risk stratification. This review summarises the current approaches to fetal echocardiographic assessment of Ebstein anomaly, focusing on anatomical, functional, and Doppler-derived markers associated with adverse outcomes, including severe tricuspid regurgitation, cardiomegaly, right and left ventricular dysfunction, abnormal pulmonary and ductal flow patterns, and circular shunt physiology. Established prognostic scoring systems were reviewed, highlighting their contributions to risk stratification. Emerging functional parameters and serial assessment strategies have been discussed as complementary tools to better capture the dynamic nature of the disease.