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

2020 ◽  
Vol 9 (4) ◽  
pp. 1124
Author(s):  
Betül Toprak ◽  
Katalin Szöcs ◽  
Elvin Zengin-Sahm ◽  
Christoph Sinning ◽  
Amra Hot ◽  
...  

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.

2015 ◽  
Vol 66 (12) ◽  
pp. 1409-1411 ◽  
Author(s):  
Evaldas Girdauskas ◽  
Mina Rouman ◽  
Kushtrim Disha ◽  
Andres Espinoza ◽  
Martin Misfeld ◽  
...  

2018 ◽  
Vol 39 (suppl_1) ◽  
Author(s):  
R M Radke ◽  
A Florian ◽  
S Orwat ◽  
C Meier ◽  
M Bietenbeck ◽  
...  

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Hanigk ◽  
E Burgstaller ◽  
H Latus ◽  
N Shehu ◽  
J Zimmermann ◽  
...  

Abstract Introduction Bicuspid aortic valve (BAV) disease leads to deviant complex helical flow patterns in the aorta - especially in the mid-ascending (AoA) part. In association with congenital BAV, aortic wall alterations such as aortic dilatation and dissection may occur. Among others, wall shear stress (WSS) could be one parameter to contribute to the prediction of the long-term outcome of patients with BAV. 4D-flow in cardiovascular magnetic resonance has been established as a valid method to estimate WSS. Purpose The aim of this study is to reevaluate WSS and comparing it to values generated in the same patient cohort with bicuspid aortic valve disease in 2008. No one of the above had aortic dilation in 2008 but proven helical flow pattern. The long term follow-up study might show changes in WSS over the period of ten years. Methods Ten complete 4D flow datasets of patients (age at follow-up: median 34.5 years; range 19–41 years) with bicuspid aortic valve disease without enlargement of the aorta were obtained in 2008 and reevaluated in 2018/2019 in the same patient collective. Mean WSS values were calculated with identical specific software tools. All data were analyzed by two experienced investigators. Results Aortic diameters at the level of the mid AoA did not change significantly in the 10-year period. The WSS values were lower in 2018 at all levels of the ascending aorta (Table 1). Indexed aortic diameters at the level of the mid ascending aorta did not change, median difference 0.06 cm/m2 (range −0.1 cm/m2 to 0.2 cm/m2; p=0.28), absolute values of indexed AoA diameters in 2018/2019 ranged from 1.27 cm/m2 to 2.2 cm/m2 (median 1.76 cm/m2). Table 1 WSS magnitudinal [N/m2] 2008 (n=10) 2018 (n=10) Median difference p-value median range median range Level aortic bulb 0.95 0.80–1.46 0.42 0.34–0.82 −0.53 <0.01 Level mid ascending aorta 0.72 0.40–0.98 0.39 0.34–0.59 −0.33 <0.01 Level brachiocephalic trunc (BCT) 0.71 0.38–1.03 0.40 0.37–0.61 −0.31 <0.01 Conclusions Indexed AoA diameters in BAV disease did not change significantly over a 10-year period. WSS of AoA was less compared to values generated in 2008. This might be explained by a slight alteration in hemodynamic flow patterns by the aging aorta, but not by changes of the aortic diameters. Possibly a drop of WSS in BAV could serve as a marker for a benign long term course.


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