There is a heterogeneous population of young women with cardiovascular disease contemplating pregnancy. Many of the conditions are rare and require teams with expertise in the management of such patients. A specific congenital or acquired cardiovascular anomaly and its physiology must be understood. The nature of prior surgical procedures and the residua and sequelae following therapy are essential to manage a pregnant woman with cardiovascular disease. Physiologic changes during pregnancy and after delivery must be known. Counseling includes maternal and fetal risk stratification and genetic counseling regarding inherited disorders. The outcome of pregnancy is favourable in many women with good functional class. These patients can be reassured. A multidisciplinary approach may be crucial to manage this population during pregnancy, labour, delivery and in the postpartum period to avoid serious maternal and fetal complications. Women with intermediate and high risk pregnancy must be followed and managed in a high risk pregnancy unit and a team from obstetricians, cardiologists, anesthetists, pediatricians, neonatologists and cardiac surgeons who are skilled in high risk pregnancies. Vaginal delivery is the method of choice in many women (class 1 woman) and cesarean section is seldom a cardiac indication. Contraindications for pregnancy are: poor maternal functional class, poor ventricular function, severe cyanosis without pulmonary hypertension (oxygen saturation < or = 85%), pulmonary vascular disease, severe mitral stenosis, severe left ventricular outflow tract obstruction, Marfan syndrome with aortic dilatation (> 40 mm) and aortic valve involvement (moderate to severe aortic regurgitation), symptomatic hypertrophic obstructive cardiomyopathy. Preconception counseling must be offered if unfavourable outcome is likely. Tubal ligation is probably the safest and most appropriate method of sterilization if there is a high risk pregnancy.