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Congenital heart disease
Original research article
Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair
  1. Matthew Cauldwell1,
  2. Philip J Steer1,
  3. Samantha Bonner2,
  4. Omar Asghar3,
  5. Lorna Swan4,
  6. Kenneth Hodson5,
  7. Catherine E G Head6,
  8. Adam Daniel Jakes7,
  9. Nicola Walker8,
  10. Margaret Simpson8,
  11. Aidan P Bolger9,
  12. Farah Siddiqui10,
  13. Katherine M English11,
  14. Lucy Maudlin12,
  15. Dilip Abraham13,
  16. Andrew J Sands14,
  17. Aarthi R Mohan15,
  18. Stephanie L Curtis16,
  19. Louise Coats17,18,
  20. Mark R Johnson1
  1. 1 Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, London, UK
  2. 2 Department of Obstetrics, St Mary’s Hospital Manchester, Manchester, UK
  3. 3 Department of Adult Congenital Heart Disease, Manchester Heart Centre, St Mary’s Hospital, Manchester, UK
  4. 4 Department of Adult Congenital Heart Disease, Royal Brompton and Harefield NHS Foundation Trust, London, UK
  5. 5 Department of Obstetrics, Royal Victoria Infirmary, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
  6. 6 Adult Congenital Heart Disease Service, St Thomas’ Hospital, London, UK
  7. 7 Department of Obstetrics, St Thomas’ Hospital, London, UK
  8. 8 Scottish Adult Congenital Cardiac Service, Golden Jubilee National Hospital, Glasgow, Scotland, UK
  9. 9 Department of Adult Congenital Heart Disease, Leicester University Hospital, Leicester, UK
  10. 10 Department of Obstetrics, Leicester University Hospital NHS Trust, Leicester, UK
  11. 11 Department of Adult Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK
  12. 12 Department of Obstetrics, Norfolk and Norwich University Hospital, Norwich, UK
  13. 13 Department of Adult Congenital Heart Disease, Norfolk and Norwich University Hospital, Norwich, UK
  14. 14 Department of Adult Congenital Heart Disease, Royal Belfast Hospital for Sick Children, Belfast, UK
  15. 15 Department of Obstetrics, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  16. 16 Department of Adult Congenital Heart Disease, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  17. 17 Department of Adult Congenital Heart Disease, Freeman Hospital, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
  18. 18 Adult Congenital Heart Disease, Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK
  1. Correspondence to Professor Mark R Johnson, Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, 369 Fulham Road London, UK; mark.johnson{at}imperial.ac.uk

Abstract

Background The population of women of childbearing age palliated with a Fontan repair is increasing. The aim of this study was to describe the progress of pregnancy and its outcome in a cohort of patients with a Fontan circulation in the UK.

Methods A retrospective study of women with a Fontan circulation delivering between January 2005 and November 2016 in 10 specialist adult congenital heart disease centres in the UK.

Results 50 women had 124 pregnancies, resulting in 68 (54.8%) miscarriages, 2 terminations of pregnancy, 1 intrauterine death (at 30 weeks), 53 (42.7%) live births and 4 neonatal deaths. Cardiac complications in pregnancies with a live birth included heart failure (n=7, 13.5%), arrhythmia (n=6, 11.3%) and pulmonary embolism (n=1, 1.9%). Very low baseline maternal oxygen saturations at first obstetric review were associated with miscarriage. All eight women with saturations of less than 85% miscarried, compared with 60 of 116 (51.7%) who had baseline saturations of ≥85% (p=0.008). Obstetric and neonatal complications were common: preterm delivery (n=39, 72.2%), small for gestational age (<10th percentile, n=30, 55.6%; <5th centile, n=19, 35.2%) and postpartum haemorrhage (n=23, 42.6%). There were no maternal deaths in the study period.

Conclusion Women with a Fontan circulation have a high rate of miscarriage and, even if pregnancy progresses to a viable gestational age, a high rate of obstetric and neonatal complications.

  • Fontan procedure
  • pregnancy
  • perinatal mortality

https://doi.org/10.1136/heartjnl-2017-311763

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    Footnotes

    • Contributors MC conceived the idea for the manuscript and contacted all other authors who assisted in providing data. PJS and MC analysed the data. MC, PJS and MRJ wrote the first draft and this was edited and approved by all authors.

    • Competing interests None declared.

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