Cardiovascular malformations and oesophageal atresia

Abstract

In a retrospective study of 345 patients with esophageal atresia, cardiovascular malformations were present in 96 patients (27.8%) and were the commonest associated malformations: 83 (86.5%) of these patients died, compared with 40 out of 156 patients (25.6%) who had esophageal atresia with no other malformations. Persistent ductus arteriosus was the commonest lesion, followed by ventricular septal defect, secundum atrial septal defect, right sided aortic arch, dextrocardia, anomalous right subclavian artery, and defects of the atrioventricular valves. Patients with cardiovascular malformations had a lower birthweight, a shorter gestation, and were more likely to have an abnormally low fetal growth rate. A right sided aortic arch or an anomalous right subclavian artery in patients with esophageal atresia should suggest the possibility of thymic or parathyroid hypoplasia or agenesis. Now that esophageal atresia itself can be corrected, the associated cardiovascular malformations are of increasing importance.