80
Treatment
Table 34. Prophylactic Aortic Surgery Before Pregnancy in
Women With Aortopathic Conditions
Condition
Surgical Threshold Before
Pregnancy* by Aortic
Diameter (cm) or Aortic
Size Index (cm/m
2
)
Marfan syndrome >4.5 cm
Marfan syndrome with risk factors (rapid aortic growth
of ≥0.3 cm/y; family history of aortic dissection)
4.0–4.5 cm
Loeys-Dietz syndrome (attributable to pathogenic
variants in TGFBR1, TGFBR2, or SMAD3)
≥4.0 cm
Loeys-Dietz syndrome (attributable to pathogenic
variants in TGFB2 or TGFB3)
≥4.5 cm
Non-syndromic heritable thoracic aortic disease
≥4.5 cm
†
Turner syndrome ≥2.5 cm/m
2
Bicuspid aortic valve
≥5.0 cm
‡
* Shared decision-making is required to determine surgical threshold before elective aortic root,
ascending aortic surgery, or both and is informed by the condition, specific pathogenic variant,
age, body size, aortic growth rate, phenotype, and family history of aortic dissection, and
surgery at smaller aortic diameters may be considered depending on individual circumstances.
†
Aortic dissection related to pregnancy has occurred at small aortic diameters in women with
ACTA2 and MYLK pathogenic variants. Prophylactic aortic surgery before pregnancy at
smaller aortic diameters may be reasonable in these conditions and other non-syndromic
heritable thoracic aortic disease and may be informed by the molecular diagnosis, family
history, and aortic growth rate.
‡
Prophylactic aortic surgery may be considered at smaller aortic diameters depending on body
size, aortic growth rate, and family history.
8.3. Surgery Before Pregnancy in Women With Aortic Disease
(cont'd)
COR LOE
Recommendations
1 C-EO 5. In patients with a BAV (in the absence of Turner syndrome or
an HTAD) and an aortic diameter of ≥5.0 cm, surgery before
pregnancy is recommended.
1 C-EO 6. In patients with sporadic aortic root aneurysms, ascending
aortic aneurysms, or both and a diameter of ≥5.0 cm, surgery
before pregnancy is recommended.
