Summary
Approximately 1600 children with a congenital heart defect are born in the Netherlands every year. The presentation of these children will be diverse and sometimes misleading, because of the many different heart defects possible. Moreover, not every child with a congenital heart defect will present in the first days of life. The first interventions have to be adequate and determine largely the clinical course. We present an overview of the most frequently seen congenital heart defects and their first presentation. We discuss initial treatment, with special attention to the indications for oxygen supply, prostaglandin therapy and criteria for safe transport.