Kim So Yeon, Wolfe Diana S, Taub Cynthia C
Tufts Medical Center, Boston, Massachusetts, USA.
Montefiore Medical Center, Bronx, New York, USA.
Congenit Heart Dis. 2018 Mar;13(2):203-209. doi: 10.1111/chd.12546. Epub 2017 Oct 23.
Pregnancy in patients with Marfan's syndrome (MFS) carries an increased risk of cardiovascular complications, resulting in increased maternal and fetal mortality and morbidity. Literature on MFS pregnant patients is relatively sparse, and there has yet to be a concrete consensus on the management of this unique patient population. The purpose of our paper is to provide a literature review of case reports and studies on MFS during pregnancy (published between 2005 and 2015) and to explore cardiovascular outcomes of patients with MFS.
Of the 852 women in our review, there were 1112 pregnancies, with an aortic dissection rate of 7.9% and mortality of 1.2%. Data demonstrated a trend that patients whose aortic diameter ≥40 mm had a greater rate of dissection than MFS patients whose aortic diameter <40 mm (Fisher's exact test, P = .0504). Fetal outcome included a 5.6% mortality rate and 41% of births were cesarean deliveries and of those reported, 75% secondary to cardiac emergencies.
Patients with MFS, especially those whose initial aortic diameters ≥40 mm, planning a pregnancy or currently pregnant should be carefully counseled about the maternal and fetal risks throughout pregnancy. MFS patients whose aortic diameters ≥40 mm should be advised to ideally await pregnancy until prophylactic aortic surgery. As MFS varies in its phenotypic expression, each patient's risk of adverse cardiac events should be assessed individually through a joint Maternal Fetal Medicine and Cardiology Center.
马凡氏综合征(MFS)患者怀孕会增加心血管并发症的风险,导致孕产妇和胎儿死亡率及发病率上升。关于MFS孕妇的文献相对较少,对于这一特殊患者群体的管理尚未达成具体共识。我们本文的目的是对2005年至2015年间发表的关于MFS孕期病例报告和研究进行文献综述,并探讨MFS患者的心血管结局。
在我们综述的852名女性中,共有1112次妊娠,主动脉夹层发生率为7.9%,死亡率为1.2%。数据显示一种趋势,即主动脉直径≥40mm的患者比主动脉直径<40mm的MFS患者夹层发生率更高(Fisher精确检验,P = 0.0504)。胎儿结局包括5.6%的死亡率,41%的分娩为剖宫产,其中报告的剖宫产中有75%继发于心脏急症。
MFS患者,尤其是初始主动脉直径≥40mm的患者,计划怀孕或正在怀孕时,应在整个孕期就孕产妇和胎儿风险接受仔细咨询。主动脉直径≥40mm的MFS患者应被建议理想情况下等待至进行预防性主动脉手术后再怀孕。由于MFS的表型表达各不相同,应通过母婴医学和心脏病学联合中心对每位患者发生不良心脏事件的风险进行个体评估。