Double-orifice mitral valve in Ebstein's anomaly.

Awoman 60 years oldwas referred to our department for Ebstein anomaly and atrial arrhythmias. Transthoracic echocardiogram visualized downward displacement of the septal leaflet, adherence of septal and inferior leaflets to the underlying myocardium, and redundant anterior leaflet consistent with Ebstein''s anomaly (Panel A1). Color Doppler revealed a mild-moderate degree of tricuspid regurgitation (see Supplementary data online, Movie S1) and a moderate left-right shunt through a 2 cm atrial septal defect (ASD). Moreover, a double orifice mitral valve was detected (DOMV) with no stenosis or regurgitation. This was diagnosed based on images from the apical two-chamber view (Panel A2 and see Supplementary data online, Movie S2) and the parasternal short-axis view (Panel A3). Cardiac MRI was performed to quantify the interatrial shunt and evaluate the right ventricular (RV) volumes and function. Cine MR confirmed the Ebstein anomaly. The DOMV was also visualized from cine SSFP ventricular short axis (Panel A4 and see Supplementary data online, Movie S3). A significant left-right shunt was calculated by phase velocity-contrast sequence (QP/QS:2/1). The RV was dilated, (end-diastolic volumes:142 mL/m2) while left ventricle end-diastolic volume was normal (62 mL/m2). Because of the absence of interatrial right-left shunting during exercise and an overall good functional status (NYHA I), a conservative medical follow-up was advised. Ebstein anomaly is a rare congenital heart disease frequently associated with ASD. DOMV can occur in the context of atrioventricular septal defects, left-sided obstructive lesions, and also in isolation. The occurrence of abnormalities in both atrioventricular valves is unusual; to our knowledge, only one case of DOMV and Ebstein anomaly has been reported.