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/m2)
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
Nonsyndromic heritable thoracic aortic disease	≥4.5 cm†
Turner syndrome	>2.5 cm/m2
Bicuspid aortic valve	≥5.0 cm‡

^*Shared decision-making is required to determine the 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 nonsyndromic 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.

Colors correspond to Class of Recommendations in Table 2.