Surgical Treatment of Pulmonary Artery Sling

Pulmonary artery (PA) sling is a rare congenital vascular anomaly. Pathologically, the left PA originates from the posterior aspect of the right PA and it passes between the trachea and esophagus toward the left lung, compressing the lower trachea. Pulmonary artery sling could be an isolated condition or associated with intracardiac defects like atrial septal defect or tetralogy of Fallot. The pathology is often called a “ring–sling complex,” as the prevalence of complete tracheal rings (50%-65%) is quite high in these patients. Patients usually present with stridor, and respiratory distress during early infancy and respiratory symptoms result from both external compression and intrinsic stenosis of the trachea. Therefore, in patients with a diagnosis of PA sling, preoperative evaluation of the trachea is mandatory. With this regard, in addition to echocardiography, preoperative airway imaging with rigid bronchoscopy and computed tomography imaging of the chest should be performed (1). Also, as a result of esophageal compression, it can be associated with dysphagia (2).

The need for tracheal surgery is related to the severity of tracheal involvement, and it has been shown that patients who do not need tracheal surgery concomitant to repair of PA sling have perfect outcomes after surgery. On the other hand, the need for tracheal surgery was found to be the major determinant of mortality after surgery (3). In this presentation, the authors present their experience in the surgical treatment of pulmonary artery sling in two pediatric patients.

References

1. Backer CL, Russell HM, Kaushal S, Rastatter JC, Rigsby CK, Holinger LD. Pulmonary artery sling: current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg. 2012;143:144-151.
2. Yurdakul Y, Arsan S, Farsak B, Peker O, Celiker A, Girbolar A. Pulmonary vascular sling responsible for esophageal and tracheal obstruction. A case report. Angiology. 1996;47:511-515.
3. Yong MS, d'Udekem Y, Brizard CP, Robertson T, Robertson CF, Weintraub R, et al. Surgical management of pulmonary artery sling in children. J Thorac Cardiovasc Surg. 2013;145:1033-1039.

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