Activation of ERK1/2 and Smad2 and enhanced vasa vasorum formation were observed in proband's aortic wall; however, these changes might be influenced by aortic dissection, while the increased vascularity in the aortic media has been reported from patients who had nondissecting thoracic aneurysm and MYLK pathogenic variants [10, 19]. The gene discussed is MAPK3; the disease is Aortic dissection.