Heart disease is the leading cause of maternal mortality in the UK. Deaths from acquired conditions such as ischaemic heart disease are increasing and often occur in patients with no history of heart disease, thus emphasising the need for vigilance for risk factors in women of childbearing age. All women with known heart disease should have pre-pregnancy counselling to assess for maternal and fetal risk. Women deemed to be at moderate or high risk should be under the care of a specialist antenatal team with experience of managing women with heart disease in pregnancy. Conditions that are considered particularly high risk (mortality >10%) include Marfan syndrome with dilated aortic root, severe left heart obstructive lesions, pulmonary hypertension, and severe left ventricular dysfunction. This article reviews the management of women with heart disease during pregnancy, labour and in the puerperium.