Ebstein’s Anomaly and Echocardiographic Features

Aim: To present the various echocardiographic spectrum of Ebstein’s malformation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year old aymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an ‘atretic’ membrane in a 28-year old cyanotic male and as a ‘blanket’ of leaflet tissue in a 30-year old cyanotic male. Conclusion: Ebstein’s anomaly has to be suspected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.