https://orcid.org/0000-0002-8599-2373
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Sitali Mushemi-Blake, Elena Surkova, Suzan Hatipoglu, Roxy Senior, Wei Li, Severe regurgitation of a double-orifice left atrioventricular valve in a patient with repaired atrioventricular septal defect: added value of 3D echocardiography, European Heart Journal - Cardiovascular Imaging, Volume 21, Issue 7, July 2020, Page 814, https://doi.org/10.1093/ehjci/jeaa005
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A 33-year-old woman with previously repaired partial atrioventricular septal defect and mild unrepaired coarctation of the aorta was admitted to our institute with worsening heart failure symptoms following her second successful pregnancy. Transthoracic echocardiography provided a diagnosis of severely dilated left ventricle (LV) with ejection fraction of 43%, severely dilated left atrium (LA; Panels A and J), severe left atrioventricular valve (LAVV) regurgitation comprising of multiple jets and mildly increased diastolic gradients through the valve (Panels B–D). 3D echocardiographic (3DE) rendering of LAVV from ventricular and atrial aspects demonstrated double-orifice valve (asterisks) with excessive bridging tissue (Panels E and F, respectively; Supplementary data online, Videos S1 and S2). There was severe prolapse of the whole of anterior leaflet through the anterior orifice and bulky appearance of the posterior leaflet (Panel G, arrows, Supplementary data online, Video S2) resulting in three jets of regurgitation (Panel H, arrows). Further investigation with cardiac magnetic resonance imaging confirmed 3DE findings showing double-orifice LAVV with severe regurgitation, severely dilated LV and LA (Panels I and J, asterisks; Supplementary data online, Videos S3 and S4). The patient underwent surgical replacement of LAVV with a 31-mm St. Jude Medical prosthesis. Post-operative course was uneventful and follow-up echocardiography showed a well-seated prosthetic valve with mild transvalvular regurgitation and no LV outflow tract obstruction. Double-orifice LAVV is a rare congenital diagnosis, which can result in both stenosis and regurgitation. 3DE allowed detailed morphological assessment of the LAVV and surrounding structures, visualization of the orifices and prolapsing leaflets, understanding the mechanism of regurgitation, and facilitated management strategy.
