2021 Volume 37 Issue 2 Pages 117-123
Background: Individualized approach is the key for the successful management of congenital heart disease (CHD) complicated by congenital intestinal atresia (CIA). There are few reports for diagnosing, outcomes and prognostic factors in these patients.
Methods: Medical records of 61 patients (40 males and 21 females; median gestational week, 38; median birth weight 2.5 kg) with CHD and CIA treated between 1994 and 2011 were retrospectively evaluated. Patients with both CHD and CIA were evaluated by a subgroup of CIA.
Results: CHD diagnoses included ventricular septal defect (n=22), patent ductus arteriosus (n=8), tetralogy of Fallot (n=7), and atrial septal defect (n=5). CIA included anal atresia (n=31), esophageal atresia (n=17), duodenal atresia/stenosis (n=10), and Hirschsprung’s disease (n=10). The overall survival rate of patients was 83%. Thirty-one had chromosomal abnormalities. Of those, 7 patients died. Forty patients received surgeries for both CHD and CIA consequently. In 1 patient with anal atresia, the site of stoma influenced cardiac operation. One patient with esophageal atresia underwent CIA operation after cardiac palliation for high pulmonary blood flow. One patient had duodenal obstruction associated with suture failure for high pulmonary blood flow. Three patients with Down syndrome that underwent proctostomy for Hirschsprung’s disease died from fulminant enteritis.
Conclusions: Our individualized approaches provided a high survival rate in neonates with CHD and CIA. Our strategy included CIA operation preceded CHD operation. The high pulmonary flow needed prompt intervention to prevent circulation failure and suture failure. Down syndrome patients undergoing proctostomy should be carefully monitored for post-surgical fulminant gastroenteritis.
