A Case of Pulmonary Artery Sling Associated with Left Bronchial Hypoplasia

Abstract

Pulmonary artery sling is a rare congenital vascular anomaly in which the left pulmonary artery arises aberrantly from the proximal part of the right pulmonary artery and encircles the right main bronchus and distal trachea before entering the hilum of the left lung. This causes compression of the trachea and right main bronchus, and most infants with this anomaly have severe respiratory distress within the first year of life. Associated anomalies are common, and particularly, tracheobronchial anomalies occur in 40% of the patients. Major cardiovascular anomalies including ventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot, common ventricle, and coarctation of the aorta, occur in about half of the affected infants. We have experienced an 11-month-old male infant with respiratory distress, wheezing, and cyanosis. He was diagnosed as pulmonary artery sling associated with left bronchial hypoplasia by echocardiography, angiography, and chest CT scan. Other associated anomalies were left kidney agenesis, right megaureter and right kidney hypertrophy, diagnosed by abdominal sonography and IVP. We report this case with a brief review of literature.