Altogether, this study added 3 novel mutations to the existing spectrum of FBN1 mutations and demonstrated different frameshift genotype impacts on cardiovascular-phenotype severity in patients with MFS, which might facilitate understanding on potential phenotype–genotype associations and refine susceptible populations of deleterious cardiovascular events such as greater risk for early aortic dissection or markedly poor heart function even after prophylactic surgery. Here, FBN1 is linked to Marfan syndrome.