Background: Pulmonary artery sling is a rare vascular ring and is commonly associated with tracheal stenosis. Symptomatic newborns and infants with these complex lesions have a high mortality rate without surgical intervention. The ideal operation remains controversial, with debate focusing on the need for left pulmonary artery for reimplantation and the technique of tracheal reconstruction.
Methods: From 1983 to 2003, 14 patients with pulmonary artery sling (mean age, 7 months; range, 6 days to 27 months) underwent repair of pulmonary artery sling alone (6 patients), tracheoplasty alone (1 patient), and pulmonary artery sling repair with tracheoplasty (7 patients). Preoperatively, 7 patients were intubated, 2 had VATER (vertebral, anal, tracheal, esophageal, and radial anomalies) syndrome, and 2 patients had agenesis of the right lung. The left pulmonary artery was reimplanted at the ductal insertion site in 13 patients. One patient had left pulmonary artery translocation. Tracheoplasty employing extracorporeal circulation consisted of autologous pericardial patch (6 patients) or slide tracheoplasty (2 patients). Six patients with pulmonary artery sling and mild tracheal stenosis required only left pulmonary artery reimplantation. Concomitant procedures included closure of atrial septal defect (4 patients), ventricular septal defect (4 patients), and shunt for Fallot's tetralogy (2 patients).
Results: There were 2 hospital deaths (2 of 14;14%) from abdominal sepsis (1) and renal failure (1). Reoperations included diaphragm plication (2), tracheostomy (1), and bronchoscopy with laser resection of granulation tissue (5 patients: 2 slide, 3 pericardium). Follow-up was complete in all patients (mean, 42 months) with 1 late death from fungal sepsis. At follow-up, all left pulmonary artery anastomoses were patent by echocardiography, and no patient has required reoperation for trachea reconstruction.
Conclusions: These data demonstrate that tracheal repair is not always necessary in the presence of pulmonary artery sling; that agenesis of the right lung is not a contraindication to successful complete repair; and that simultaneous correction of intracardiac defects can be safely performed in selected patients. This study suggests that in newborns and infants, pericardial patch and slide tracheoplasty are effective methods for trachea reconstruction.