We identified 51 families with a FTAAD caused by genes other than FBN1. The diagnosis was confirmed with the identification of a disease-causing variant in one of the following genes: TGFBR2 (N = 21), TGFBR1 (n = 8), SMAD3 (n = 7), TGFB3 (n = 4), ACTA2 (n = 2), FBN2 (n = 2), LOX (n = 2), MYH11 (n = 1), MYLK (n = 1), SKI (n = 1), and COL5A1 (n = 1). The gene discussed is MYLK; the disease is familial thoracic aortic aneurysm and aortic dissection.