Congenital vascular rings as a cause of respiratory and esophageal problems in children

Abstract

Vascular rings (VR), are anomalies of the aortic arch that result from abnormal development of the embryonic aortic arches. The prevalence of VR is approximately 1%, of which 55% are double aortic arch. This congenital heart defect can be either isolated or combined with other congenital heart defects. Associated cardiac pathology occurs in 12.6%, and includes ventricular septal defect and Tetralogy of Fallot. Sometimes this defect is associated with DiGeorge’s and Turner’s syndrome. The timing of appearance of clinical symptoms varies from the early neonatal period to adulthood. However, most patients develop clinical symptoms in the first months after birth, and they indicate the need of cardiac surgery during the first year of life. Given the serious consequences of this anomaly, VR should be included in the list of differential diagnoses of wheezing, stridor, difficulty swallowing and bronchial obstruction syndrome in children, especially when these symptoms appear since birth, are persistent and respond poorly to standard therapeutical approaches. Therefore, it is important for pediatricians to be alert for a congenital heart defect such as VR as one of the possible causes of congenital stridor, wheezing, and swallowing disorders in infants. Contrast-enhanced computed tomography and magnetic resonance imaging of the heart are the diagnostic modalities of choice for VR diagnosis. These diagnostic methods provide accurate location, nature of branching and dominance of the aortic arch as well as the degree of compression of the airway and esophagus. Three-dimensional reconstruction helps to plan future surgical intervention