Diagnosis and surgical repair of congenital double aortic arch in infants

Abstract

Objectives Double 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. Methods The clinical data of 24 cases with double aortic arch (DAA) from January 2008 to January 2018 in our hospital was reviewed retrospectively. Results A 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. Conclusions Tracheal 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.

Importance of Acute Anterior Angulation in Double Aortic Arch Needing Attention at Primary Surgery

“…Management of a vascular ring has evolved since it was first repaired in 1945 by Dr. Robert Gross in Boston Children's Hospital ( 1 ). Despite advanced diagnostics and improved surgical techniques, a considerable number of children still continue to have symptoms after surgery ( 2 – 4 ).…”

Noisy Breathing in an Infant: A Case Report

“…While not performed in our patient, barium esophagography has its place in the diagnostic algorithm, specifically when other evaluations such as echocardiography are not readily available for patients presenting with suspicious symptoms. As for echocardiography, one diagnostic weakness is its inability to visualize atretic segments of vascular rings [ 8 ]. Furthermore, in a double aortic arch, the left arch is often in an appropriate position and may give the impression of normal anatomy if detailed sweeps are not obtained.…”

Aortic Arch Variants and Anomalies: Embryology, Imaging Findings, and Clinical Considerations

“… 69) The long-term prognosis of patients with a double aortic arch who have undergone surgical treatment is excellent. 70) During surgery, the smaller segment or atretic portion of the aortic arch is usually resected to disrupt the complete vascular ring and resolve tracheoesophageal compression.…”