Necrotizing enterocolitis in pulmonary atresia with intact ventricular septum

Necrotizing enterocolitis (NEC) occurred in 2 neonates with pulmonary atresia and intact ventricular septum. Both infants underwent open heart surgery for the insertion of a right ventricular outflow tract patch under profound hypothermia, surface cooling and a fibrillating heart, without circulatory arrest. In both patients the development of NEC preceded both the cardiac catheterization study and open heart surgery. Urgent repair of the cardiac lesion was undertaken in an attempt at improving the critical bowel wall ischemia. Postoperatively, exacerbation of the NEC reached an advanced stage rapidly leading to the death of the patients. Recommendations regarding the management of future cases exhibiting this potentially lethal combination of disease are presented.