Aim: to search for available evidence in the literature about nursing care for newborns with congenital heart disease in neonatal units. Method: An integrative review of the literature performed between July and August 2015, based on research in LILACS, PubMed and CINAHL databases. Result: Nine articles were identified according to established criteria. The information was analyzed and synthesized in three thematic categories: Detecting congenital heart disease; Providing care for the neonate in the intensive care unit; Family and the care measures to the newborn. Discussion: Neonatal screening for critical heart defects by pulse oximetry and nursing care at bedside surgeries were the main health care measures of nurses for infants with neonatal heart disease. Conclusion: There is a need for more professional involvement in the provision of care for these children in order to provide evidence-based clinical practice, and there are gaps in nurses' knowledge production that demonstrate this reality.