Within the Loeys-Dietz syndrome group, mean freedom from proximal aortic surgery was similar with TGFBR1 (48 ± 5 years, 95% CI 38–58), TGFBR2 (49 ± 5 years, 95% CI 39–58), and SMAD3 pathogenic variants (68 ± 6 years, 95% CI 55–80; p = 0.143; Figure 2). Here, SMAD3 is linked to Loeys-Dietz syndrome.