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Objective: Because of their poor clinical status, infants may require surgery for congenital heart disease regardless of weight or prematurity. This retrospective review describes a single-centre experience with open-heart surgery in low-weight infants., Methods: From November 1997 to December 2006, 411 open-heart surgery procedures were performed in neonates. This included 46 consecutive infants weighing less than 2500 g, who underwent cardiopulmonary bypass for correction of congenital heart defects (n=34) or Norwood stage I palliation of hypoplastic left heart syndrome (HLHS) (n=12). In the low-weight group were 23 males and 23 females with a median age of 10 days and a median weight of 2.26 kg (range: 1.28-2.49 kg)., Results: Early mortality was 8.2% in patients weighing more than 2.5 kg and 13% in the low-weight group. Within the low-weight group, weight at surgery, history of prematurity and prevalence of additional extracardiac malformations did not influence early mortality. At a median follow-up time of 32 months overall mortality was 21%. Thirty-four patients had a neurological follow-up examination 30 months postoperatively. Of the 34 survivors, 11 showed neurological deficits., Conclusions: In our patient population, early mortality was higher for infants weighing less than 2.5 kg. However, within the low-weight group, lower weight at surgery or history of prematurity was not associated with a higher mortality or bad neurological outcome.