Severe degeneration of a sub-coronary pulmonary autograft in a young adult

Figures

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  Figure 1.. Trans-thoracic echo, short axis view at level of aorta showing calcified leaflets with restricted closure and mal-coaptation (A). Trans-thoracic echo, parasternal long axis view showing color Doppler of the aortic valve with severe aortic regurgitation (B). Transesophageal echo, 120-degree view showing the aortic valve with restricted calcific leaflets in 2D (C) and color Doppler showing severe aortic regurgitation (D). Trans-thoracic echo, apical four chamber view showing dilated hypertrophied RV due to pressure overload (E).

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  Figure 2.. MSCT derived 3D models of the aortic root showing severe distortion of the aortic root with aneurysmal dilatation above the right coronary sinus (A) as well as significant stenosis of the origin of the right coronary (A). Severe calcification involving parts of the leaflets and the inside of the aneurysm (B). 3D models of the RV and outflow showing severe calcification and stenosis of the homograft conduit inserted 23 years previously (C).

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  Figure 3.. Macroscopic view of all the leaflets.

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  Figure 4.. Continuous wave Doppler over neo-pulmonary valve showing no significant gradients across valve 1-month post-surgery (A). Continuous wave Doppler over neo-aortic valve showing no significant gradients across valve 1-month post-surgery (B). 2D echocardiogram of the neo-aortic valve (Freestyle prosthesis) with full leaflet motion (C). RV outflow view showing the neo-pulmonary valve with full leaflet motion in 2D (D) and color Doppler showing laminar flow across the valve (E).

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  Figure 5.. Sections from leaflet A stained with elastin van Gieson (A), haemotoxylin and eosin (B), Alcian blue/Sirius red (C) and alizarin red (D). Colour-coded magnified views of panels in A (E–G) and in panel D (H). A normal pulmonary valve stained with elastin van Gieson (I) and Alcian blue/Sirius red (J). The right side of each image is the co-apting edge and the left side is the hinge side. The top is the fibrosal side and the bottom the ventricularis. Scale bar represents 2.5 mm in top 4 panels, bottom 2 panels and 100 μm in other panels.

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  Figure 6.. Sections from leaflet B stained with elastin van Gieson (A), haemotoxylin and eosin (B), Alcian blue/Sirius red (C), alizarin red (D), colour coded magnified views of panels in C (E) and in panel B (F). Positive endothelial staining for α-SMA (Figure 6G) and Runx2 (Figure 6H). The right side of each image is the co-apting edge and the left side is the hinge side. The top is the fibrosal side and the bottom the ventricularis. Scale bar represents 2.5 mm in top 4 panels, 250 μm in the next row and 100 μm in the bottom 2 panels.

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  Figure 7.. Sections from leaflet C stained with elastin van Gieson (A), Alcian blue/Sirius red (C), haemotoxylin and eosin (B) and alizarin red (D), colour coded magnified views of panels in B (E,F). The right side of each image is the co-apting edge and the left side is the hinge side. The top is the fibrosal side and the bottom the ventricularis. Scale bar represents 2.5 mm in top 4 panels, 50 μm in panel E and F.

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  Figure 8.. Panels show immunostaining of specific markers. Scale bar represents 100 μm.

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  Figure 9.. CT-generated 3D models of the freestanding aortic root showing variable degrees of dilatation.

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  Figure 10.. The use of autologous tissue (autologous aortic wall and pericardium) to support the freestanding root (the loose jacket technique) as used in our centre.