Rare Association of Pulmonary Artery Sling (PAS) and Right Lung Aplasia (RLA) in an Infant

A one-month-old baby presented to the pulmonology unit at Mansoura University Children's Hospital, Mansoura, Egypt, with respiratory distress and a wheezy chest. A chest X-ray showed an opacified right side of the chest with a marked shift of the mediastinum to the right. A flexible fiberoptic bronchoscopy (FFB) was completed and showed a primitive right bronchial dimple with moderate compression of the lower trachea and marked compression of the left mainstem bronchus.

A cardiac multidetector CT with virtual bronchoscopy was done and showed an absent right lung. The main pulmonary artery continued as the left pulmonary artery passed behind the lower end of the trachea. The left main stem bronchus was seen compressed posteriorly between the left upper pulmonary vein anteriorly and the left pulmonary artery. Virtual bronchoscopy showed a right bronchial dimple and marked compression of the left main stem bronchus.

The patient was managed surgically through median sternotomy and aortic and single venous cannulation with a beating heart. The patient was extubated on postoperative day two and went home on postoperative day eight.

Reference(s)

Phillips RR, Culham JA: Pulmonary artery sling and hypoplastic right lung: Diagnostic appearances using MRI. Pediatr Radiol 23:117-119, 1993

Pu WT, Chung T, Hoffer FA, et al: Diagnosis and management of agenesis of the right lung and left pulmonary artery sling. Am J Cardiol 78:723-727, 1996