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A bicuspid aortic valve (BAV) is the most prevalent congenital cardiac deformity, which is associated with an increased risk to develop a thoracic aortic aneurysm and/or an aortic dissection as compared to persons with a tricuspid aortic valve. Due to the high prevalence of a BAV in the general population and the associated life-long increased risk for adverse vascular events, BAV disease places a considerable burden on the public health. The aim of the present review is to discuss the role of transforming growth factor beta (TGF-β) signaling in the development of the vascular wall and on how this complex signaling pathway may be involved in thoracic aortic aneurysm formation in tricuspid and BAV patients., Competing Interests: Conflict of interestThe authors declare no conflict of interest., (© The Author(s) 2023.)

Background: An aortic dissection is the most devastating complication of thoracic aortic disease. Several non- and syndromic conditions such as a bicuspid aortic valve (BAV) and Marfan syndrome (MFS) have a severely increased risk to develop a thoracic aortic aneurysm and dissection. To date, the medial layer has been extensively studied in search of the pathogenetic mechanisms leading to aortic complications., Objective: We aim to determine whether intimal layer pathology is characteristic in all thoracic aortopathy regardless of the underlying etiology., Method: A total of 176 aortic wall specimen were studied for the intimal layer architecture including the intimal thickness, endothelial cell morphology, and atherosclerosis. Specimens were derived from four patient groups: BAV ( n  = 70, age 57 ± 8.9 years), isolated tricuspid aortic valve (TAV) ( n  = 38, age 64.9 ± 11.0 years), MFS with a TAV ( n  = 8, age 34.2 ± 11.0 years), type A dissections with a TAV ( n  = 60, age 62.7 ± 10 years)., Results: The intimal layer is significantly thinner in BAV, MFS, and type A aortic dissection as compared to the isolated TAV patients ( p  < 0.001). Intimal atherosclerosis was also significantly less present in the three groups as compared to the isolated TAV ( p  < 0.05)., Discussion: A thin intimal layer is a common finding in the thoracic aortopathy patients. Studies aiming at preventing future aortic complications should focus on the intimal pathology as a common effector pathway in thoracic aortopathy., Competing Interests: Conflict of interestNone., (© The Author(s) 2023.)