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Abstract
Pregnancy poses a threat to women with aortopathy. Conclusive data on the obstetric and aortic outcome in this risk collective, especially when it comes to aortic complications in the long term, are still missing. This study offers a comparative analysis of pregnancy-associated outcome in 113 consecutive women with Marfan syndrome or bicuspid aortic valve disease, including 46 ever-pregnant and 37 never-pregnant women with Marfan syndrome, and 23 ever-pregnant and 7 never-pregnant females with bicuspid aortic valve disease. The overall obstetric outcome was comparable between ever-pregnant women with Marfan syndrome and with bicuspid aortic valve disease (p = 0.112). Pregnancy-associated aortic dissection occurred in two women with Marfan syndrome (3%) during a total of 62 completed pregnancies, whereas no single case of aortic event occurred in women with bicuspid aortic valve disease during a total of 36 completed pregnancies (p = 0.530). In the long-term follow-up, aortic dissection occurred in 21% of ever-pregnant women with Marfan syndrome, but in none of the women with bicuspid aortic valve disease (p = 0.022). Proximal aortic surgery was performed with similar frequency in ever-pregnant women with Marfan syndrome and with bicuspid aortic valve disease in the long term (p = 0.252). However, ever-pregnant women with Marfan syndrome were younger when surgery was performed (44 ± 9 vs. 59 ± 7 years; p = 0.041). In Marfan syndrome, long-term growth of the aorta was comparable between ever-pregnant and never-pregnant women. Pregnancy thus exhibited an increased immediate aortic risk only in women with Marfan syndrome, but not in women with bicuspid aortic valve disease. Previous pregnancy did not relate to an increased long-term risk of adverse aortic events in women with Marfan syndrome or with bicuspid aortic valve disease.
Highlights
Pregnancy induces hemodynamic changes in response to the increased metabolic demands of the mother and fetus [1]
Mean age of ever-pregnant women was similar in Marfan syndrome and bicuspid aortic valve disease, both at initial diagnosis (p = 0.775) and at initial contact to our clinic (p = 0.980)
Use of aortoprotective medication in ever-pregnant females was more common with Marfan syndrome than with bicuspid aortic valve disease (p = 0.020)
Summary
Pregnancy induces hemodynamic changes in response to the increased metabolic demands of the mother and fetus [1]. Some authors have reported an increased risk of aortic events even during long-term follow-up after pregnancy [15,16], as well as of obstetric complications in pregnant women with Marfan syndrome [17,18]. Some pathogenetic similarities between aortopathy in Marfan syndrome and in bicuspid aortic valve disease have led authors to suggest that there is some increased aortic risk in pregnancy of women with bicuspid aortic valve disease [21,22]. Studies comparing the outcome of pregnancy in Marfan syndrome and in bicuspid aortic valve disease are currently missing. We provide the first analysis with a special focus on the comparison of immediate and long-term aortic outcomes of pregnancy in women with Marfan syndrome and with bicuspid aortic valve disease
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