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Marfan's syndrome and other aortopathies in pregnancy - PubMed

Marfan's syndrome and other aortopathies in pregnancy

Fiona M Stewart. Obstet Med. 2013 Sep.

Abstract

Aortopathies, or disease affecting the aorta, are associated with a significant mortality risk for the mother and foetus during pregnancy because of an increased rate of aortic dissection. The hereditary aortopathies; Marfan's syndrome, bicuspid aortic valve, Loeys-Dietz syndrome, Ehlers-Danlos (type IV) syndrome, Turner's syndrome and nonsyndromic familial thoracic aortic aneurysm and dissection are all associated with an increased risk of aortic dissection particularly during the third trimester and early postpartum period. Maternal outcome in pregnancy depends on the underlying disorder and the aortic dimensions prior to pregnancy. The foetus has up to 50% chance of inheriting the underlying genetic defect. Vasculitis, particularly Takayasu's arteritis may also be a problem in pregnancy and predispose to aortic dissection. Prepregnancy review, including careful assessment of the aorta and prophylactic aortic surgery for an aortic aneurysm may reduce the risk of aortic dissection in pregnancy for some of the aortopathies but for women with Marfan's syndrome, Loeys-Dietz syndrome and Ehlers-Danlos (vascular type IV) who have had surgery, the risk of death remains high. A subgroup of women with Marfan's syndrome or a bicuspid aortic valve and normal aortic dimensions prepregnancy should do well in a pregnancy. Multidisciplinary pregnancy care with agreement on pregnancy follow-up, delivery and postpartum care with a crisis plan for an aortic dissection can improve pregnancy outcome and ensure prompt management of an aortic dissection should it occur.

Keywords: Cardiovascular; aorta; high-risk pregnancy.

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Figures

Figure 1.
Figure 1.

Transthoracic echocardiogram showing a Type A aortic dissection (a, b) and aortic regurgitation (c).

Figure 2.
Figure 2.

Magnetic resonance image showing dilated aortic root (a) and dural ectasia (b) in a patient with Marfan syndrome.

Figure 3.
Figure 3.

Magnetic resonance image of the thoracic (a) and abdominal (b) aorta in a woman who presented with an aortic arch dissection (red arrows) at 23 weeks in her first pregnancy that required urgent repair. At 32 weeks a further dissection occurred complicated by left renal and splenic infarction (b) with collateral formation (yellow arrow). A Bentall’s procedure was performed at a later date for ascending aortic aneurysm.

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