Maternal, pregnancy, and neonatal outcomes for women with Marfan syndrome.

Abstract

Marfan syndrome (MFS) is an autosomal dominant hereditary disorder which affects cardiovascular structure and function. With medical advances, more women with MFS experience pregnancy, which may increase maternal and neonatal risk. Existing research has been limited by small or clinical samples. This study examines the association of MFS and adverse maternal, neonatal, and obstetric outcomes. We conducted a cross-sectional study using the discharge abstract database, containing all labor and delivery hospitalizations in Canada (excluding Quebec) from fiscal years 2004-2015 where women delivered a live- or stillbirth. We measured maternal and neonatal morbidity, preterm births (<37 weeks), small-for-gestational-age births, perinatal mortality, and adverse maternal cardiovascular events. For each outcome, we calculated the absolute risk for women with and without MFS and used generalized estimating equations with a logit function to calculate odds. Overall, 2,682,461 women delivered a live or stillborn infant in Canada during the study period, with 135 birth events to women with MFS. Women with MFS did not have significantly higher odds of severe maternal morbidity during their delivery (aOR:1.3; 95%CI: 0.4-4.0). Similarly, their infants did not have significantly higher odds of neonatal morbidity. However, infants born to women with MFS were significantly more likely to be born preterm (aOR:2.6; 95%CI: 1.6-4.3) and to be small-for-gestational-age (aOR:1.8; 95%CI:1.0-3.1). This population-based study indicates that, although some women with MFS may experience higher odds of maternal and/or neonatal morbidity during labor and delivery, the majority of women with MFS can have healthy births with proper clinical management.