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. 2022 Jul;11(4):380–388. doi: 10.21037/acs-2021-bav-14

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2022 Annals of Cardiothoracic Surgery. All rights reserved.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0.

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Developmental origin of the BAV and aortic wall. Progenitor neural crest and second heart field cells contribute to the normal development of an aortic valve and the vascular smooth muscle cells in the ascending aortic wall. Mutations in genes related to the neural crest and second heart lead to the development of a BAV and a maturation defect of vascular smooth muscle cells in the aortic root, ascending aorta and arch. Type 1 RCC-NCC and type 1 RCC-LCC have been attributed to defects in the neural crest and second heart field cell signalling respectively. RCC-NCC: fusion between the RCC and NCC. LCC-NCC: fusion between the LCC and NCC. RCC-LCC: fusion between the RCC and LCC. TAD: reduced glutathione. BAV, bicuspid aortic valve; RCC, right coronary cusp; NCC, non-coronary cusp; LCC, left coronary cusp; VSMC, vascular smooth muscle cell; SHF, second heart field.