The impact of aortic arch failure on the management and results of treatment in congenital esophageal atresia patients

Background: Surgical treatment of newborns with esophageal atresia (EA) and aortic arch failure is not established. Patients with EA and aortic arch failure have higher incidence of early complications and similar survival compared to patients without aortic defects.

Aim of this study is to evaluate the impact of aortic arch failure on surgical treatment and outcome of EA patients.

Material and methods: During 2006-2010 aortic arch failure was diagnosed in 6 out of 29 (21%) newborns treated for EA. Patients’ data were analyzed retrospectively.

Results: Aortic arch failures: right aortic arch (RAA) – 2, accessory right subclavian artery (ARSA) – 2, accessory left subclavian artery (ALSA) – 1, interrupted aortic arch (IAA) – 1. Approach and type of EA surgery: RAA (right thoracotomy – esophageal anastomosis – 1, left thoracotomy [dextrocardia] – esophageal fistula ligation – 1), ARSA (right thoracotomy: esophageal anastomosis – 1, esophageal division – 1), ALSA (right thoracotomy – esophageal anastomosis – 1), IAA – esophageal division – 1. Early complication: chylothorax – 2, leak – 1. Two patients died on postoperative day 9 and 180 due to heart failure. The patient with IAA is just after cardiac surgery. The follow-up of remaining patients is 12-41 months.

Conclusions: Preoperative diagnosis of esophageal atresia and right aortic arch or right accessory subclavian artery is not an indication for left thoracotomy. Intraoperative finding of artery crossing the esophageal axis, that excludes its primary anastomosis, is not an indication for esophageal division, but for angio-CT. In case of esophageal atresia and dextrocardia access from left thoracotomy allows fistula ligation, but does not allow primary anastomosis. Mortality in patients with esophageal atresia and aortic arch failure results mainly from the presence of accompanying severe heart failures.