Abstract： Objective To analyze the early outcomes of open heart surgery for congenital heart diseases in sixty low birth weight infants and premature infants. Methods Sixty low birth weight infants （body weight＜2 500 g） and premature infants with congenital heart diseases undergoing surgical repair from May 2003 to October 2011 were studied retrospectively in Guangdong Cardiovascular Institute. There were 43 male patients and 17 female patients with their mean gestational age of 33.5±4.1 weeks （ranging from 26 to 42 weeks） and mean age at operation of 24.9±12.5 d（ranging from 4 to 55 d）. Among them there were 47 premature infants with their mean birth weight of 1 729.3±522.5 g（ranging from 640 to 2 500 g）and mean weight at operation of 1 953.2±463.6 g （ranging from 650 to 2 712 g）. All the patients received preoperative treatment in newborn intensive care unit（NICU） and underwent surgical repair under general anesthesia, including 29 patients without cardiopulmonary bypass （CPB）and 31 patients with CPB . All surviving patients received postoperative monitoring and treatment in NICU, and their postoperative complications and in-hospital death were reported. Results A total of 13 patients died during hospitalization with a total in-hospital mortality of 21.7%（13/60）, including 4 intra-operative deaths, 6 early deaths （within 72 h postoperatively） and 3 patients giving up postoperative treatment. CPB time was 121.0±74.7 min, aortic clamp time was 74.8±44.7 min, and postoperative mechanicalventilation time was （136.9±138.1）h. Thirteen patients underwent delayed sternal closure. Eight patients underwentreexploation for postoperative bleeding. Ten patients had severe pneumonia, 2 patients had pulmonary hypertensive crisis, and 8 patients had low cardiac output syndrome. All the postoperative complications were resolved or improved after proper treatment. Follow-up was complete in 47 patients from 2 to 12 monthes, and all the patients were alive during follow-up. Conclusion Early surgical repair for low birth weight infants and premature infants with congenital heart diseases is safe and effective.