Internal view of the right chambers of a heart with Ebstein's anomaly with mild (Grade I) tethering of the septal leaflet (asterisk). Thickening of the free portion of the leaflet (arrow) is evident. The dotted line represents the atrioventricular junction where the tricuspid fibrous ring is located. The atrialized portion of the right ventricle is small. Note the patent foramen ovale type atrial septal defect. The 4 chamber echocardiographic image shows the same type of findings seen in the anatomic specimen. The dotted line indicates the plane of atrioventricular junction. The majority of the right ventricle is functional. Abbreviations: RA: Right atrium; ARV: Atrialized right ventricle; FRV: Functional right ventricle; AL: Anterior leaflet; PL: Posterior leaflet; SL: Septal leaflet; ASD: Atrial septal defect; LA: Left atrium; LV: Left ventricle.