Abstract

ObjectivesDouble aortic arch (DAA) is a rare congenital vascular malformation. This study aims to summarize the experience of diagnosis and surgical treatment for congenital double aortic arch.MethodsThe clinical data of 24 cases with double aortic arch (DAA) from January 2008 to January 2018 in our hospital was reviewed retrospectively.ResultsA total of 24 cases, including 12 patients with isolated DAA and 12 patients with DAA and associated intracardiac defects were identified. There were 14 males and 10 females, with an average age of 11 months. The associated intracardiac malformations included ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), tetralogy of Fallot (TOF), transposition of the great arteries (TGA), pulmonary stenosis (PS), and patent foramen ovale (PFO). Of the 12 patients with DAA and intracardiac malformations, 7 patients underwent intracardiac repair simultaneously, however, 3 patients underwent isolated double aortic arch correction. One patient with DAA and TGA underwent surgical correction of congenital vascular ring at the first stage, and the arterial switch operation was performed at the second stage. The clinical outcomes of 23 patients were promising, however, in one patient, parents decided not to do the surgery due to personal reasons. The average follow-up time was 35 months.ConclusionsTracheal and esophageal compression are commonly seen in patients with DAA, however could be relieved significantly after surgery. In particular cases, the simultaneous intracardiac defects repair could be performed. Misdiagnosis was easily established with isolated echocardiography. Fortunately, the correct diagnosis of DAA and associated intracardiac defects could be established with the use of combined chest computed tomography.

Highlights

  • The Congenital Double Aortic Arch (DAA) which accounts for 46–76% of the complete rings is the most common vascular malformation in the congenital annulus [1, 2]

  • Tracheal and esophageal compression are commonly seen in patients with DAA

  • We introduce the concept of tracheal stenosis index, which means The diameter of tracheal stenosis (DTS)/ The length of tracheal stenosis (LTS) × 100%, can be used as a quantitative indicator to determine the degree of tracheal stenosis, the smaller the index value, the more severe the tracheal stenosis

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Summary

Introduction

The Congenital Double Aortic Arch (DAA) which accounts for 46–76% of the complete rings is the most common vascular malformation in the congenital annulus [1, 2]. The anatomy of the double aortic arch is mainly the right arch sends out the right subclavian artery and the right common carotid artery. The left part of the left arch produces the left subclavian artery and the left. Tracheal and esophageal compression are commonly seen in patients with DAA. In the early stage,the symptoms of tracheal compression are more obvious,such as repeated pulmonary infection, wheeze, shortness of breath and so on. It is less difficult to swallow due to esophageal stricture. The difficulty of swallowing is relatively obvious as times go on, so early intervention is very important

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