Management of pulmonary artery sling associated with tracheal stenosis

Background. Pulmonary artery sling is often associated with congenital tracheal stenosis and intracardiac anomalies. This study examines the influence of concomitant anomalies and individual surgical procedures. Methods. Between 1984 and 2006, 31 patients underwent surgical repair of pulmonary artery sling (median, 6 months; range, 29 days to 9 years). Twenty-eight of them underwent left pulmonary artery reimplantation and tracheoplasty, whereas the remaining 3 received only left pulmonary reimplantation. The first 4 patients with long segment tracheal stenosis were treated with a costal cartilage graft and the next 19 with slide tracheoplasty. Five patients underwent tracheal resection with end-to-end anastomosis. Associated cardiac anomalies in 10 patients were repaired concomitantly with left pulmonary artery reimplantation and tracheoplasty. Results. Two patients died early of low-output syndrome (n = 1) and ventricular arrhythmia (n = 1). Follow-up was complete in all patients (median, 4.6 years) with 3 late deaths arising from residual tracheal stenosis or pulmonary hypertension. Two survivors with a costal cartilage graft remain free of respiratory problems. Of 16 survivors who underwent slide tracheoplasty, 9 who did not have tracheostomy are fully active and 7 had tracheostomy to treat tracheomalacia and granulation. Among 5 survivors of tracheal resection, 4 are doing well without tracheostomy. Echocardiography showed that the left pulmonary artery was patent in all assessed patients, but 1 required balloon arterioplasty. Conclusions. Left pulmonary reimplatation with simultaneous repair of tracheal stenosis and intracardiac anomalies results in low operative mortality and satisfactory left pulmonary artery patency. However, the management of younger infants with tracheoplasty for long segment stenosis involving carina or complex cardiac anomalies remains challenging.