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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. [ABSTRACT FROM AUTHOR]

Introduction: The risk of peri-partum vascular dissection in women with Marfan syndrome (MFS) is uncertain due to ascertainment bias, pre-partum lack of knowledge of diagnosis and of potential underlying large aortic aneurysm, and insufficient imaging data to define a robust threshold for increased dissection risk. Aim: We describe a single-center experience with a large number of MFS women, including those with serial peri-partum imaging. Methods: Relevant factors between ever- and never-pregnant women were compared in 147 of 196 women fulfilling Ghent criteria (33 deceased or lost to follow-up; 16 not yet consented; 65 [44%] ever-pregnant with 103 live births). Changes in aortic diameters during observed pregnancies were evaluated with ANOVA. Results: Prophylactic aortic root replacement rates were similar in never- vs. ever-pregnant groups (41 vs. 38%) but were performed at a younger age in the former group (31 vs. 42 years, p=0.002). Aortic dissections, especially Type B, occurred more commonly in ever-pregnant women (28 vs. 18%, p=0.02) but at an earlier age in never-pregnant women (31 vs. 46 years, p = 0.005). Five dissections were associated with pregnancy: two Type A in the third trimester in women unaware of their MFS diagnosis, and two Type B and one isolated coronary artery dissection post-partum in women with non-prohibitive aortic root diameters. Of 44 observed, 'sanctioned' pregnancies, mean aortic diameters were largely stable throughout pregnancy, including 15 pregnancies with pre-pregnancy diameters of 4.0-4.5 cm (see graph). Conclusions: In our large series of MFS women, pregnancy-related Type A dissection only occurred in women who were unaware of their diagnosis, whereas post-partum Type B dissection remains an unpredictable complication. Stability of aortic dimensions, notably in those with pre-partum aortic root diameters of 4.0-4.5 cm, provides a rationale to update existing U.S. guidelines for the management of pregnancy in MFS. [ABSTRACT FROM AUTHOR]