Bicuspid aortic valve is the most common congenital heart malformation and predisposes patients to thoracic aortic aneurysms and aortic dissections. Current peripartum guidelines are extrapolated from other heritable causes of thoracic aortic disease and do not account for unique characteristics of bicuspid aortic valve patients. We therefore evaluated the prevalence of maternal and fetal complications of women with early-onset complications of bicuspid aortic valve disease in the UTHealth Bicuspid Aortic Valve Research Registry. We found that the rate of cardiovascular complications was high and that relatively few women received guideline-recommended care.