Major adverse maternal cardiovascular-related events in those with aortopathies. What should we expect?

•We descriptively analyze the incidence and types of major adverse maternal cardiovascular-related events (MAMCRE) in women with aortopathy•Pre-pregnancy cross-sectional-area to height ratio was associated with increased MAMCRE, the majority of which were found to occur in heritable vascular disease and heritable fibrillinopathy•Heritable fibrillinopathy patients were more likely to require aortic surgery•Overall and emergent rates of cesarean section were higher in pregnant women with aortopathy•Postpartum hemorrhage was higher in pregnant women with aortopathy

Major adverse maternal cardiovascular-related events (MAMCRE) in aortopathy patients undergoing pregnancy are poorly defined. The aim was to assess for MAMCRE in pregnant patients with aortopathy or aortic enlargement in conotruncal defects (CTD), and determine if there are differences between groups.We conducted a single-center retrospective review of pregnant women (2000-2013) with hereditary vascular disease (HVD: BAV, COA), heritable fibrillinopathies (HF: MFS, EDS, LDS, FTAAS), and CTD with aortic dilatation (TOF, d-TGA, DORV). MAMCRE included: aortic dissection/surgery, therapeutic abortion, change in mode of delivery, and aortic growth > 0.5 cm within 1 year.We identified 73 patients/97 pregnancies (39/50 HVD, 15/20 HF, and 19/27 CTD). There were 14 MAMCRE (14%); 85% (n = 12) occurred in HV and HF patients and was associated with higher baseline cross-sectional-to-height (CSA/Ht) ratio (6.6 + 2.5 vs. 5.1 + 1.3, p = 0.005). There was more aortic surgery in the HF (vs. HV) (RR 3.9, p = 0.12). Only 2 MAMCRE (aortic growth) occurred in CTD. Overall and emergent C-section was higher than the general population (52% vs. 29%, p < 0.001 and 16% vs. 3%, p < 0.001) as was postpartum hemorrhage (PPH) (6% vs. 1.5%, p < 0.001).We describe the largest series of pregnant women with aortopathy and found a substantial incidence of MAMCRE, which was associated with higher pre-pregnancy CSA/Ht ratio. Rates of C-section and PPH were higher than the general population. Our data suggest that larger, multi-center studies are needed to define risks that predict MAMCRE/obstetric events in women with aortopathies, allowing optimal medical care during pregnancy.