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. 2021 May 7;11:9751. doi: 10.1038/s41598-021-89164-w

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© The Author(s) 2021

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Pediatric CAVS demonstrate high deposition of immature collagen compared to age-matched controls. (A,B) Herovici staining of SKU DB27, a 17-year-old normal sample with regions of Hinge, Belly, and Free Margin 10 × zoom shown (B). Blue = immature. Purple-mature. Normal immature collagen production is localized to the ventricularis, while pCAVS immature production becomes increasing dysregulated towards the free margin. (C,D) Herovici staining of SKU DB16, an age-matched pCAVS sample with regions of Hinge, Belly, and Free Margin 10 × zoom shown in (D). (E) Patient specific breakdown of Herovici color deconvolution analysis. pCAVS patients show increased immature collagen production compared to age matched controls. (F) Boxplot showing quantification of Herovici color deconvolution, showing larger heterogeneity amongst pCAVS patients. Scale Bar: 300 μm. Normal n = 4; pCAVS n = 11; AVI n = 5.