Although women with heart disease are rare in the obstetric population, they are the main cause of maternal mortality and morbidity. During pregnancy large hemodynamic changes take place. In developing countries, rheumatic heart disease is still a major problem. While in the developed countries congenital heart disease is most often encountered, but acquired heart diseases such as aortic dissection and acute coronary syndrome cause the highest maternal mortality rates. Specific Cardiac Disease. Congenital cardiology covers a wide spectrum of heart diseases, ranging from simple shunts lesions to very rare and complex disorders. Patients with unrepaired lesions, cyanotic lesions, diminished systemic ventricular function, complex congenital heart disease, left ventricular outflow tract obstruction, and mechanical valves are at highest risk of complications during pregnancy. Although other conditions such as peripartum cardiomyopathy, Marfan syndrome, and pulmonary arterial hypertension are also associated with high maternal mortality and compromised pregnancy outcome. Management. Pre-pregnancy evaluation should focus on identifying and quantifying the risk to the mother and on the outcomes of the potential pregnancy. Current medication use should be discussed and if necessary changed, since some medication may be harmful for the unborn child. The most common cardiac complications in pregnant patients with heart disease are arrhythmias and heart failure. The balance between maternal and fetal outcome should always be considered in treatment choices and should be discussed with the future parents. Generally, vaginal delivery is preferred in women with adequate cardiac output. Neonatal outcome is strongly correlated with maternal outcome.