Pulmonary artery sling diagnosed and corrected in an 11-year-old boy with refractory pulmonary infections and childhood-onset asthma

Pulmonary artery sling (PAS) is a congenital anomaly in which a pulmonary artery branch crosses respiratory tract organs (trachea or main bronchi) from the opposite side with their overlapping and airway obstruction. The defect is usually diagnosed in early infancy because of an evident clinical manifestation related more to respiratory than circulatory problems [1]. This rare anomaly is usually identified in the settings of complex congenital anomalies, although it can occur as an isolated problem or partial form with usually coexisting tracheal stenosis [2].
We report an adolescent patient with PAS that was diagnosed late after recurrent and refractory pulmonary infections, and symptoms mimicking childhood-onset asthma. Surgical correction with anatomic reconstruction of the anatomic position of the main pulmonary artery with its bifurcation into the natural course was planned as a procedure of choice.
The 11-year-old boy, a 28 kg body weight adolescent, was referred from a regional pediatric cardiology center after the initial diagnosis of pulmonary bed defect (PA sling) in the course of recurrent respiratory infections and pediatric asthma.
Before the scheduled anatomic correction, he underwent computed tomography angiography (angio-CT) with precise reconstruction of natural anatomical settings (Fig. 1 A). Additionally we performed tracheal bronchoscopy t o assess the anatomy, inner diameters and the evidence of external impression of the trachea with its bifurcation. Great care was taken to exclude the evidence of secondary permanent tracheal malacia in the effect of chronic impression by the pathological course of the left pulmonary artery (LPA) (Fig. 1 B).Diagnostic transthoracic echocardiography (TTE) proved good cardiac function with moderate pulmonary valve insufficiency, and the picture of continued flow from the pulmonary artery (PA) trunk directly to the right pulmonary artery (RPA), while the position of the LPA was difficult to visualize with carefully repeated classic TTE.
He was finally referred for surgical correction of the defect. The procedure was performed with normothermic extracorporeal circulation on a beating heart. Intraoperatively an extended PA trunk and oversized RPA were identified. After meticulous preparation an anomalous origin of the LPA arising from the extended proximal RPA crossing the trachea from its posterior side, directly above the tracheal bifurcation, was found. Initially the LPA was...


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