Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome. Issue 22 (25th May 2019)

• Record Type: Journal Article
• Authors: Cauldwell, Matthew; Steer, Philip J; Curtis, Stephanie L; Mohan, Aarthi; Dockree, Samuel; Mackillop, Lucy; Parry, Helen M; Oliver, James; Sterrenberg, Monique; Wallace, Suzanne; Malin, Gemma; Partridge, Gemma; Freeman, Leisa J; Bolger, Aidan P; Siddiqui, Farah; Wilson, Dirk; Simpson, Margaret; Walker, Niki; Hodson, Ken; Thomas, Katherine; Bredaki, Foteini; Mercaldi, Rebecca; Walker, Fiona; Johnson, Mark R
• Is Part Of: Heart. Volume 105:Issue 22(2019)
• Page Start: 1725
• Page End: 1731
• Publication Date: 2019-05-25
• Subjects: pregnancy -- marfan and associated disorders -- aortic dissection or intramural hematoma; Heart -- Diseases -- Treatment -- Periodicals; Cardiology -- Periodicals; 616.12
• DOI: 10.1136/heartjnl-2019-314817
• Languages: English
• ISSNs: 1355-6037

Abstract

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.