CH Xie, FQ Gong, GP Jiang, SL Fu

Abstract

Background: This study aimed to report the diagnosis, treatment, outcome and variable anomalies of the children with double aortic arch (DAA). Methods: The medical records of the patients with DAA treated at Children's Hospital, Zhejiang University School of Medicine were reviewed for the following information: demographic information, clinical characteristics, imaging results, treatment and outcome. Results: Eleven patients with DAA were included into the study. Age of diagnosis raged from 5 days to 6.6 years with a mean of 26 months; age of onset ranged from 3 days to 8 months. 81.8% patients (9/11) had initial respiratory symptoms including cough, tachypnoea and wheezing; five patients had feeding difficulty or dysphagia. Seven patients had heart murmur and 3 had cyanosis. Primary diagnosis was asthma or respiratory infections in six patients, congenital heart anomalies in 4 and double aortic arch only in 1. All patients were indicated for surgery corrections for DAA or associated heart anomalies. Five out of the 11 patients underwent surgery timely after diagnosis; five parents refused surgery treatment for their children and one delayed surgery for their child because of economical problems. Outcome was favorable for the patients who underwent surgery at early age and associated with minor heart anomalies; and they were symptom-free at six months after surgery. Conclusion: For the children with recurrent respiratory symptoms, vascular rings should be considered in differential diagnosis. Symptomatic patients with DAA should be subjected to surgeries timely. Medical insurance policies should be improved for those families with low income and immigrant populations.