Stewart Fiona M
Greenlane Cardiovascular Service and National Women's Health, Auckland City Hospital, Auckland, New Zealand.
Obstet Med. 2013 Sep;6(3):112-119. doi: 10.1177/1753495X13496237. Epub 2013 Aug 9.
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.
主动脉病变，即影响主动脉的疾病，在孕期会使母亲和胎儿面临显著的死亡风险，因为主动脉夹层的发生率会增加。遗传性主动脉病变，如马凡综合征、二叶式主动脉瓣、洛伊迪茨综合征、埃勒斯-当洛综合征（IV型）、特纳综合征以及非综合征性家族性胸主动脉瘤和夹层，均与主动脉夹层风险增加相关，尤其是在孕晚期和产后早期。孕期母亲的预后取决于潜在疾病以及孕前的主动脉尺寸。胎儿有高达50%的几率遗传潜在的基因缺陷。血管炎，尤其是高安动脉炎在孕期也可能是个问题，并易引发主动脉夹层。孕前评估，包括仔细评估主动脉以及对主动脉瘤进行预防性主动脉手术，对于某些主动脉病变而言，可能会降低孕期主动脉夹层的风险，但对于接受过手术的马凡综合征、洛伊迪茨综合征和埃勒斯-当洛综合征（血管IV型）女性，死亡风险仍然很高。一部分孕前主动脉尺寸正常的马凡综合征或二叶式主动脉瓣女性在孕期预后良好。多学科孕期护理，就孕期随访、分娩和产后护理达成一致，并制定主动脉夹层危机预案，可改善妊娠结局，并确保在发生主动脉夹层时能迅速进行处理。
