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............................................................................................................................................................ 5.1 Maternal and offspring risk Haemodynamic and hormonal changes during pregnancy increase the susceptibility to dissection.165 Dissection occurs most often in the last trimester of pregnancy (50%) or the early post-partum period (33%). All women with a genetically proven syndrome or familial aortic pathology should have counselling on the risk of dissection and the recurrence risk, and have a complete evaluation including imaging of the entire aorta before pregnancy (see section 3). When assessing aortic diameters, body surface area should be considered, especially in women of small stature. Parity seems associated with increased aortic diameter.166 The effect of pregnancy on aortic dilatation is not clear.167,168 The diagnosis of aortic dissection should be considered in all patients with chest pain during pregnancy.

5.2 Specific syndromes

Marfan syndrome is thought to affect 1 in 5000 individuals. Although bicuspid aortic valve is more common (1–2% of the population), associated aortic complications are uncommon, accounting for only 6% of type A dissections during pregnancy.169

5.2.1 Marfan syndrome The overall risk of a woman with Marfan syndrome having an aortic dissection associated with pregnancy is 3%.170 Aortic size is a major determinant of risk, but even women with an aortic root <40 mm have a risk of dissection of 1%.170,171 Although there are limited data, pregnancy should be avoided in Marfan patients with an aortic root diameter >45 mm as there is an increased risk of dissection. When the aorta is 40–45 mm, other factors should be considered, such as family history of dissection and rate of aortic growth.163 Distal aortic dissection and dissection of other vessels are also a risk. For this reason, even after successful aortic root replacement, patients remain at risk of further events.172 Studies focusing on the potential growth during pregnancy in Marfan patients demonstrated contradicting results; some demonstrated no significant growth, while others demonstrated growth >_3 mm with a partial diameter decrease postpartum.167,168,173 Other important cardiac complications include progressive mitral regurgitation (MR) due to mitral valve prolapse, new arrhythmia, and HF due to ventricular dysfunction.174,175 Obstetric complications are also increased, including premature rupture of membranes.19

5.2.2 Bicuspid aortic valve Aortic dilatation occurs in <_50% of patients with a bicuspid aortic valve and can occur even when valve function is normal. The dilatation can be in the distal ascending aorta, which cannot be adequately visualized by echocardiography. If not visible with echocardiography, MRI or CT should be performed pre-pregnancy. The risk of dissection is small. Risk factors are the type of bicuspid aortic valve morphology, aortic dilatation, and CoA.176 Pregnancy should be avoided when the aorta diameter is >50 mm.

5.2.3 Vascular Ehlers–Danlos syndrome Serious vascular complications occur almost exclusively in type IV Ehlers–Danlos syndrome (vascular). Maternal mortality is significant, and relates to uterine rupture and dissection of major arteries and veins. Pregnancy is therefore considered as a very high-risk undertaking and not advised.177 These women should be engaged in a shared decision-making process when contemplating pregnancy.

5.2.4 Turner syndrome Turner syndrome is associated with an increased risk of congenital heart disease, aortic dilatation, hypertension, diabetes, and atherosclerotic events.178 Aortic dissection occurs rarely in Turner syndrome, but it is six times more common at younger ages than in the general population179 Risk factors for aortic dissection include aortic dilation, bicuspid aortic valve, and CoA.20,180 Pregnancy should be avoided when the aortic size index is >25 mm/m2. Also, after aortic root surgery, the patient remains at risk of type B dissection. Spontaneous pregnancy can occur in mosaic Turner patients (0.5–10%), but pregnancy is now most commonly secondary to assisted fertility techniques. Cardiovascular evaluation is recommended before starting fertility treatment. Good BP control and diabetes management is mandatory for all Turner patients, especially during pregnancy.178

5.2.5 Other autosomal dominant aortopathies With improved genotyping, a series of new aortopathies are being reported. These includes syndromic and non-syndromic HTAD. These conditions are considered high-risk, especially when the aorta is dilated, and may also have multisystem involvement with additional risks such as uterine rupture.181–184

5.3 Management

5.3.1 Follow-up and medical therapy Depending on the aortic diameter, patients with aortic pathology should be monitored by echocardiography at regular intervals throughout the pregnancy and 6 months post-partum. In women with a highrisk of dissection or an already severely dilated aorta, monitoring every month is warranted, while in low-risk women with only a mildly dilated aorta, monitoring every 12 weeks seems reasonable. When needed, cardiac MRI without contrast can be used. Pregnancy should be supervised by a cardiologist and obstetrician who are alert to the possible complications. Strict BP control is advised, and antihypertensive treatment that is safe for the foetus should be initiated if necessary.185 In women with HTAD, beta-blocker therapy throughout pregnancy should be considered. In patients with Ehlers–Danlos syndrome type IV, celiprolol is recommended (also in normotensive women) because of the very high-risk of dissections and the benefit demonstrated in Downloaded from academic.oup.com/eurheartj/article/39/34/3165/5078465 by guest on 07 October 2026