Pulmonary Artery Sling with Situs Solitus Dextroposition of Heart and Left Superior Vena Cava

ABSTRACT

Pulmonary artery sling is a rare congenital cardiovascular anomaly which presents clinical obstructive symptoms and signs of trachea, right main bronchus and esophagus due to left pulmonary artery abnormaly arises from proximal part of right pulmonary artery. Aberrant left pulmonary artery courses to the right above right main bronchus and then turns to the left, and courses to between the anterior of the esophagus and the posterior aspect of trachea. One of important clinical significances of the pulmonary artery sling is associated with intracardiac anomalies, and with tracheal stenosis and bronchomalacia due to press tracheobronchial tree by the aberrant pulmonary artery. It has been thought that embryological malconnection between the pulmonary artery bud from left sixth arch and the pulmonary posterior branchial plexus in the left lung bud. We had experienced 21 months old male infant with left pulmonary artery sling associated with persistent left superior vena cava and dextropostioned heart. The heart seems to move to right hemithorax due to hypoplasia or segmental atelectasis of right lung.