The Importance of Aortic Valve Bicuspid Phenotype in Valvular Evolution in Pediatric Patients: A Case Report and Literature Mini-Review

Figures

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  Figure 1. In image (A), we can see the aortic valve thickening, with reduced opening during systole (arrow). In image (B), evident presence of calcifications within the aortic valve leaflets, contributing to the narrowing of the valve orifice, as revealed by the bright, echo-dense areas with a prominent acoustic shadow (arrow).

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  Figure 2. Angiographic computed tomography (CT) image illustrating severe aortic valve stenosis associated with aortic bicuspidy with clear visualization of the aortic cusps and focal valve calcifications at the aortic commissure (red arrow). These calcifications are evident as bright, dense areas within the valve leaflets (A). In a sagittal plane, the ascending aorta appears significantly dilated, measuring 4.7 cm in diameter, surpassing the normal range (the colored lines were used as a guide for adjusting imaging planes to the standard cardiac planes- the pink line for the horizontal long axis and the yellow one for the short axis) (B). On the three-dimensional (3D) reconstructed angiographic computed tomography (CT) scan, we can see the dilatation of the ascending aorta. Adjacent to the dilated segment, a sacciforme aneurysm is evident (red arrow). Furthermore, the image showcases aortic ulcerations in the right anterior part of the ascending aorta (blue arrow) (C,D).

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  Figure 3. Aortic valve leaflets showing degenerative changes, with architectural distortion (A), primarily due to fibrous layer expansion (B). Also, multiple calcified foci were present (C,D). HE: Hematoxylin–Eosin staining.

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  Figure 4. Mucoid accumulation, as emphasized by alcian-blue staining (A,C) and fibrous layer expansion, as evidenced by Masson-trichrome staining (B,D).

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  Figure 5. Aortic wall sections showing moderate medial degeneration (A,B). The mucoid extracellular matrix accumulation is seen, predominantly with intralamellar extension (MEMA-i) (C,D). Mild elastic fiber fragmentation and collapse, as revealed by van Gieson staining for elastin evidentiation (E). Mild medial fibrosis, as revealed by Masson-trichrome staining (F). HE: Hematoxylin–Eosin staining.