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.
- marfan and associated disorders
- aortic dissection or intramural hematoma
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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.