Furthermore, Familial Thoracic Aortic aneurysm and dissection (FTAAD) and its variant with bicuspid aortic valve lack of marfanoid skeletal features, involve different genes (ACTA2 (MIM * 102,620), MYLK (MIM * 600,922), PRKG1 (MIM * 176,894), MYH11 (MIM * 160,745), MFAP5 (MIM * 601,103), MAT2A (MIM * 601,468) but show aortic enlargement and early onset of rupture by dissection or aneurysm formation [31], which is also more challenging for pathologists. Here, ACTA2 is linked to familial thoracic aortic aneurysm and aortic dissection.