Loeys-Dietz syndrome subtypes exhibit distinct clinical behavior and aortic cellular transcriptomic profiles.

Five deaths occurred in TGFBR1/2 patients (estimated survival 97% [99-82], 86.% [96-61], and 76% (91-45] at 5, 10, and 15 years, respectively) compared with 1 perioperative and no late deaths in SMAD3 patients (estimated survival 94% at both 5- and 10-year follow-up [99-63]), although this comparison did not reach statistical significance ( P = . 96) ( Figure 2 , B ).

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