Aortic Valve Dysfunction and Aortopathy Based on the Presence of Raphe in Patients with Bicuspid Aortic Valve Disease

Figures

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  Figure 1. Bicuspid aortic valve classification: valve type according to the absence (A,B) or presence (arrow, C,D) of a raphe on cardiac CT in double-oblique short-axis plane in the systole.

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  Figure 2. Example of the measurement of the aortic dimensions at different locations. Oblique sagittal reformatted CT image shows the sinus of Valsalva and tubular portion of the ascending aorta at mid-systole (A). The maximum dimension of the tubular portion of the ascending aorta (white line) was measured in a double-oblique transverse view (B) obtained perpendicular to the aortic lumen (C,D) because the axial measurement exceeded the true measurement perpendicular to the long axis of the ascending aorta. The maximal aortic root dimension (white line) was measured sinus-to-sinus in a bicuspid aortic valve with a raphe (E) in a double oblique transverse view obtained perpendicular to the aortic sinus of Valsalva (F,G).

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  Figure 3. Diameters of the ascending aorta measured via cardiac computed tomography at two different levels (sinus of Valsalva [white arrow] and tubular portion [black arrow]) at mid-to-end systole. The ascending aorta was assigned to one of four main anatomical types according to the segment of the vessel exclusively or predominantly involved in dilatation based on the indexed maximal aorta diameter of exceeding 21 mm/m2 of body surface area: (A) normal; (B) type 1, root dilatation; (C) type 2, ascending aorta dilatation; (D) type 3, combined dilatation of the aortic root and middle ascending aorta.

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  Figure 4. Bicuspid aortic valve (BAV) with a raphe with ascending aorta root dilatation in a 48-year-old male. (A) Double-oblique CT reconstruction parallel to the aortic valve during systole demonstrates a BAV with fusion of the right and left coronary cusps. (B) Double-oblique coronal CT reconstruction through the left ventricular outflow tract during systole shows the diameters of the sinuses of Valsalva (white arrow) at 40.8 mm (size index 2.02 cm/m2) mm and the mid-ascending aorta (black arrow) at 53.6 mm (size index 2.65 cm/m2), which were measured at mid-systole. (C) Color Doppler echocardiogram shows a jet regurgitating into the left ventricle. Aortic regurgitation was considered mild.

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  Figure 5. Bicuspid aortic valve (BAV) without a raphe and combined aortic root, as well as mid-ascending aorta in a 64-year-old male. (A) Double-oblique CT reconstruction parallel to the aortic valve demonstrates thickened, calcified BAV cusps without a raphe. (B) Diameters of the sinuses of Valsalva (white arrow) at 43.5 mm (size index 2.24 cm/m2) and the mid-ascending aorta (black arrow) at 46.4 mm (size index 2.48 cm/m2), which were measured at mid-systole. (C) Continuous-wave Doppler recording of the aortic stenosis jet from an apical approach shows a maximum velocity of 3.07 m/sec. The continuity equation of the aortic valve area was 1.2 cm2, corresponding to moderate aortic stenosis.