Ultrasonic features of fetal ebstein anomaly

Objectives: We analyzed and summarized the ultrasonic and anatomical features of fetal Ebstein anomaly to improve the ultrasonographic diagnostic rate. Methods: We analyzed the fetal echocardiographs and either the postnatal echocardiographs or anatomical specimens of 13 cases of fetal Ebstein anomaly. The cases were divided into typical and rare types according to the features of the Ebstein anomaly lesions. Results: Among the 13 cases of Ebstein anomaly examined, 10 cases were typical, and 3 cases were rare types. The typical features of Ebstein anomaly included a downward displacement of the septal and posterior tricuspid leaflets, which were shortened, while the anterior tricuspid leaflet was long. In addition to the typical pathological changes, the rare forms exhibited unique characteristics. The rare forms include an absence of the chordae tendineae and papillary muscle, valve thickening and Ebstein anomaly associated with ventricular noncompaction. Conclusions: Ebstein anomaly is characterized by extensive pathological changes involving the tricuspid valve, chordae tendineae, and papillary and cardiac muscles. According to the features of these lesions, Ebstein anomaly has been divided into typical and rare types. Understanding the ultrasonic features of the different types of Ebstein anomaly can improve the ultrasonographic diagnostic rate of this disease.