BAV morphology and especially the presence and extent of a raphe is of clinical importance, as a complete raphe predisposes to more valvular dysfunction and aortopathy. Regarding variations in coronary anatomy, it turns out that patients with left-right BAVs without raphe and patients with BAV and CoA more often have a left dominant coronary artery system. Patients with left-right BAVs without raphe also seem more at risk of developing significant coronary artery disease. In addition, separate ostia of the LAD and LCX are more common in these patients. Patients with Turner syndrome and BAV do not show significant differences in coronary dominance as compared to patients with Turner syndrome with TAV and to patients with isolated BAV. However, patients with TS and BAV show more separate ostia of the LAD and LCX as compared to patients with isolated BAV. In BAV with associated complex congenital heart disease, a high take-off of the coronary arteries, occurred more frequently than reported in structurally normal hearts and in hearts with isolated BAV. Finally, there should be awareness of possible bilateral semilunar valvular disease, which is associated with strictly bicuspid BAVs and in many cases related to chromosomal abnormalities.