Within the Loeys-Dietz syndrome group, mean freedom from death was similar with TGFBR1 (70 ± 3 years, 95% CI 64–77), TGFBR2 (73 ± 5 years, 95% CI 63–83), and SMAD3 pathogenic variants (76 ± 6 years, 95% CI 64–89; p = 1.000; Figure 1). Here, TGFBR1 is linked to Loeys-Dietz syndrome.