During the past decades, the incidence of pregnant women with heart disease in Denmark has decreased from 0.9% to 0.3%. Heart disease in connection with pregnancy still remains the commonest non-obstetric cause of death. Previously, rheumatic valvular heart disease constituted the majority of all forms of heart disease in pregnant women. At present, congenital heart disease constitutes at least 80% of the maternal heart disease. Progress in heart surgery has had the result that increasingly more complicated forms of cardiac deformities can be corrected so that more survive to adult life. The range of cardiac deformities has therefore increased in recent accounts concerning pregnant women with heart disease. Ventricular septum defect, atrial septum defect and persistent ductus arteriosus still constitute approximately 50% of the forms of heart disease in pregnant women. In Denmark, the incidence of congenital heart disease is approximately 0.6% but between 2.5% and 4.2% of infants of women with congenital heart disease also have heart disease. In counselling about the risks in pregnancy, the NYHA scale provides a good guideline. Alle conditions with pulmonary hypertension are contraindications for pregnancy. The greatest risk for death occurs early in the puerperium. Guidelines for control and treatment of pregnant women with heart disease are presented. It is concluded, that pregnant women with heart disease should be assessed and be followed up in centres with the necessary cardiac, obstetric and anaesthesiologic expertise.