Pulmonary artery sling: An overview

Abstract

Pulmonary artery sling is a rare childhood vascular tracheobronchial compression syndrome that is frequently associated with tracheal stenosis. Consequently, neonates or infants may present with critical airway obstruction if there is long segment airway narrowing and complete rings. Rapid diagnosis of this cardiac vascular malformation and evaluation of the extent and severity of airway involvement is essential to plan surgery, typically a slide tracheoplasty to relieve critical airway obstruction. Long term outcome can be excellent following surgical repair of the stenosed airway and reimplantation of the left pulmonary artery. In this review we focus on the embryology, diagnostic workup, airway investigations and management for this rare but challenging congenital condition.

Left pulmonary artery sling and partial anomalous right pulmonary artery in a child with tetralogy of fallot

“…PAS is a rare congenital anomaly characterized by the anomalous origin of one of the branch pulmonary artery from the contralateral side pulmonary artery, with left PAS being much more common than right PAS. 1 The aberrant left pulmonary artery typically courses between the trachea and the esophagus, with or without extrinsic compression of the airway. Wells et al classified PAS into two types based on the tracheobronchial anatomy, with type 1 having a normal tracheobronchial pattern and type II having an abnormal trachea-bronchial tree pattern without a well-defined carina.…”

“…2 It may be isolated or associated with other thoracic and extra-thoracic anomalies, with a 40%-80% reported incidence of associated congenital heart diseases. 1 Clinical features range from an asymptomatic state to severe airway compromise. Early diagnosis and management are crucial for an optimal clinical outcome.…”