Marfan syndrome and pregnancy: maternal and neonatal outcomes

Authors

  • RA Curry,

    Corresponding author
    1. Department of Maternal and Fetal Medicine, Institute for Women's Health, University College London, London, UK
    2. Academic Department of Obstetrics and Gynaecology, Faculty of Medicine, Imperial College London, Chelsea and Westminster NHS Foundation Trust, London, UK
    • Correspondence: Dr RA Curry, Department of Maternal and Fetal Medicine, Institute for Women's Health, University College London, Room 401, 74 Huntley Street, London, WC1E 6AU, UK. Email r.curry@ucl.ac.uk

    Search for more papers by this author
  • E Gelson,

    1. Academic Department of Obstetrics and Gynaecology, Faculty of Medicine, Imperial College London, Chelsea and Westminster NHS Foundation Trust, London, UK
    Search for more papers by this author
  • L Swan,

    1. Adult Congenital Heart Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK
    2. National Heart and Lung Institute at Imperial College, London, UK
    Search for more papers by this author
  • D Dob,

    1. Magill Department of Anaesthesia, Intensive Care and Pain Management, Chelsea & Westminster NHS Foundation Trust, London, UK
    Search for more papers by this author
  • SV Babu-Narayan,

    1. Adult Congenital Heart Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK
    2. National Heart and Lung Institute at Imperial College, London, UK
    Search for more papers by this author
  • MA Gatzoulis,

    1. Adult Congenital Heart Disease Unit, Royal Brompton and Harefield NHS Foundation Trust, London, UK
    2. National Heart and Lung Institute at Imperial College, London, UK
    Search for more papers by this author
  • PJ Steer,

    1. Academic Department of Obstetrics and Gynaecology, Faculty of Medicine, Imperial College London, Chelsea and Westminster NHS Foundation Trust, London, UK
    Search for more papers by this author
  • MR Johnson

    1. Academic Department of Obstetrics and Gynaecology, Faculty of Medicine, Imperial College London, Chelsea and Westminster NHS Foundation Trust, London, UK
    Search for more papers by this author

Abstract

Objective

To report outcomes in a recent series of pregnancies in women with Marfan syndrome (MFS).

Design

Retrospective case note review.

Setting

Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals).

Sample

Twenty-nine pregnancies in 21 women with MFS between 1995 and 2010.

Methods

Multidisciplinary review of case records.

Main outcome measures

Maternal and neonatal mortality and morbidity of patients with MFS and healthy controls.

Results

There were no maternal deaths. Significant cardiac complications occurred in five pregnancies (17%): one woman experienced a type–A aortic dissection; two women required cardiac surgery within 6 months of delivery; and a further two women developed impaired left ventricular function during the pregnancy. Women with MFS were also more likely to have obstetric complications (OR 3.29, 95% CI 1.30–8.34), the most frequent of which was postpartum haemorrhage (OR 8.46, 95% CI 2.52–28.38). There were no perinatal deaths, although babies born to mothers with MFS were delivered significantly earlier than those born to the control group (median 39 versus 40 weeks of gestation, Mann–Whitney U–test, = 0.04). These babies were also significantly more likely to be small for gestational age (24% in the MFS group versus 6% in the controls; OR 4.95, 95% CI 1.58–15.55).

Conclusions

Pregnancy in women with MFS continues to be associated with significant rates of maternal, fetal, and neonatal complications. Effective pre-pregnancy counselling and meticulous surveillance during pregnancy, delivery, and the puerperium by an experienced multidisciplinary team are warranted for women with MFS.

Ancillary