Aortopathy slider

In women with MFS and aortic root diameters between 40 and 45 mm, surgery before pregnancy may be considered if risk factors (growth >3 mm/year, family history of aortic dissection) are present.Guideline rec

Class IIbLevel CRec Table 8 · §8 · p.4504 · S10

Applies only if: Aortic growth >3 mm/year · or · Family history of aortic dissection

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In women with an aorta 40–45 mm, vaginal delivery with epidural anaesthesia and an expedited second stage should be considered.Guideline rec

Class IIaLevel CRec Table 8 · §8 · p.4504 · D2

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In women with an aorta 40–45 mm, caesarean section may be considered.Guideline rec

Class IIbLevel CRec Table 8 · §8 · p.4504 · D5

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Source discrepancy: Rec Table 8 prints vaginal delivery with epidural and an expedited second stage (IIa) and caesarean section (IIb) for 40–45 mm, and caesarean section (IIa) for ≥45 mm. At exactly 45 mm all three printed rows apply; the guideline does not reconcile them. Figure 8 draws the same overlap.

Counselling

It is recommended that women with aortic disease have counselling about the risk of aortic dissection in pregnancy and the post-partum period.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · O1

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Imaging

Imaging of the entire aorta (CT or CMR) is recommended before pregnancy in women with known or suspected aortic disease.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · I1

^d In women with vascular Ehlers–Danlos syndrome and LDS, imaging should encompass the entire aorta, including supra-aortic vessels as well as iliac and femoral arteries.

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In women with low-risk aortic disease (mWHO 2.0 classes II and II–III), one-time echocardiographic imaging between 20 and 30 weeks of gestation and imaging at 6 months post-partum is recommended.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · I3

Applies by mWHO 2.0 class (II and II–III); the class is not set here.

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In women with moderate to high-risk aortic disease (mWHO 2.0 classes III and IV), repeated echocardiographic imaging every 4–12 weeks (depending on diagnosis and severity of dilatation) is recommended during pregnancy and until 6 months post-partum.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · I4

Applies by mWHO 2.0 class (III and IV); the class is not set here.

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CMR (without gadolinium) imaging of the entire aorta is recommended in pregnant women at risk of or with known aortic dilatation who have not had recent pre-pregnancy cross sectional imaging.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · I5

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Medical treatment

When a woman with known aortic dilatation, history of dissection, or P/LP variant associated with aortic disease becomes pregnant, strict and individualized BP control is recommended.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · O4

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Beta-blocker therapy throughout pregnancy and in the post-partum period is recommended in women with MFS and other HTADs.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · O5

Printed for 'MFS and other HTADs'.

^e See Section 5.2.9 for beta-blocker choice.

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Surgery

It is recommended that indications for pre-pregnancy aortic root and/or ascending aortic surgery are guided by aortic morphology, underlying pathology, family history, genetic variant, previous vascular events, and patient’s preference.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · O7

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It is recommended that centres managing pregnancies in women with moderate to high-risk aortic disease (mWHO 2.0 class III/IV) can provide cardiovascular surgery in case of peripartum adverse events.Guideline rec

Class ILevel CRec Table 8 · §8 · p.4503 · O8

Applies by mWHO 2.0 class (III/IV).

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Delivery

The use of ergometrine post-delivery is not recommended in women with aortopathy.Guideline rec

Class IIILevel CRec Table 8 · §8 · p.4504 · O9

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Cardiac surgery during pregnancy

Delivery before cardiac surgery should be considered as soon as the foetus is viable, taking gestational age, comorbidities, and the available level of neonatal care into account.Guideline rec

Class IIaLevel CRec Table 8 · §8 · p.4504 · O10

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Cardiac surgery may be considered during pregnancy when conservative and medical therapy has failed, and in situations that threaten the mother’s life or that are not amenable to percutaneous treatment.Guideline rec

Class IIbLevel CRec Table 8 · §8 · p.4504 · O11

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Third stage of labour (uterotonics) [SUPP]

First-line	Oxytocin
Second-line	Misoprostol, Carboprost
Printed conditions	Marfan syndrome and Loeys–Dietz with aortic dilatation >40 mm Bicuspid aortopathy and aortic dilatation >45 mm Vascular Ehlers Danlos syndrome Previous aortic dissection TS and ASI >25 cm/m²

Supplement Table S3 (PDF p11), row 'Significant aortopathy'. Management of the third stage of labour for women in mWHO 2.0 classes III and IV. No class or level is printed.

Delivery, 40–45 mm and ≥45 mm: Rec Table 8 prints vaginal delivery with epidural and an expedited second stage (IIa) and caesarean section (IIb) for 40–45 mm, and caesarean section (IIa) for ≥45 mm. At exactly 45 mm all three printed rows apply; the guideline does not reconcile them. Figure 8 draws the same overlap.
Aortic disease without a variant, ≥45 mm (Class IIa): This row is in Rec Table 8 (p4504) but is not drawn in Figure 8. Rec Table 8 is authoritative.
Marfan 40–45 mm: Rec Table 8 prints 'growth >3 mm/year'; Figure 8 footnote a prints 'rapid aortic growth (≥3 mm/year)'. The table wording is used. Figure 8 footnote a also lists uncontrolled hypertension as a risk factor, which Rec Table 8 does not for Marfan.
BAV, ≥45 mm (Class IIa): Rec Table 8 prints 'root phenotype or family history of aortic aneurysm or dissection'. Figure 8 footnote b adds root phenotype to the risk factors in footnote a (family history of dissection, rapid growth, uncontrolled hypertension). The table wording is used.
Non-syndromic HTAD, 40–44 mm (Class IIb): Rec Table 8 prints 'aortic root diameters ≥40–44 mm' with 'depending on the genetic variant, family history, and aortic growth rate'; Figure 8 draws it as 'nsHTAD with risk factors'. Values between 44 and 45 mm fall in neither printed range.
Loeys–Dietz, TGFB2/TGFB3/SMAD2/SMAD3: Class IIa at ≥45 mm in both Rec Table 8 and Figure 8. The figure writes the genes as 'TGFB2/B3, SMAD2/3'.
Vascular Ehlers–Danlos syndrome: Rec Table 8 prints caesarean section at 37 weeks (Class I) for vascular Ehlers–Danlos syndrome, and prints the diameter-based delivery rows for 'women with an aorta' without excluding it. Both are shown; the guideline does not reconcile them. No surgical diameter threshold is printed for vascular Ehlers–Danlos syndrome.
Aortic dissection: Rec Table 8 prints no surgical threshold for aortic dissection. It prints pre-pregnancy counselling by an extended Pregnancy Heart Team (I), surgical indications guided by morphology, aetiology and genetics (I), and caesarean section 'should be considered' (IIa). Only those printed rows are shown.

Inputs

Output

Counselling

Imaging

Medical treatment

Surgery

Delivery

Cardiac surgery during pregnancy

Third stage of labour (uterotonics) [SUPP]