pediatrics
Ebstein Anomaly
Diagnosis of Ebstein Anomaly
Chest x-ray and ECG Echocardiography MRI The ECG is usually abnormal with right axis deviation, low voltage right bundle branch block, and first-degree atrioventricular block. Wolff-Parkinson-White (WPW) syndrome is present in 20% of patients. Chest x-ray findings include a variable degree of cardiomegaly with prominent right atrial borders. A subset of infants with severe Ebstein anomaly have massive cardiomegaly, referred to as a wall-to-wall heart shadow. Echocardiography clearly delineates the anatomic and functional abnormalities of the tricuspid valve and any associated defects. MRI provides valuable additional detail regarding the tricuspid valve, which is helpful in planning surgical intervention. 