While in the past, rheumatic heart disease accounted for the largest number of pregnant women with heart disease, the incidence has declined over the years. On the other hand, because of advances in medical treatment and surgical management, the ratio of adolescent females with congenital heart disease increased significantly during the last decade. Pregnancy is associated with many physiologic adjustments such as changes in blood volume, stroke volume, and cardiac output, and may result in deleterious clinical effects in patients with congenital heart disease. Although a good outcome for these women can often be expected, congenital heart disease still presents a high-risk pregnancy involving both mother and infant. The actual risk depends on the type of malformation and functional impairment of the mother. To bring these women safely through pregnancy, a strong cooperation between pediatric and adult cardiologists, obstetrician, and other physicians is necessary.