TABLE 6.

Cause of TAA

HTAD (see Table 7): syndromic

■ Marfan syndrome

■ Loeys-Dietz syndrome

■ Vascular Ehlers-Danlos syndrome

■ Smooth muscle dysfunction syndrome

■ Others: attributable to pathogenic variants in FLNA, BGN, LOX

HTAD (see Table 7): nonsyndromic

■ ACTA2, MYH11, PRKG1, MYLK, and others

■ Familial thoracic aortic aneurysm without identified pathogenic variants in a known gene for HTAD

Congenital conditions

■ Bicuspid aortic valve

■ Turner syndrome

■ Coarctation of the aorta

■ Complex congenital heart defects (tetralogy of Fallot, transposition of the great vessels, truncus arteriosus)

Hypertension

Atherosclerosis

Degenerative

Previous aortic dissection

Inflammatory aortitis

■ Giant cell arteritis

■ Takayasu arteritis

■ Behçet disease

■ Immunoglobulin G4-related disease, antineutrophil cytoplasmic antibody-related, sarcoidosis

Infectious aortitis

■ Bacterial, fungal, syphilitic

Previous traumatic aortic injury

HTAD indicates heritable thoracic aortic diseases; and TAA, thoracic aortic aneurysms.