Clinical expression of congenital malformations of the aortic arch and/or its major branches in childhood

Pressure on the trachea and/or esophagus by congenital malformations of the aorta and/or its major branches is not uncommon. 10 children with such anomalies were diagnosed in the past three years, of whom four had double aortic arch, four a right aberrant subclavian artery and in two, anomalies of the innominate artery. The mean age at diagnosis was six months. The most important clinical signs were repeated upper respiratory infections in 70% of the cases and respiratory distress in 50% of the cases. Noisy respiration, hoarseness, wheezing, congenital stridor and dysphagia were also found. The presence of permanent stridor was rare. The diagnosis was made in all the cases by esophageal screening that demonstrated signs of compression of the esophagus; the trachea or both.