Background

The risk of pregnancy-associated vascular complications in Marfan syndrome (MFS) is uncertain because of ascertainment bias, prepartum lack of knowledge of diagnosis, and insufficient peripartum imaging data. Furthermore, U.S. and European guidelines differ in pregnancy recommendations in MFS.

Objectives

This study describes a single-center experience of 169 MFS women to address these gaps.

Methods

Clinical, imaging, and pregnancy history were compared in never vs ever-pregnant MFS women, and pregnancy-associated vascular complications were described.

Results

A total of 74 ever-pregnant women had 112 live births. Elective aortic root replacement occurred at a younger age in never-pregnant women (33 years vs 42 years; P = 0.0026). Although aortic dissection prevalence did not differ between never-pregnant vs ever-pregnant women (23% vs 31%; P = 0.25), it tended to occur at an earlier age in the former group (38 years vs 45 years; P = 0.07). Of observed “sanctioned” pregnancies with prepartum diameters ≤4.5 cm, mean pregnancy-related aortic diameters remained stable. In total, 5 dissections were associated with pregnancy: 2 type A in women unaware of their diagnosis; and 2 type B and 1 isolated coronary artery dissection in women aware of their diagnosis. Dissection rates were 5-fold higher in the pregnancy vs nonpregnancy period.

Conclusions

Pregnancy-related type A dissection only occurred in patients unaware of their diagnosis. Type B dissection remains an unpredictable complication. Although there were baseline differences between the never- and ever-pregnant groups, no difference in dissection risk was observed outside the peripartum period. Those with prepartum aortic diameters between 4.0 and 4.5 cm demonstrated stable aortic dimensions throughout pregnancy. These findings provide a rationale to update existing U.S. guidelines for the management of pregnancy in MFS.

中文翻译：

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马凡综合征女性妊娠相关的主动脉并发症

背景

由于确定偏倚、产前缺乏诊断知识以及围产期影像学数据不足，马凡综合征 (MFS) 中妊娠相关血管并发症的风险尚不确定。此外，美国和欧洲的指南对 MFS 的妊娠建议有所不同。

目标

本研究描述了 169 名 MFS 女性的单中心经验，以解决这些差距。

方法

比较了从未怀孕与曾经怀孕的 MFS 女性的临床、影像学和妊娠史，并描述了与妊娠相关的血管并发症。

结果

共有 74 名曾经怀孕的妇女有 112 名活产儿。在从未怀孕的女性中进行选择性主动脉根部置换的年龄较小（33 岁 vs 42 岁；P = 0.0026）。尽管从未怀孕与曾怀孕的女性之间的主动脉夹层患病率没有差异（23% 对 31%；P = 0.25），但前者的发病年龄往往更早（38 岁对 45 岁；P =0.07）。在观察到的产前直径 ≤ 4.5 cm 的“批准”妊娠中，与妊娠相关的平均主动脉直径保持稳定。总共有 5 处夹层与怀孕有关：2 处 A 型女性不知道自己的诊断；和 2 例 B 型和 1 例孤立的冠状动脉夹层，女性知道自己的诊断。妊娠期解剖率比非妊娠期高 5 倍。

结论

妊娠相关的 A 型夹层只发生在不知道自己诊断的患者中。B 型夹层仍然是一种不可预测的并发症。尽管从未怀孕组和从未怀孕组之间存在基线差异，但在围产期之外未观察到夹层风险的差异。那些产前主动脉直径在 4.0 到 4.5 厘米之间的人在整个怀孕期间表现出稳定的主动脉尺寸。这些发现为更新现有的美国 MFS 妊娠管理指南提供了依据。