Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications

Toprak B, Szöcs K, Zengin-Sahm E, Sinning C, Hot A, Bannas P, Hecher K, Hüneke B, Mir TS, Rybczynski M, Girdauskas E, Blankenberg S, von Kodolitsch Y.
J Clin Med. 2020 Apr 15;9(4). pii: E1124. doi: 10.3390/jcm9041124.
PMID: 32326432 Free Article
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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.

 

Figure 1 Aortic dissection. Kaplan–Meier curve analysis displays the cumulative probability of freedom from aortic dissection, with comparison between ever-pregnant women with Marfan syndrome (MFS) and with bicuspid aortic valve disease (BAV), and within Marfan group according to pregnancy history. Never-pregnant women with BAV are not shown due to the limited number of cases and lack of events.

 

Figure 2 Proximal aortic surgery. Kaplan–Meier curve analysis displays the cumulative probability of proximal aortic surgery, with comparison between ever-pregnant women with Marfan syndrome (MFS) and with bicuspid aortic valve disease (BAV), and within Marfan group according to pregnancy history. Never-pregnant women with BAV are not shown due to the limited number of cases and lack of events.

 

Figure 3 Distal aortic repair. Kaplan–Meier curve analysis displays the cumulative probability of freedom from distal aortic repair, with comparison between ever-pregnant women with Marfan syndrome (MFS) and with bicuspid aortic valve disease (BAV), and within Marfan group according to pregnancy history. Never-pregnant women with BAV are not shown due to the limited number of cases and lack of events.

 

Figure 4 Long-term aortic growth at the level of the aortic root (a), ascending aorta (b), descending aorta (c), and abdominal aorta (d) in women with Marfan syndrome (MFS; left panel) and with bicuspid aortic valve disease (BAV; right panel) according to pregnancy history. Red lines visualize slopes of growth for each subgroup. N identifies number of individuals with available diameters at first and last contact.

 

source:https://pubmed.ncbi.nlm.nih.gov/32326432/

 

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