Includes bibliographical references. / ith the advances made in the management of cardiac conditions, much importance has been placed on the maternal outcome in pregnancies complicated by heart disease. However, to enable attending clinicians to provide suitable counseling and manage the pregnancy appropriately, the potential complications arising in the fetus and neonate also require attention. Adverse neonatal and perinatal outcome is more common in pregnant women with cardiac disease. Analysis of the available data pertaining to the South African population is important, as this population’s profile, like that of Africa, differs from that of industrialized countries. The relevance of maternal heart disease is highlighted by the National Committee for the Confidential Enquiries into Maternal Deaths (NCCEMD) in South Africa ( http://www.doh.gov.za/docs/reports/2012/Report_on_Confidential_Enquiries_into_ Maternal_Deaths_in_South_Africa ). Objectives To describe the perinatal outcome in women with heart disease and to determine whether there is an associated adverse outcome related to babies born to mothers with heart disease. Methods 82 patients were collected serially over 18 months. Neonatal outcome was recorded. Adverse neonatal outcome was defined as perinatal mortality, admission to NICU and the need for delivery room resuscitation. Results Perinatal mortality rate in this cohort was good, and better than the rate in the general population from whence this cohort came, but was linked to a high rate of obstetric intervention. The rate of adverse neonatal outcome is better than the rate in industrialized countries. Conclusion Perinatal outcome is good when mothers with heart disease are managed in a multidisciplinary clinic.