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Objective: To evaluate incidence and mortality of congenital heart disease in very low birthweight babies. Method: Retrospective analysis of a 12 year period. Results: Forty seven babies were diagnosed with severe congenital heart disease. The most common lesions were ventricular septal defect and coarctation of the aorta. Mortality attributed to congenital heart disease was 32%. Coarctation of the aorta, the second most common lesion, was fatal in 62% of cases. Closure of a patent ductus arteriosus with indomethacin proved to be detrimental in babies with undiagnosed coarctation, causing rapid deterioration in some. Conclusion: Very low birthweight neonates with severe congenital heart disease have a higher mortality than babies with higher birth weight. A contributing factor is closure of a patent ductus arteriosus if an underlying lesion has not been recognised. This could be of significance if the use of prophylactic treatment with indomethacin becomes more common. [ABSTRACT FROM AUTHOR]