Unicuspid Aortic Valve Presenting With Severe Aortic Regurgitation and Successfully Repaired by Bicuspidization

Figures

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  Figure 1. Transthoracic Echocardiography Demonstrating Left Ventricular Enlargement Transthoracic echocardiography showing left ventricular enlargement in parasternal long-axis and apical 4-chamber views at end-diastole and end-systole.

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  Figure 2. Severe Aortic Regurgitation on Color Doppler Imaging Color Doppler imaging demonstrating severe aortic regurgitation with an eccentric jet directed toward the anterior mitral leaflet.

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  Figure 3. Transthoracic Echocardiography Suggesting Bicuspid Aortic Valve Parasternal short-axis view at end-diastole and end-systole suggesting a bicuspid aortic valve with right and left coronary cusp fusion (arrow indicating the fused commissure).

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  Figure 4. Transesophageal Echocardiography Revealing Unicuspid Aortic Valve Transesophageal echocardiography demonstrating severe aortic regurgitation with an eccentric regurgitant jet and a unicommissural unicuspid aortic valve with a single commissure between the left coronary cusp and the noncoronary cusp (red arrow), and 2 raphes between the right and left coronary cusps (white arrow) and between the right coronary cusp and the noncoronary cusp (white dotted arrow).

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  Figure 5. 3-Dimensional Transesophageal Echocardiography Confirming Valve Morphology 3-dimensional transesophageal echocardiography demonstrating unicuspid aortic valve morphology with a single commissure (red arrow) and 2 raphes (white arrow and white dotted arrow).

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  Figure 6. Computed Tomographic Assessment of the Unicuspid Aortic Valve and Aorta Three-dimensional computed tomographic reconstruction demonstrating unicuspid aortic valve morphology, consistent with the transesophageal echocardiographic findings (green arrow: single commissure; white arrow and white dotted arrow: 2 raphes). Computed tomography also demonstrated dilation of the sinus of Valsalva, sinotubular junction, and ascending aorta.

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  Figure 7. Intraoperative Findings and Surgical Procedure (A) Intraoperative view of the aortic valve demonstrating unicuspid morphology with a single commissure between the left coronary cusp and the noncoronary cusp (red arrow), and 2 raphes between the right and left coronary cusps (white arrow) and between the right coronary cusp and the noncoronary cusp (white dotted arrow), consistent with the preoperative transesophageal echocardiography findings. (B and C) Bicuspidization of the unicuspid aortic valve creating a symmetric bicuspid configuration. (D) Valve-sparing aortic root replacement using the reimplantation technique.

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  Visual Summary. Diagnostic and Surgical Strategy for Unicuspid Aortic Valve Presenting With Severe Aortic Regurgitation AR = aortic regurgitation; BAV = bicuspid aortic valve; LV = left ventricular; LVEF = left ventricular ejection fraction; TEE = transesophageal echocardiography; TTE = transthoracic echocardiography; UAV = unicuspid aortic valve.