Introduction: There is very limited literature on the impact of bicuspid aortic valve (BAV) on pregnancy outcome despite the potential critical importance of this disorder. The importance of the topic becomes even more emphasized if we take into account a number of possibilities: cases of known or newly diagnosed BAV in pregnancy, differences in the functional status of the aortic valve during pregnancy, and the presence and degree of progression of aortopathy. (1-3)

Material and Methods: Relevant international scientific databases search were conducted to find original scientific papers, registries, meta-analyses and review papers on BAV in pregnancy that were published in the last 20 years.

Results: BAV in pregnancy can cause life-threatening cardiovascular events, among which aortic dissection stands out, although the development of valvular dysfunction or acute endocarditis should not be neglected. All acute complications of BAV in pregnancy potentially threaten maternal outcome or fetal demise. In the review of data from the literature, we provide key data on recommendations for diagnostic follow-up, recommendations for possible interventions in case of strict indications such as severe aortic stenosis, aortic regurgitation or aortic dissection, as well as elaboration of approaches in birth of a child planning.

Conclusion: According to currently available scientific data, it is crucial to individualize the approach to pregnant women with BAV in all phases, from diagnosis, individual counseling, detailed diagnostic monitoring, to planning the necessary cardiovascular interventions to optimal preparation and management of child delivery.