30
Treatment
6.1.2.1.1. Surgical Considerations for Non-syndromic
Heritable TAAs and No Identified Genetic Cause
COR LOE
Recommendations
1 C-LD 1. In asymptomatic patients with aneurysms of the aortic root
or ascending aorta with nonsyndromic heritable thoracic
aortic disease (nsHTAD) and no identified genetic cause,
determining the timing of surgical repair requires shared
decision-making and is informed by known aortic diameters at
the time of aortic dissection, TAA repair, or both in affected
family members.
1 C-LD 2. In asymptomatic patients with aneurysms of the aortic root
or ascending aorta with nsHTAD and no identified genetic
cause but no information on aortic diameters at the time of
dissection or aneurysm repair in affected family members and
who have no high-risk features for adverse aortic events (Table
9) it is recommended to repair the aorta when the maximal
diameter reaches ≥5.0 cm.
2a C-LD 3. In patients with aneurysms of the aortic root or ascending
aorta with nsHTAD and no identified genetic cause and
a maximal aortic diameter of ≥4.5 cm, who have high-risk
features for adverse aortic events (Table 9), or who are
undergoing cardiac surgery for other indications, aortic repair
is reasonable when performed by experienced surgeons in a
Multidisciplinary Aortic Team.
Table 9. Features Associated With an Increased Risk of Aortic
Dissection in Patients With Heritable Thoracic Aortic
Aneurysms
Heritable Thoracic Aortic Aneurysms and No Identified Genetic Cause
Family history of aortic dissection at an aortic diameter <5.0 cm
Family history of unexplained sudden death at age <50 y
Rapid aortic growth (≥0.5 cm in 1 y or ≥0.3 cm/y in 2 consecutive y)
