Indeed, these questions were considered in a study conducted by Takeda et al. involving 248 patients with FBN1 mutations, where HI variants were again proved to have a higher risk for severe aortic events (type A aortic dissection, aortic root replacement and aorta-related death) than DN mutations, although they identified a subgroup within the DN variants with similar deleterious effect as HI ones. Here, FBN1 is linked to Aortic dissection.