It is exceedingly uncommon for a woman in the UK to die during pregnancy, with maternal mortality in the region of one death per 10,000 maternities1. Although there have been very significant improvements in antenatal care, such as a marked reduction in the number of deaths due to thromboembolic disease, other areas are trailing behind. One such area is cardiac disease – now the leading cause of maternal death in the UK. These relatively rare deaths also mask the much larger issues of maternal, fetal and perinatal morbidity. The most recent Confidential Enquiries into Maternal Death and the new European Society of Cardiology guidelines summarised in this issue of the PCCJ highlight the major clinical issues and attempt to provide consensus opinion regarding optimal care in what is a relatively evidence-sparse field.