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. 2025 Sep 8:zwaf574.
doi: 10.1093/eurjpc/zwaf574. Online ahead of print.

Cardiovascular outcomes and aortic growth in pregnant women with Turner syndrome: data from the ESC EORP Registry Of Pregnancy And Cardiac disease (ROPAC) III

J A Van Der Zande  1   2 , J Grewal  3 , A Franx  2 , J Hlebowicz  4 , L Demulier  5 , T C Konings  6 , A J Kinsara  7 , J C Mwita  8 , L Bal-Theoleyre  9 , W A Al Mahmeed  10 , L McGrath  11 , R Hall  12 , M R Johnson  13 , J W Roos-Hesselink  1 ; ROPAC investigators
Collaborators, Affiliations

Cardiovascular outcomes and aortic growth in pregnant women with Turner syndrome: data from the ESC EORP Registry Of Pregnancy And Cardiac disease (ROPAC) III

J A Van Der Zande et al. Eur J Prev Cardiol. .

Abstract

Background and aims: Data on cardiovascular outcomes and aortic growth in pregnant women with Turner syndrome is limited. We examine the cardiovascular and pregnancy outcomes in these women and analyze aortic growth throughout pregnancy.

Methods: The ROPAC III is a global, prospective, observational registry that enrolled pregnancies of women pre-pregnancy known with Turner syndrome from 2018 to 2023. We compared baseline characteristics and outcomes between women at low and moderate-to-high risk of aortic events and between women with a pregnancy conceived with assisted reproductive technology (ART) and those who conceived spontaneously. Linear mixed models were used to analyze aortic growth with the examinations and aortic diameter as independent and dependent variable, respectively, and random effects were used to account for repeated measurements.

Results: In total, 47 pregnancies were included, of which 49% were classified as moderate-to-high risk (bicuspid aortic valve, aortic dilatation and/or hypertension), and 60% occurred after ART. No maternal mortality or dissection occurred during or after pregnancy. Hypertensive disorders occurred in 26%. No differences in pregnancy outcomes were found when comparing the risk groups, but women who conceived after ART were delivered by Cesarean section more often (65% vs 42%; p=0.011) and had earlier deliveries (38 vs. 39 weeks; p=0.010) compared to women with a spontaneous conception. We observed a significant aortic growth at the sinus of Valsalva during pregnancy, however, aortic diameters during a median follow-up of seven months were not different from pre-pregnancy diameters.

Conclusions: Pregnancy outcomes in women known with Turner syndrome are better than reported before. While aortic dilatation may occur during pregnancy, it appears reversible postpartum. Attention for hypertension remains warranted.

Keywords: Aortic dilatation; Aortic dissection; Bicuspid aortic valve; Pregnancy; Turner syndrome.

Plain language summary

In a cohort of 47 pregnancies in women with Turner syndrome, diagnosed prior to pregnancy, no maternal mortality, aortic dissection or other cardiac events occurred. Pregnancy outcomes in women with known Turner syndrome are lower than previously reported, but hypertensive disorders in pregnancy are common.Significant aortic growth occurred during pregnancy, however, this growth appears to be reversible.

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