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Original research
Cardiovascular outcomes of pregnancy in Turner syndrome
  1. Jasmine Grewal1,
  2. Anne Marie Valente2,
  3. Alexander C Egbe3,
  4. Fred M Wu2,
  5. Eric V Krieger4,
  6. Virginia P Sybert5,
  7. Iris M van Hagen6,
  8. Luc M Beauchesne7,
  9. Fred H Rodriguez8,
  10. Craig S Broberg9,
  11. Anitha John10,
  12. Elisa A Bradley11,
  13. Jolien W Roos-Hesselink6
  14. the AARCC Investigators
  1. 1Division of Cardiology, St.Paul's Hospital, The University of British Columbia, Vancouver, British Columbia, Canada
  2. 2Division of Cardiology, Boston Children's Hospital, Harvard University, Boston, Massachusetts, USA
  3. 3Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA
  4. 4Seattle Adult Congenital Heart Service, University of Washington Medical Center, Seattle, Washington, USA
  5. 5Division of Medical Genetics, University of Washington School of Medicine, Seattle, Washington, USA
  6. 6Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands
  7. 7Division of Cardiology, University of Ottawa Heart Institute, Ottawa, Ontario, Canada
  8. 8Division of Cardiology, Emory University School of Medicine, Atlanta, Georgia, USA
  9. 9Division of Cardiology, Oregon Health & Sciences University, Portland, Oregon, USA
  10. 10Division of Pediatric Cardiology, Children's National Health System, Washington, DC, USA
  11. 11Division of Cardiovascular Medicine, Ohio State University, Columbus, Ohio, USA
  1. Correspondence to Dr Jasmine Grewal, The University of British Columbia, Vancouver, BC V6P5H6, Canada; jasmine.grewal{at}vch.ca

Abstract

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.

  • pregnancy
  • aortic dissection or intramural hematoma
  • aortic coarctation
  • bicuspid aortic valve
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Footnotes

  • Twitter @fredwumd

  • Contributors All of the authors have contributed to study planning, patient identification, data collection, data reporting and manuscript writing. JG is responsible for the overall content of this manuscript as guarantor.

  • Funding JG is supported by research funds awarded by the University of British Columbia Division of Cardiology. AMV is supported by the Brigham and Women's Hospital Barton and Weinberg Family Fund.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were involved in the design, or conduct, or reporting, or dissemination plans of this research. Refer to the Methods section for further details.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available on reasonable request. Deidentified patient data available from the first author.

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