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The objective was to demonstrate the consequences on maternal health, in women with diagnosed and undiagnosed MFS at the time of pregnancy and childbirth. By using national health care registries, we identified all pregnancy related outcomes, from women with MFS (<jats:italic>n<\/jats:italic> = 183) and an age\u2010matched background population (<jats:italic>n<\/jats:italic> = 18,300). We found 91 pregnancies during follow\u2010up. Significantly fewer women with MFS gave birth, compared to the background population. No women with known MFS had a pregnancy related aortic dissection but complications related to the cervix were increased (HR:19.8 [95% CI:2.2\u2013177.5]). Fifty women with MFS were undiagnosed at the time of their first pregnancy and\/or childbirth. Among these, there were more birth canal related complications HR:27.2 (95% CI: 2.3\u2013315.0), preeclampsia (HR:2.25 [95% CI: 1.11\u20134.60]), fetal deaths (HR:12.3 [95% CI: 1.51\u201399.8]), and all delivery\u2010related dissections came from this subgroup. In conclusion, undiagnosed women with MFS experienced more pregnancy and childbirth related complications including fetal death, birth canal issues, preeclampsia, and aortic disease, which emphasizes the need for an early MFS diagnosis and special care during pregnancy and childbirth.<\/jats:p>","DOI":"10.1002\/ajmg.a.62122","type":"journal-article","created":{"date-parts":[[2021,2,18]],"date-time":"2021-02-18T18:16:26Z","timestamp":1613672186000},"page":"1414-1420","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Maternal health and pregnancy outcome in diagnosed and undiagnosed Marfan syndrome: A registry\u2010based study"],"prefix":"10.1002","volume":"185","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3452-8298","authenticated-orcid":false,"given":"Kristian A.","family":"Groth","sequence":"first","affiliation":[{"name":"Department of Cardiology Aalborg University Hospital  Aalborg 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