Abstract

A 76-year-old male with congestive heart failure, accompanied by dyspnoea and irregular rhythm, was transferred to our hospital for treatment of congestive heart failure (NYHAIII/IV). Atrial fibrillation was confirmed by electrocardiography, and transoesophageal echocardiography (TEE) for electrical cardioversion was performed due to poor response to medical therapy. Two- and three-dimensional TEE revealed a quadricuspid aortic valve (QAV) with central leaflet malcoaptation and restriction of valve leaflet opening (Panels A and B; Supplementary data online, Videos S1 and S2). After electrical cardioversion, transthoracic echocardiography showed severe aortic stenosis with a 4.9 m/s peak aortic valve flow velocity, mean pressure gradient of 56 mm Hg (Panel C), and severe aortic regurgitation; the regurgitant volume was 61 mL/beat, and vena contracta width was 6.1 mm (Panel D; Supplementary data online, Video S3). Multi-detector row computed tomography confirmed the QAV and showed no dilatation of the ascending aorta (diameter, 34 mm) (Panels E and F). Four-dimensional flow cardiovascular magnetic resonance was confirmed that blood flow in the ascending aorta had a normal flow pattern; peak systolic wall shear stress values were not high, and symmetrical helical flow was observed (Panels G and H; Supplementary data online, Videos S4 and S5). Based on these results, TAVI was also considered, but we determined that simultaneous intervention for atrial fibrillation was necessary. Therefore, surgical aortic valve replacement (bioprosthetic valve), pulmonary vein isolation, and left atrial appendage closure were performed (Panels I and J). This case underlines the importance of multimodality approach to the diagnosis and treatment strategy of QAV.

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