Pulmonary Artery Sling Causing Tracheal Stenosis in a Neonate.

Abstract

A 12-day-old boy (weight: 5 lb, 4 oz) presented with severe respiratory distress. A systolic ejection murmur was heard over the pulmonary artery. A transthoracic echocardiogram showed an anomalous origin of the left pulmonary artery (LPA) (Fig. 1). Computed tomograms showed that the LPA originated from the distal right pulmonary artery (RPA) and coursed between the trachea and esophagus to the left lung hilum (Figs. 2 and ​and3).3). A 27-mm tracheal stenosis was seen in the mid-distal trachea (Fig. 4). The diagnosis was pulmonary artery sling with consequent tracheal stenosis. Fig. 1. Transthoracic echocardiogram shows an expanded main pulmonary artery (MPA) and an anomalous origin of the left pulmonary artery (LPA). Fig. 2. Computed tomogram shows an anomalous origin of the left pulmonary artery (LPA) from the right pulmonary artery (RPA), and the LPA's course between the trachea and the esophagus to the left lung hilum. Fig. 3. Computed tomogram (3-dimensional reconstruction) shows a normal right pulmonary artery (RPA) arising from the main pulmonary artery (MPA), and an anomalous origin of the left pulmonary artery (LPA) from the RPA. Fig. 4. Computed tomographic reconstruction of the trachea shows a 27-mm stenotic segment (arrow) in the mid-distal trachea. After median sternotomy, cardiopulmonary bypass (CPB) was started. We transected the LPA from its origin on the RPA and anastomosed it, end-to-side, to the left lateral aspect of the main pulmonary artery in front of the trachea. We used continuous 6-0 Prolene suture to close the stump of the RPA. We then opened the trachea anteriorly through the area of the complete tracheal rings. The mid portion of the stenotic trachea (6 rings, 15-mm length) was removed for use as an autograft. After we had anastomosed the trachea posteriorly, we trimmed the autograft patch and inserted it anteriorly to augment the tracheal lumen. The patient recovered uneventfully.