Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium

Part of Esophageal Atresia & Tracheoesophageal Fistula 5 items Congenital Gastrointestinal Anomalies 14 items

pubmed.ncbi.nlm.nih.gov

Daniel Ostlie

Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF. Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up. Results: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54-2.65) vs. 2.57 kg (2.00-3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32-37) vs. 37 weeks (35-39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/T

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Article · Aug 2018 · 1 min read

In brief

Multi-institutional study of 396 infants with esophageal atresia found 5% had right aortic arch, associated with lower birth weight, earlier gestational age, and 90% incidence of congenital heart disease. Most underwent successful repair via right thoracotomy, though optimal surgical approach remains debated.

Written by the GCMD Library team from the article.

Daniel Ostlie

Statements in this article

Right sided aortic arch occurs in 5% of infants with esophageal atresia.
Research
Infants with esophageal atresia and right aortic arch have a lower median birth weight compared to those with left sided arch (1.96 kg vs. 2.57 kg, p = 0.01).
ResearchResults
Infants with esophageal atresia and right aortic arch have an earlier median gestational age compared to those with left sided arch (34.5 weeks vs. 37 weeks, p = 0.01).
ResearchResults
Infants with esophageal atresia and right aortic arch have a higher incidence of congenital heart disease compared to those with left sided arch (90% vs. 32%, p < 0.0001).
ResearchResults
The most common cardiac lesions in infants with esophageal atresia and right aortic arch are ventricular septal defect, tetralogy of Fallot, and vascular ring.
ResearchResults
Among infants with right aortic arch undergoing esophageal atresia repair, 71% underwent right thoracotomy and 29% underwent left thoracotomy.
ResearchResults