Endocrine Abstracts (2019) 65 P354 | DOI: 10.1530/endoabs.65.P354

Maternal cardiovascular risk and pregnancy outcomes in turner syndrome - new evidence supports current guidance

Matilde Calanchini1,2, James Bradley-Watson3, Elizabeth Orchard3 & Helen E Turner1


1Oxford Centre for Diabetes, Endocrinology and Metabolism, Oxford University Hospital NHS Trust, Oxford, UK; 2Department of Systems Medicine, Endocrinology & Metabolism Unit, University of Rome Tor Vergata, Rome, Italy; 3ACHD Cardiology, Oxford University Hospital NHS Trust, Oxford, UK


Introduction: The risk of maternal death from aortic-dissection(AoD) during pregnancy/post-partum in TS is increased, due to TS-associated risk factors (bicuspid-aortic-valve(BAV), aortic-coarctation, aortic-dilatation, hypertension) and the increased cardiovascular strain of pregnancy itself. TS-guidelines advice against pregnancy in the presence of severe aortic-dilatation or moderate dilatation with AoD-risk factors; and after aortic surgery a high risk remains. However, few studies focus on cardiovascular outcomes in pregnant TS.

Methods: Retrospective study on 42 life-birth pregnancies among 25 TS women. Echocardiography/CMR pre-pregnancy(<2y pre-partum) and post-pregnancy(<2y post-partum) were collected. Measurements of sinuses of Valsalva(SoV) and ascending aorta(AA) were reviewed and adjusted for body-surface-area(ASI). AA-ASI≥20 mm/m2 defined moderately dilated aorta, ≥25 mm/m2 severely dilatated. Change in diameter pre- and post-pregnancy were compared with the growth rate of 70 nulliparous-TS.

Results: Cardiac-status at preconception was evaluated in 11/25 women. 2/11 had AA-ASI≥20 mm/m2, respectively, with BAV and hypertension. 3/11 had BAV, with repaired aortic-coarctation in 2. 1/25 had a previous AA-replacement. 2/25 had twin pregnancies with oocyte-donation. Post-pregnancy SoV and AA were significantly increased compared with pre-pregnancy values. The annual aortic-diameter-growth pregnancy-related was higher vs. nulliparous women. Among the five women with pre-exisiting AoD-risk factors, aortic-growth was higher, although not significant. There were no peri/post-pregnancy AoD.

Time between cardiac-scans 3.6 (1.6–4) yPre-pregnancyPost-pregnancyPregnantNulliparous
SoV, mm28.0 ± 2.629.5 ± 3.8<0.000
AA, mm25.9 ± 3.728.1 ± 5.5<0.000
SoV growth-rate, mm/y0.53 ± 0.680.13 ± 0.590.044
AA growth-rate, mm/y0.61 ± 0.670.22 ± 0.830.142

Conclusions: This is the first study evaluating pre-pregnancy and post-pregnancy cardiovascular status in TS and suggest that aortic diameters increase during pregnancy, especially in women with pre-pregnancy AoD-risk factors. These data support the current guidelines regarding careful cardiovascular evaluation prior to any pregnancy and close pre and post-pregnancy monitoring.

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