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Congenital heart disease
Original research article
Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome
  1. Matthew Cauldwell1,
  2. Philip J Steer1,
  3. Stephanie L Curtis2,
  4. Aarthi Mohan3,
  5. Samuel Dockree4,
  6. Lucy Mackillop4,
  7. Helen M Parry5,
  8. James Oliver5,
  9. Monique Sterrenberg6,
  10. Suzanne Wallace7,
  11. Gemma Malin7,
  12. Gemma Partridge8,
  13. Leisa J Freeman9,
  14. Aidan P Bolger10,
  15. Farah Siddiqui11,
  16. Dirk Wilson12,
  17. Margaret Simpson13,
  18. Niki Walker14,
  19. Ken Hodson15,
  20. Katherine Thomas16,
  21. Foteini Bredaki17,
  22. Rebecca Mercaldi18,
  23. Fiona Walker18,
  24. Mark R Johnson1
  1. 1Chelsea and Westminster Hospital, Imperial College London, London, UK
  2. 2Department of Cardiology, Bristol Royal Infirmary, Bristol, UK
  3. 3Department of Obstetrics, St Michael’s Hospital Bristol, Bristol, UK
  4. 4Department of Obstetrics, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  5. 5Department of Obstetrics, Leeds Teaching Hospitals NHS Trust, Leeds, UK
  6. 6Department of Obstetrics, Princess Anne Hospital, Southampton, UK
  7. 7Department of Obstetrics, Nottingham University Hospitals, Nottingham, UK
  8. 8Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK
  9. 9Department of Cardiology, Norfolk and Norwich University NHS hospital, Norwich, UK
  10. 10East Midlands Congenital Heart Centre, Glenfield Hospital, University Hospitals of Leciester, Leicester, UK
  11. 11Department of Obstetrics, Leicester Royal Infirmary, Leicester, UK
  12. 12Department of Paediatric Cardiology, University Hospital of Wales, Cardiff, UK
  13. 13Scottish Adult Congenital Cardiac Service, Golden Jubilee National Hospital, Clydebank, UK
  14. 14Adult Congenital Heart Disease, Golden Jubilee National Hospital, Clydebank, UK
  15. 15Department of Obstetrics, Royal Victoria Infirmary, Newcastle upon Tyne, UK
  16. 16Department of Obstetrics, Royal Victoria Infirmary, Newcastle upon Tyne, UK
  17. 17University College London Hospital, London, UK
  18. 18Barts Health NHS Trust, London, UK
  1. Correspondence to Dr Matthew Cauldwell, Chelsea and Westminster Hospital, Imperial College London, London SW10 9NH, UK; mrc100{at}ic.ac.uk

Abstract

Objectives Information to guide counselling and management for pregnancy in women with Marfan syndrome (MFS) is limited. We therefore conducted a UK multicentre study.

Methods Retrospective observational study of women with MFS delivering between January 1998 and March 2018 in 12 UK centres reporting data on maternal and neonatal outcomes.

Results In total, there were 258 pregnancies in 151 women with MFS (19 women had prior aortic root replacements), including 226 pregnancies ≥24 weeks (two sets of twins), 20 miscarriages and 12 pregnancy terminations. Excluding miscarriages and terminations, there were 221 live births in 139 women. Only 50% of women received preconception counselling. There were no deaths, but five women experienced aortic dissection (1.9%; one type A and four type B—one had a type B dissection at 12 weeks and subsequent termination of pregnancy). Five women required cardiac surgery postpartum. No predictors for aortic dissection could be identified. The babies of the 131 (65.8%) women taking beta-blockers were on average 316 g lighter (p<0.001). Caesarean section rates were high (50%), particularly in women with dilated aortic roots. In 55 women, echocardiographic aortic imaging was available prepregnancy and postpregnancy; there was a small but significant average increase in AoR size of 0.84 mm (Median follow-up 2.3 months)

Conclusion There were no maternal deaths, and the aortic dissection rate was 1.9% (mainly type B). There with no identifiable factors associated with aortic dissection in our cohort. Preconception counselling rates were low and need improvement. Aortic size measurements increased marginally following pregnancy.

  • pregnancy
  • marfan and associated disorders
  • aortic dissection or intramural hematoma

https://doi.org/10.1136/heartjnl-2019-314817

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    Footnotes

    • Contributors MC conceived the idea for the study. Data for the study was collated by MC, SLC, LM, AM, SD, HMP, JO, MS, SW, GM, GP, LJF, APB, FS, DW, MS, NW, KH, KT, FB and RM. PS and MC analysed and approved the data. MC, PS, MRJ and FW wrote the first draft, which was reviewed and approved by all authors.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Disclaimer No sponsor played any role in the design and conduct of this study, including in data collection, analysis, data interpretation and in the preparation, review or approval of the manuscript.

    • Competing interests None declared.

    • Ethics approval The study protocol was approved by the research governance team at Imperial College Healthcare.

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

    • Patient consent for publication Not required.