Cardiovascular outcomes of pregnancy in Turner syndrome
Open accessAbstract
OBJECTIVES: Women with Turner syndrome (TS) are frequently counselled against pregnancy due to lack of data and unclear aortic dissection risk. However, with advances in fertility therapy, more women with TS are contemplating pregnancy. This study compared rates of adverse cardiovascular (CV) outcomes among: (1) pregnant and non-pregnant women with TS and (2) pregnant women with TS with/without structural heart disease. METHODS: Retrospective analysis of pregnant and age-matched non-pregnant controls with TS (2005-2017) across 10 CV centres was done. Data were collected at initial evaluation in pregnancy and outcomes were assessed to 6 months postpartum. Adverse CV events were defined as CV death, aortic dissection/rupture and/or aortic intervention. Non-pregnant age-matched controls were followed over the same time period. RESULTS: Sixty-eight pregnancies were included (60 women, mean age 33 years, 48% primigravid, 49% fertility therapy, 80% structurally normal heart, 25% XO karyotype). Based on American Society of Reproductive Medicine criteria, 10 pregnancies occurred in women stratified to high-risk category. There were no CV events in the pregnant women or in the non-pregnant women with TS. Obstetric events complicated 12 (18%) pregnancies with 9 (13%) attributed to hypertensive disorder of pregnancy. Fetal events included small for gestational age neonates (18%), preterm delivery (15%) and fetal death (3%). CONCLUSIONS: This study helps to refine the approach to pregnancy in women with TS. Among women with TS without structural heart disease, pregnancy does not impose an increased risk of CV outcomes. Among women with TS with structural heart disease, the risk of pregnancy is not as prohibitive as previously described but does require ongoing evaluation.
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Initial estimates of maternal deaths due to aortic dissections during pregnancy were much higher than in more recent studies, probably because pre-pregnancy cardiac evaluations were performed less frequently in older cohorts.
“Initial estimates of maternal deaths due to aortic dissections during pregnancy were much higher than in more recent studies, probably because pre-pregnancy cardiac evaluations were performed less frequently in older cohorts.510-513”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [510]
Assisted reproductive technologies were proposed to be a risk factor for aortic dilation or dissection, but recent studies did not show any difference in aortic complications.
“Assisted reproductive technologies were proposed to be a risk factor for aortic dilation or dissection, but recent studies did not show any difference in aortic complications.510”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [510]
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- Authors
- Grewal, J., Valente, A. M., Egbe, A. C., Wu, F. M., Krieger, E. V., Sybert, V. P., van Hagen, I. M., Beauchesne, L. M., Rodriguez, F. H., Broberg, C. S., John, A., Bradley, E. A., & Roos-Hesselink, J. W.
- Affiliations
- University of British Columbia (CA)
- Boston Children's Hospital (US)
- Mayo Clinic (US)
- University of Washington Medical Center (US)
- University of Washington (US)
- Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands
- Ottawa Heart Institute (CA)
- Emory University (US)
- Oregon Health & Science University (US)
- Children's National (US)
- The Ohio State University (US)
- Corresponding author
- Grewal, Jasmine
- ORCID
- Venue
- Heart
- Publisher
- BMJ
- Published
- 2020-07-15
- Volume
- 107
- Issue
- 1
- Pages
- 61-66
- ISSN
- 1355-6037 (ISSN-L)
- 1468-201X
- OpenAlex
- W3042685221
- Cited by
- 38
- References
- 18
- Citations by year
- 2026: 1 · 2025: 7 · 2024: 7 · 2023: 4 · 2022: 13 · 2021: 4 · 2020: 2
- FWCI
- 3.56
- Type
- journal article
- Language
- en
- Open access
- green
- pure.eur.nl
- Licence
- other-oa
- Locations
- Heart (publishedVersion)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, other-oa)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Funders
- University of British Columbia, Division of Cardiology
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Issues in Pregnancy
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
- Keywords
- Turner syndrome, pregnancy, cardiovascular outcomes, aortic dissection, aortic rupture, structural heart disease, hypertensive disorders of pregnancy, pregnancy counseling, cardiovascular risk
- MeSH terms
- Adult, Female, Humans, Infant, Newborn, Pregnancy, Pregnancy Complications, Pregnancy Outcome, Retrospective Studies, Turner Syndrome
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