Vascular Rings and Pulmonary Artery Sling

The pulmonary artery sling is a rare vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery and encircles the right main stem bronchus and distal trachea before entering the hilum of the left lung. The history of vascular ring surgery began with the division of a double aortic arch by Gross at Boston Children's Hospital on June 9, 1945. Most children with vascular rings present with symptoms within the first several weeks to months of life. The symptoms typically include some combination of respiratory distress, stridor, the classic “seal bark” cough, apnea, dysphagia, and recurrent respiratory tract infections. The diagnosis of vascular rings requires a high index of suspicion, because of the relative infrequency of this diagnosis compared with other conditions that cause respiratory distress in children such as asthma, reflux, and upper respiratory tract infection.